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Metastatic Primary Extramammary Paget Disease: A Case Series
Metastatic Primary Extramammary Paget Disease: A Case Series
Extramammary Paget disease (EMPD) is a rare cutaneous malignancy typically seen in apocrine-rich areas, including the axillae and anogenital region. It presents as a slow-growing, erythematous patch or plaque that commonly is misdiagnosed as an infectious or inflammatory condition.1,2 Primary EMPD occurs as a intraepithelial neoplasm, whereas secondary EMPD occurs due to epidermotropic metastases or direct extension of an underlying adenocarcinoma into the skin.1 Most commonly, primary EMPD occurs in situ; however, when present, dermal invasion and metastases from the skin are associated with poorer outcomes.3 Given the rarity of metastatic disease, existing literature is limited to case reports and case series.
We present 2 patients with metastatic primary EMPD who had evidence of invasion on initial biopsy and died secondary to metastatic EMPD. We conducted a comprehensive review of the literature for invasive and metastatic EMPD to highlight key clinicopathologic features, treatment considerations, and the potential for rapid disease progression in cases of invasive EMPD.
Case Series
Patient 1—A 68-year-old White man with a history of breast cancer (in remission) presented to our clinic for further management of biopsy-proven scrotal EMPD. Prior to biopsy, he described a 6-month history of worsening scrotal rash treated with topical antifungals, oral antibiotics, and topical steroids due to presumed diagnosis of intertrigo, cellulitis, and dermatitis, respectively. Clinical examination showed indurated, erythematous, ulcerated plaques involving the bilateral groin, genitalia, and perineum (Figure 1). Skin biopsy confirmed a diagnosis of EMPD with both dermal and lymphovascular invasion. An immunohistochemical profile was positive for CK7 and carcinoembryonic antigen (CEA) and negative for CK20 (Figure 2).
At presentation, the patient had palpable lymphadenopathy and scrotal edema concerning for inguinal and iliac lymph node metastases. Workup for an underlying adenocarcinoma included computed tomography (CT) of the chest, abdomen, and pelvis; urologic consultation with cystoscopy; and a screening colonoscopy. The CT scan revealed multiple enlarged inguinal and external iliac lymph nodes. Fine-needle aspiration revealed CK7- and CEA-positive neoplastic cells consistent with metastatic EMPD. The patient was treated with 6 cycles of carboplatin-paclitaxel, palliative radiation therapy, and pembrolizumab with minimal response to treatment and development of osteolytic vertebral lesions concerning for disease progression. He died 1 year after the initial diagnosis secondary to the disease.
Patient 2—A 79-year-old White man presented for further management of an outside diagnosis of superficially invasive primary EMPD of the bilateral inguinal folds and scrotum that had been present for 5 months prior to biopsy and diagnosis. Clinical examination at initial presentation revealed erythematous patches of the bilateral inguinal folds and scrotum, as well as an erythematous scaling plaque in the right axilla. There was no palpable clinical lymphadenopathy. Biopsy of the axilla and groin were both consistent with invasive EMPD with positive staining for CK7 and negative staining for CK20 and CDX2. Workup for underlying adenocarcinoma with whole-body positron emission tomography/CT, mammography, esophagogastroduodenoscopy, serum CEA, colonoscopy, and cystoscopy were all negative for a metastatic adenocarcinoma. There was no imaging or clinical evidence of lymphadenopathy. Complete circumferential peripheral and deep-margin assessment was performed in a staged manner on both sites, and negative margins were obtained.
Surveillance imaging 6 months after surgery revealed suspicious hepatic lesions. Fine-needle aspiration of the hepatic lesions demonstrated positive staining for CK7 and negative staining for CK20, CDX2, prostate-specific antigen, and thyroid transcription factor 1, consistent with metastatic EMPD. Oncology recommended carboplatin and docetaxel or docetaxel monotherapy chemotherapy. The patient was further managed by an outside oncologist due to ease of travel but died secondary to the disease 15 months following the initial diagnosis.
Comment
Extramammary Paget disease is an uncommon cutaneous malignancy that manifests as pruritic erythematous plaques within apocrine-rich areas such as the genitalia, axillae, or anal region. It most commonly occurs in patients older than 65 years, with White women and Asian men being affected at disproportionately higher rates.1,4 Delay in diagnosis is common, as EMPD can mimic other benign inflammatory or infectious conditions, including contact dermatitis, seborrheic dermatitis, tinea, candidiasis, and eczema.1
Metastatic and multifocal cases of primary EMPD are especially rare. According to a search of PubMed articles indexed for MEDLINE published through December 2023 using the terms extramammary Paget disease, EMPD, neoplasm metastasis, invasive extramammary, and neoplasm invasiveness, we identified 5040 cases of invasive EMPD and 477 cases of metastatic EMPD.5-37 Of the reports that disclosed patient demographic information, 3627 patients were female 1410 were male, and the mean age was 67 years. Sites of metastases included regional lymph nodes, liver, lungs, cervix, bladder, bone, brain, skin, kidney, and adrenal glands
Workup for EMPD—The initial steps for workup of EMPD include a thorough physical examination and lymph node assessment. A skin biopsy also should be performed for patients presenting with refractory, pruritic, and eczematous rashes in apocrine-rich areas to evaluate for EMPD.1 Characterization of large and complex tumors is better achieved through multiple biopsies with particular focus on nodular or thickened areas, as these may indicate invasive disease.2 Primary EMPD is characterized by pagetoid cells with abundant pale cytoplasm proliferating in a single-cell or nested pattern within the epidermis or dermis in invasive disease and often is accompanied by dermal lymphocytic inflammation.1 Immunohistochemistry demonstrates positive staining for CEA, CK7, and CK8, with negative staining for indicators of secondary EMPD including CK20 and CDX2.1,2
As part of the workup, it is critical to distinguish between primary disease and secondary EMPD.1 Beyond skin and clinical lymph node examination, additional workup should be based on age-appropriate and location-directed malignant neoplasm screenings, including colonoscopy, cystoscopy, prostate examination, mammography, and Papanicolaou test. Advanced imaging such as CT, positron emission tomography, or magnetic resonance imaging can be used to assess for metastatic disease if internal malignant neoplasms are present on initial screening or clinical lymphadenopathy is identified.2 Additionally, it can be helpful in the evaluation for nodal disease in cases of invasive EMPD.
The likelihood of associated underlying carcinomas varies depending on the site of involvement.38,39 For example, vulvar involvement constitutes approximately 65% of EMPD cases, with 11% to 20% of cases being associated with underlying gastrointestinal or genitourinary carcinomas. Involvement of the male genitalia, as in our 2 patients, is rare, accounting for approximately 14% of cases, 11% of which are associated with prostate, testicular, and bladder carcinoma. Perianal involvement comprises 20% of EMPD cases and has the greatest risk for underlying malignancy with an incidence of 33% to 86%, the majority of which are rectal or tubo-ovarian cancers.38,39 Consideration of the frequency and types of underlying carcinoma of respective sites of involvement can be helpful when ruling out secondary EMPD.
In both of our patients, palpable lymphadenopathy at the time of original diagnosis and histologic invasive disease on initial biopsy warranted thorough imaging and laboratory workup; there was no evidence of primary malignancy. Given the absence of an underlying carcinoma, both patients were classified as having metastatic primary EMPD.
Assessment of lymphadenopathy is an essential aspect of disease workup, as it is associated with a statistically higher rate of lymph node metastases. A study by Fujisawa et al20 demonstrated that 80% of patients with lymphadenopathy had regional metastases compared to only 15% of patients without clinical lymphadenopathy. The presence of invasive disease also has been shown to correspond with lymph node metastases.40 Ogata et al40 showed that 0% of cases with in situ EMPD had a positive sentinel lymph node biopsy (SLNB) compared to 4% and 43% in cases that showed evidence of microinvasion and dermal invasion, respectively. Lymph node metastases are associated with poor prognosis, with increasingly worse prognosis when there are multiple lymph nodes affected.41 In our case series, patient 1 had lymphadenopathy and both patients had invasive EMPD; they both later developed metastases and died.
Lymphadenopathy should be further investigated with imaging and biopsy or fine-needle aspiration.42 Recent expert consensus guidelines recommended this method of investigation over routine use of SLNB, as there is no evidence that a positive SLNB affects treatment that changes disease-specific survival.2
Treatment of EMPD—Surgical excision of the primary lesion is the first-line treatment of EMPD,1,2 which can be performed by wide local excision; however, studies have demonstrated higher recurrence-free survival with margin-controlled surgery (complete circumferential peripheral and deep margin assessment) or Mohs micrographic surgery (MMS), especially with CK7 immunostaining.2,37,43 The literature on MMS of invasive EMPD is sparse, accounting for 57 patients.25,37,44 Other reports describe management with surgical excision, wide local excision, regional resection, or vulvectomy, in addition to lymph node dissection, radiation therapy (RT), and/or chemotherapy.1-36,39,43-46 Despite the improved outcomes with MMS, the predominance of other surgical approaches in our search suggests that MMS may be currently underutilized for the treatment of invasive or locally advanced EMPD.
Among patients with unresectable disease or distant metastases, management includes RT with curative intent, chemotherapy, or a combination of both.1,2 In our review, 267 cases were treated using RT and 77 with chemotherapy. Radiation therapy is an effective therapeutic option with a reported response rate of 62% to 100% and can be employed as either primary or adjuvant treatment.3 For patients with lymph node metastasis the combination of RT and lymph node dissection has been shown to have improved outcomes compared to lymph node dissection alone, with 1 study showing a 5-year survival of 75% for patients who received adjuvant RT compared to 0% for lymph node dissection alone.45
There are currently no consensus guidelines on the best chemotherapeutic regimen for metastatic EMPD. Several regimens have been reported, including docetaxel monotherapy; low-dose 5-fluorouracil and cisplatin; combination chemotherapy FECOM (5-fluorouracil, epirubicin, carboplatin, vincristine, mitomycin); or combination therapy with cisplatin, epirubicin, and paclitaxel.1
Prognosis of Metastatic EMPD—Because invasive and metastatic EMPD is rare, its natural history is hard to predict. Poor prognosis is associated with nodule formation, tumor thickness, perianal or vaginal involvement, lymphovascular invasion, nodal metastasis, and distant metastasis. The 5-year survival for metastatic EMPD has been reported to be less than 10%.46 Our cases underscore the poor prognostic risk associated with metastatic EMPD.
For all cases of EMPD, close follow-up is warranted. Guidelines recommend physical examination with lymph node assessment every 3 to 6 months for 3 years and every 6 to 12 months for the subsequent 5 years.2 Specific recommendations for follow-up in invasive disease have not yet been described, though the 20% probability of developing an internal malignancy within 5 years after diagnosis and poor prognostic outcomes associated with invasive and metastatic disease support the need for close monitoring.2
Conclusion
Although in situ EMPD often is a slow-growing tumor with good prognosis, invasive disease has high potential to behave aggressively with high morbidity and mortality. Increased awareness and prompt identification of invasive EMPD, expedited comprehensive workup, and early multidisciplinary management might impact patient outcomes.
Acknowledgment—The authors would like to thank Ellen Aaronson, MLIS, AHIP (Mayo Clinic Libraries [Jacksonville, FL]), for creating and conducting the narrative literature search in the MEDLINE database.
- Hashimoto H, Ito T. Current management and treatment of extramammary Paget’s disease. Curr Treat Options Oncol. 2022;23:818-830. doi:10.1007/s11864-021-00923-3
- Kibbi N, Owen JL, Worley B, et al. Evidence-based clinical practice guidelines for extramammary Paget disease. JAMA Oncol. 2022;8:618-628. doi:10.1001/jamaoncol.2021.7148
- Morris CR, Hurst EA. Extramammary Paget’s disease: a review of the literature part II: treatment and prognosis. Dermatol Surg. 2020;46:305-311. doi:10.1097/DSS.0000000000002240
- Merritt BG, Degesys CA, Brodland DG. Extramammary Paget disease. Dermatol Clin. 2019;37:261-267. doi:10.1016/j.det.2019.02.002
- Aroche Gutierrez LL, Holloway SB, Donthi D, et al. Docetaxel treatment for widely metastatic invasive vulvar extramammary Paget’s disease with multifocal bone metastasis. Gynecol Oncol Rep. 2022;45:101114. doi:10.1016/j.gore.2022.101114
- Ueda M, Omori M, Sakai A. Invasive extramammary Paget’s disease with lymph node metastases and high-grade B-cell lymphoma. An Bras Dermatol. 2023;98:414-418. doi:10.1016/j.abd.2022.04.012
- Rathore R, Yadav D, Agarwal S, et al. Primary extra mammary Paget’s disease of vulva, with apocrine adenocarcinoma, signet ring cell differentiation and distant metastasis. J Family Reprod Health. 2020;14:276-280. doi:10.18502/jfrh.v14i4.5213
- Kawahara Y, Umeda Y, Yamaguchi B, et al. Long-term resolution of invasive extramammary Paget’s disease with multiple regional lymph node metastases solely with regional lymph node dissection. J Dermatol. 2021;48:E452-E453. doi:10.1111/1346-8138.16007
- Hanyu T, Fujitani S, Ito A, et al. Brain metastasis from extramammary Paget’s disease. Nagoya J Med Sci. 2020;82:791-798. doi:10.18999/nagjms.82.4.791
- Waki Y, Nobeyama Y, Ogawa T, et al. Case of extramammary Paget’s disease causing pulmonary tumor embolism. J Dermatol. 2020;47:E133-E134. doi:10.1111/1346-8138.15267
- Li ZG, Qin XJ. Extensive invasive extramammary Paget disease evaluated by F-18 FDG PET/CT: a case report. Medicine (Baltimore). 2015;94:E371. doi:10.1097/MD.0000000000000371
- Kato N, Matsue K, Sotodate A, et al. Extramammary Paget’s disease with distant skin metastasis. J Dermatol. 1996;23:408-414. doi:10.1111/j.1346-8138.1996.tb04043.x
- Hosomi M, Miyake O, Matsumiya K, et al. Extramammary Paget’s disease with a large mass in male genitalia: a case report. Article in Japanese. Hinyokika Kiyo. 1989;35:1981-1984.
- Hardy LE, Baxter L, Wan K, et al. Invasive cervical adenocarcinoma arising from extension of recurrent vulval Paget’s disease. BMJ Case Rep. 2020;13e232424. doi:10.1136/bcr-2019-232424
- Onaiwu CO, Ramirez PT, Kamat A, et al. Invasive extramammary Paget’s disease of the bladder diagnosed 18 years after noninvasive extramammary Paget’s disease of the vulva. Gynecol Oncol Case Rep. 2014;8:27-29. doi:10.1016/j.gynor.2014.03.004
- Yao H, Xie M, Fu S, et al. Survival analysis of patients with invasive extramammary Paget disease: implications of anatomic sites. BMC Cancer. 2018;18:403. doi:10.1186/s12885-018-4257-1
- Kato H, Watanabe S, Kariya K, et al. Efficacy of low-dose 5-fluorouracil/cisplatin therapy for invasive extramammary Paget’s disease. J Dermatol. 2018;45:560-563. doi:10.1111/1346-8138.14247
- Yoshino K, Fujisawa Y, Kiyohara Y, et al. Usefulness of docetaxel as first-line chemotherapy for metastatic extramammary Paget’s disease. J Dermatol. 2016;43:633-637. doi:10.1111/1346-8138.13200
- Shu B, Shen XX, Chen P, et al. Primary invasive extramammary Paget disease on penoscrotum: a clinicopathological analysis of 41 cases. Hum Pathol. 2016;47:70-77. doi:10.1016/j.humpath.2015.09.005References
- Fujisawa Y, Yoshino K, Kiyohara Y, et al. The role of sentinel lymph node biopsy in the management of invasive extramammary Paget’s disease: multi-center, retrospective study of 151 patients. J Dermatol Sci. 2015;79:38-42. doi:10.1016/j.jdermsci.2015.03.014
- Dai B, Kong YY, Chang K, et al. Primary invasive carcinoma associated with penoscrotal extramammary Paget’s disease: a clinicopathological analysis of 56 cases. BJU Int. 2015;115:153-160. doi:10.1111/bju.12776
- Shiomi T, Noguchi T, Nakayama H, et al. Clinicopathological study of invasive extramammary Paget’s disease: subgroup comparison according to invasion depth. J Eur Acad Dermatol Venereol. 2013;27:589-592. doi:10.1111/j.1468-3083.2012.04489.x
- Hatta N, Morita R, Yamada M, et al. Sentinel lymph node biopsy in patients with extramammary Paget’s disease. Dermatol Surg. 2004;30:1329-1334. doi:10.1111/j.1524-4725.2004.30377.x
- Karam A, Dorigo O. Treatment outcomes in a large cohort of patients with invasive extramammary Paget’s disease. Gynecol Oncol. 2012;125:346-351. doi:10.1016/j.ygyno.2012.01.032
- Guo L, Liu X, Li H, et al. Clinicopathological features of extramammary Paget’s disease: a report of 75 cases. Article in Chinese. Zhonghua Yi Xue Za Zhi. 2015;95:1751-1754.
- Kilts TP, Long B, Glasgow AE, et al. Invasive vulvar extramammary Paget’s disease in the United States. Gynecol Oncol. 2020;157:649-655. doi:10.1016/j.ygyno.2020.03.018
- Kusatake K, Harada Y, Mizumoto K, et al. Usefulness of sentinel lymph node biopsy for the detection of metastasis in the early stage of extramammary Paget’s disease. Eur J Dermatol. 2015;25:156-161. doi:10.1684/ejd.2015.2534
- Jeong BK, Kim KR. Invasive extramammary Paget disease of the vulva with signet ring cell morphology in a patient with signet ring cell carcinoma of the stomach: report of a case. Int J Gynecol Pathol. 2018;37:147-151. doi:10.1097/PGP.0000000000000405
- Pagnanelli M, De Nardi P, Martella S, et al. Local excision of a mucinous adenocarcinoma of the anal margin (extramammary Paget’s disease) and reconstruction with a bilateral V-Y flap. Case Rep Surg. 2019;2019:9073982. doi:10.1155/2019/9073982
- Sopracordevole F, Di Giuseppe J, De Piero G, et al. Surgical treatment of Paget disease of the vulva: prognostic significance of stromal invasion and surgical margin status. J Low Genit Tract Dis. 2016;20:184-188. doi:10.1097/LGT.0000000000000191
- Evans AT, Neven P. Invasive adenocarcinoma arising in extramammary Paget’s disease of the vulva. Histopathology. 1991;18:355-360. doi:10.1111/j.1365-2559.1991.tb00857.x
- Kitano A, Izumi M, Tamura K, et al. Brain metastasis from cutaneous squamous cell carcinoma coexistent with extramammary Paget’s disease: a case report. Pathol Int. 2019;69:619-625. doi:10.1111/pin.12846
- Miracco C, Francini E, Torre P, et al. Extramammary invasive Paget’s disease and apocrine angiomatous hamartoma: an unusual association. Eur J Dermatol. 2018;28:853-855. doi:10.1684/ejd.2018.3438
- Kambayashi Y, Fujimura T, Ohuchi K, et al. Advanced invasive extramammary Paget’s disease concomitant with cecal cancer possessing rare variant of TP53 single nucleotide polymorphism. Case Rep Oncol. 2019;12:855-860. doi:10.1159/000504339
- Fujimura T, Furudate S, Kambayashi Y, et al. Potential use of bisphosphonates in invasive extramammary Paget’s disease: an immunohistochemical investigation. Clin Dev Immunol. 2013;2013:164982. doi:10.1155/2013/164982
- Kawamura H, Ogata K, Miura H, et al. Patellar metastases. A report of two cases. Int Orthop. 1993;17:57-59. doi:10.1007/BF00195227
- Damavandy AA, Terushkin V, Zitelli JA, et al. Intraoperative immunostaining for cytokeratin-7 during Mohs micrographic surgery demonstrates low local recurrence rates in extramammary Paget’s disease. Dermatol Surg. 2018;44:354-364. doi:10.1097/DSS.0000000000001355
- Morris CR, Hurst EA. Extramammary Paget disease: a review of the literature-part I: history, epidemiology, pathogenesis, presentation, histopathology, and diagnostic work-up. Dermatol Surg. 2020;46:151-158. doi:10.1097/DSS.0000000000002064
- Simonds RM, Segal RJ, Sharma A. Extramammary Paget’s disease: a review of the literature. Int J Dermatol. 2019;58:871-879. doi:10.1111/ijd.14328
- Ogata D, Kiyohara Y, Yoshikawa S, et al. Usefulness of sentinel lymph node biopsy for prognostic prediction in extramammary Paget’s disease. Eur J Dermatol. 2016;26:254-259. doi:10.1684/ejd.2016.2744
- Ohara K, Fujisawa Y, Yoshino K, et al. A proposal for a TNM staging system for extramammary Paget disease: retrospective analysis of 301 patients with invasive primary tumors. J Dermatol Sci. 2016;83:234-239. doi:10.1016/j.jdermsci.2016.06.004
- Fujisawa Y, Yoshino K, Kiyohara Y, et al. The role of sentinel lymph node biopsy in the management of invasive extramammary Paget’s disease: multi-center, retrospective study of 151 patients. J Dermatol Sci. 2015;79:38-42. doi:10.1016/j.jdermsci.2015.03.014
- Kim SJ, Thompson AK, Zubair AS, et al. Surgical treatment and outcomes of patients with extramammary Paget disease: a cohort study. Dermatol Surg. 2017;43:708-714. doi:10.1097/DSS.0000000000001051
- Wollina U. Extensive invasive extramammary Paget’s disease: surgical treatment. J Cutan Aesthet Surg. 2013;6:41-44. doi:10.4103/0974-2077.110098
- Tsutsui K, Takahashi A, Muto Y, et al. Outcomes of lymph node dissection in the treatment of extramammary Paget’s disease: a single-institution study. J Dermatol. 2020;47:512-517. doi:10.1111/1346-8138.15285
- Guercio BJ, Iyer G, Kidwai WZ, et al. Treatment of metastatic extramammary Paget disease with combination ipilimumab and nivolumab: a case report. Case Rep Oncol. 2021;14:430-438. doi:10.1159/000514345
Extramammary Paget disease (EMPD) is a rare cutaneous malignancy typically seen in apocrine-rich areas, including the axillae and anogenital region. It presents as a slow-growing, erythematous patch or plaque that commonly is misdiagnosed as an infectious or inflammatory condition.1,2 Primary EMPD occurs as a intraepithelial neoplasm, whereas secondary EMPD occurs due to epidermotropic metastases or direct extension of an underlying adenocarcinoma into the skin.1 Most commonly, primary EMPD occurs in situ; however, when present, dermal invasion and metastases from the skin are associated with poorer outcomes.3 Given the rarity of metastatic disease, existing literature is limited to case reports and case series.
We present 2 patients with metastatic primary EMPD who had evidence of invasion on initial biopsy and died secondary to metastatic EMPD. We conducted a comprehensive review of the literature for invasive and metastatic EMPD to highlight key clinicopathologic features, treatment considerations, and the potential for rapid disease progression in cases of invasive EMPD.
Case Series
Patient 1—A 68-year-old White man with a history of breast cancer (in remission) presented to our clinic for further management of biopsy-proven scrotal EMPD. Prior to biopsy, he described a 6-month history of worsening scrotal rash treated with topical antifungals, oral antibiotics, and topical steroids due to presumed diagnosis of intertrigo, cellulitis, and dermatitis, respectively. Clinical examination showed indurated, erythematous, ulcerated plaques involving the bilateral groin, genitalia, and perineum (Figure 1). Skin biopsy confirmed a diagnosis of EMPD with both dermal and lymphovascular invasion. An immunohistochemical profile was positive for CK7 and carcinoembryonic antigen (CEA) and negative for CK20 (Figure 2).
At presentation, the patient had palpable lymphadenopathy and scrotal edema concerning for inguinal and iliac lymph node metastases. Workup for an underlying adenocarcinoma included computed tomography (CT) of the chest, abdomen, and pelvis; urologic consultation with cystoscopy; and a screening colonoscopy. The CT scan revealed multiple enlarged inguinal and external iliac lymph nodes. Fine-needle aspiration revealed CK7- and CEA-positive neoplastic cells consistent with metastatic EMPD. The patient was treated with 6 cycles of carboplatin-paclitaxel, palliative radiation therapy, and pembrolizumab with minimal response to treatment and development of osteolytic vertebral lesions concerning for disease progression. He died 1 year after the initial diagnosis secondary to the disease.
Patient 2—A 79-year-old White man presented for further management of an outside diagnosis of superficially invasive primary EMPD of the bilateral inguinal folds and scrotum that had been present for 5 months prior to biopsy and diagnosis. Clinical examination at initial presentation revealed erythematous patches of the bilateral inguinal folds and scrotum, as well as an erythematous scaling plaque in the right axilla. There was no palpable clinical lymphadenopathy. Biopsy of the axilla and groin were both consistent with invasive EMPD with positive staining for CK7 and negative staining for CK20 and CDX2. Workup for underlying adenocarcinoma with whole-body positron emission tomography/CT, mammography, esophagogastroduodenoscopy, serum CEA, colonoscopy, and cystoscopy were all negative for a metastatic adenocarcinoma. There was no imaging or clinical evidence of lymphadenopathy. Complete circumferential peripheral and deep-margin assessment was performed in a staged manner on both sites, and negative margins were obtained.
Surveillance imaging 6 months after surgery revealed suspicious hepatic lesions. Fine-needle aspiration of the hepatic lesions demonstrated positive staining for CK7 and negative staining for CK20, CDX2, prostate-specific antigen, and thyroid transcription factor 1, consistent with metastatic EMPD. Oncology recommended carboplatin and docetaxel or docetaxel monotherapy chemotherapy. The patient was further managed by an outside oncologist due to ease of travel but died secondary to the disease 15 months following the initial diagnosis.
Comment
Extramammary Paget disease is an uncommon cutaneous malignancy that manifests as pruritic erythematous plaques within apocrine-rich areas such as the genitalia, axillae, or anal region. It most commonly occurs in patients older than 65 years, with White women and Asian men being affected at disproportionately higher rates.1,4 Delay in diagnosis is common, as EMPD can mimic other benign inflammatory or infectious conditions, including contact dermatitis, seborrheic dermatitis, tinea, candidiasis, and eczema.1
Metastatic and multifocal cases of primary EMPD are especially rare. According to a search of PubMed articles indexed for MEDLINE published through December 2023 using the terms extramammary Paget disease, EMPD, neoplasm metastasis, invasive extramammary, and neoplasm invasiveness, we identified 5040 cases of invasive EMPD and 477 cases of metastatic EMPD.5-37 Of the reports that disclosed patient demographic information, 3627 patients were female 1410 were male, and the mean age was 67 years. Sites of metastases included regional lymph nodes, liver, lungs, cervix, bladder, bone, brain, skin, kidney, and adrenal glands
Workup for EMPD—The initial steps for workup of EMPD include a thorough physical examination and lymph node assessment. A skin biopsy also should be performed for patients presenting with refractory, pruritic, and eczematous rashes in apocrine-rich areas to evaluate for EMPD.1 Characterization of large and complex tumors is better achieved through multiple biopsies with particular focus on nodular or thickened areas, as these may indicate invasive disease.2 Primary EMPD is characterized by pagetoid cells with abundant pale cytoplasm proliferating in a single-cell or nested pattern within the epidermis or dermis in invasive disease and often is accompanied by dermal lymphocytic inflammation.1 Immunohistochemistry demonstrates positive staining for CEA, CK7, and CK8, with negative staining for indicators of secondary EMPD including CK20 and CDX2.1,2
As part of the workup, it is critical to distinguish between primary disease and secondary EMPD.1 Beyond skin and clinical lymph node examination, additional workup should be based on age-appropriate and location-directed malignant neoplasm screenings, including colonoscopy, cystoscopy, prostate examination, mammography, and Papanicolaou test. Advanced imaging such as CT, positron emission tomography, or magnetic resonance imaging can be used to assess for metastatic disease if internal malignant neoplasms are present on initial screening or clinical lymphadenopathy is identified.2 Additionally, it can be helpful in the evaluation for nodal disease in cases of invasive EMPD.
The likelihood of associated underlying carcinomas varies depending on the site of involvement.38,39 For example, vulvar involvement constitutes approximately 65% of EMPD cases, with 11% to 20% of cases being associated with underlying gastrointestinal or genitourinary carcinomas. Involvement of the male genitalia, as in our 2 patients, is rare, accounting for approximately 14% of cases, 11% of which are associated with prostate, testicular, and bladder carcinoma. Perianal involvement comprises 20% of EMPD cases and has the greatest risk for underlying malignancy with an incidence of 33% to 86%, the majority of which are rectal or tubo-ovarian cancers.38,39 Consideration of the frequency and types of underlying carcinoma of respective sites of involvement can be helpful when ruling out secondary EMPD.
In both of our patients, palpable lymphadenopathy at the time of original diagnosis and histologic invasive disease on initial biopsy warranted thorough imaging and laboratory workup; there was no evidence of primary malignancy. Given the absence of an underlying carcinoma, both patients were classified as having metastatic primary EMPD.
Assessment of lymphadenopathy is an essential aspect of disease workup, as it is associated with a statistically higher rate of lymph node metastases. A study by Fujisawa et al20 demonstrated that 80% of patients with lymphadenopathy had regional metastases compared to only 15% of patients without clinical lymphadenopathy. The presence of invasive disease also has been shown to correspond with lymph node metastases.40 Ogata et al40 showed that 0% of cases with in situ EMPD had a positive sentinel lymph node biopsy (SLNB) compared to 4% and 43% in cases that showed evidence of microinvasion and dermal invasion, respectively. Lymph node metastases are associated with poor prognosis, with increasingly worse prognosis when there are multiple lymph nodes affected.41 In our case series, patient 1 had lymphadenopathy and both patients had invasive EMPD; they both later developed metastases and died.
Lymphadenopathy should be further investigated with imaging and biopsy or fine-needle aspiration.42 Recent expert consensus guidelines recommended this method of investigation over routine use of SLNB, as there is no evidence that a positive SLNB affects treatment that changes disease-specific survival.2
Treatment of EMPD—Surgical excision of the primary lesion is the first-line treatment of EMPD,1,2 which can be performed by wide local excision; however, studies have demonstrated higher recurrence-free survival with margin-controlled surgery (complete circumferential peripheral and deep margin assessment) or Mohs micrographic surgery (MMS), especially with CK7 immunostaining.2,37,43 The literature on MMS of invasive EMPD is sparse, accounting for 57 patients.25,37,44 Other reports describe management with surgical excision, wide local excision, regional resection, or vulvectomy, in addition to lymph node dissection, radiation therapy (RT), and/or chemotherapy.1-36,39,43-46 Despite the improved outcomes with MMS, the predominance of other surgical approaches in our search suggests that MMS may be currently underutilized for the treatment of invasive or locally advanced EMPD.
Among patients with unresectable disease or distant metastases, management includes RT with curative intent, chemotherapy, or a combination of both.1,2 In our review, 267 cases were treated using RT and 77 with chemotherapy. Radiation therapy is an effective therapeutic option with a reported response rate of 62% to 100% and can be employed as either primary or adjuvant treatment.3 For patients with lymph node metastasis the combination of RT and lymph node dissection has been shown to have improved outcomes compared to lymph node dissection alone, with 1 study showing a 5-year survival of 75% for patients who received adjuvant RT compared to 0% for lymph node dissection alone.45
There are currently no consensus guidelines on the best chemotherapeutic regimen for metastatic EMPD. Several regimens have been reported, including docetaxel monotherapy; low-dose 5-fluorouracil and cisplatin; combination chemotherapy FECOM (5-fluorouracil, epirubicin, carboplatin, vincristine, mitomycin); or combination therapy with cisplatin, epirubicin, and paclitaxel.1
Prognosis of Metastatic EMPD—Because invasive and metastatic EMPD is rare, its natural history is hard to predict. Poor prognosis is associated with nodule formation, tumor thickness, perianal or vaginal involvement, lymphovascular invasion, nodal metastasis, and distant metastasis. The 5-year survival for metastatic EMPD has been reported to be less than 10%.46 Our cases underscore the poor prognostic risk associated with metastatic EMPD.
For all cases of EMPD, close follow-up is warranted. Guidelines recommend physical examination with lymph node assessment every 3 to 6 months for 3 years and every 6 to 12 months for the subsequent 5 years.2 Specific recommendations for follow-up in invasive disease have not yet been described, though the 20% probability of developing an internal malignancy within 5 years after diagnosis and poor prognostic outcomes associated with invasive and metastatic disease support the need for close monitoring.2
Conclusion
Although in situ EMPD often is a slow-growing tumor with good prognosis, invasive disease has high potential to behave aggressively with high morbidity and mortality. Increased awareness and prompt identification of invasive EMPD, expedited comprehensive workup, and early multidisciplinary management might impact patient outcomes.
Acknowledgment—The authors would like to thank Ellen Aaronson, MLIS, AHIP (Mayo Clinic Libraries [Jacksonville, FL]), for creating and conducting the narrative literature search in the MEDLINE database.
Extramammary Paget disease (EMPD) is a rare cutaneous malignancy typically seen in apocrine-rich areas, including the axillae and anogenital region. It presents as a slow-growing, erythematous patch or plaque that commonly is misdiagnosed as an infectious or inflammatory condition.1,2 Primary EMPD occurs as a intraepithelial neoplasm, whereas secondary EMPD occurs due to epidermotropic metastases or direct extension of an underlying adenocarcinoma into the skin.1 Most commonly, primary EMPD occurs in situ; however, when present, dermal invasion and metastases from the skin are associated with poorer outcomes.3 Given the rarity of metastatic disease, existing literature is limited to case reports and case series.
We present 2 patients with metastatic primary EMPD who had evidence of invasion on initial biopsy and died secondary to metastatic EMPD. We conducted a comprehensive review of the literature for invasive and metastatic EMPD to highlight key clinicopathologic features, treatment considerations, and the potential for rapid disease progression in cases of invasive EMPD.
Case Series
Patient 1—A 68-year-old White man with a history of breast cancer (in remission) presented to our clinic for further management of biopsy-proven scrotal EMPD. Prior to biopsy, he described a 6-month history of worsening scrotal rash treated with topical antifungals, oral antibiotics, and topical steroids due to presumed diagnosis of intertrigo, cellulitis, and dermatitis, respectively. Clinical examination showed indurated, erythematous, ulcerated plaques involving the bilateral groin, genitalia, and perineum (Figure 1). Skin biopsy confirmed a diagnosis of EMPD with both dermal and lymphovascular invasion. An immunohistochemical profile was positive for CK7 and carcinoembryonic antigen (CEA) and negative for CK20 (Figure 2).
At presentation, the patient had palpable lymphadenopathy and scrotal edema concerning for inguinal and iliac lymph node metastases. Workup for an underlying adenocarcinoma included computed tomography (CT) of the chest, abdomen, and pelvis; urologic consultation with cystoscopy; and a screening colonoscopy. The CT scan revealed multiple enlarged inguinal and external iliac lymph nodes. Fine-needle aspiration revealed CK7- and CEA-positive neoplastic cells consistent with metastatic EMPD. The patient was treated with 6 cycles of carboplatin-paclitaxel, palliative radiation therapy, and pembrolizumab with minimal response to treatment and development of osteolytic vertebral lesions concerning for disease progression. He died 1 year after the initial diagnosis secondary to the disease.
Patient 2—A 79-year-old White man presented for further management of an outside diagnosis of superficially invasive primary EMPD of the bilateral inguinal folds and scrotum that had been present for 5 months prior to biopsy and diagnosis. Clinical examination at initial presentation revealed erythematous patches of the bilateral inguinal folds and scrotum, as well as an erythematous scaling plaque in the right axilla. There was no palpable clinical lymphadenopathy. Biopsy of the axilla and groin were both consistent with invasive EMPD with positive staining for CK7 and negative staining for CK20 and CDX2. Workup for underlying adenocarcinoma with whole-body positron emission tomography/CT, mammography, esophagogastroduodenoscopy, serum CEA, colonoscopy, and cystoscopy were all negative for a metastatic adenocarcinoma. There was no imaging or clinical evidence of lymphadenopathy. Complete circumferential peripheral and deep-margin assessment was performed in a staged manner on both sites, and negative margins were obtained.
Surveillance imaging 6 months after surgery revealed suspicious hepatic lesions. Fine-needle aspiration of the hepatic lesions demonstrated positive staining for CK7 and negative staining for CK20, CDX2, prostate-specific antigen, and thyroid transcription factor 1, consistent with metastatic EMPD. Oncology recommended carboplatin and docetaxel or docetaxel monotherapy chemotherapy. The patient was further managed by an outside oncologist due to ease of travel but died secondary to the disease 15 months following the initial diagnosis.
Comment
Extramammary Paget disease is an uncommon cutaneous malignancy that manifests as pruritic erythematous plaques within apocrine-rich areas such as the genitalia, axillae, or anal region. It most commonly occurs in patients older than 65 years, with White women and Asian men being affected at disproportionately higher rates.1,4 Delay in diagnosis is common, as EMPD can mimic other benign inflammatory or infectious conditions, including contact dermatitis, seborrheic dermatitis, tinea, candidiasis, and eczema.1
Metastatic and multifocal cases of primary EMPD are especially rare. According to a search of PubMed articles indexed for MEDLINE published through December 2023 using the terms extramammary Paget disease, EMPD, neoplasm metastasis, invasive extramammary, and neoplasm invasiveness, we identified 5040 cases of invasive EMPD and 477 cases of metastatic EMPD.5-37 Of the reports that disclosed patient demographic information, 3627 patients were female 1410 were male, and the mean age was 67 years. Sites of metastases included regional lymph nodes, liver, lungs, cervix, bladder, bone, brain, skin, kidney, and adrenal glands
Workup for EMPD—The initial steps for workup of EMPD include a thorough physical examination and lymph node assessment. A skin biopsy also should be performed for patients presenting with refractory, pruritic, and eczematous rashes in apocrine-rich areas to evaluate for EMPD.1 Characterization of large and complex tumors is better achieved through multiple biopsies with particular focus on nodular or thickened areas, as these may indicate invasive disease.2 Primary EMPD is characterized by pagetoid cells with abundant pale cytoplasm proliferating in a single-cell or nested pattern within the epidermis or dermis in invasive disease and often is accompanied by dermal lymphocytic inflammation.1 Immunohistochemistry demonstrates positive staining for CEA, CK7, and CK8, with negative staining for indicators of secondary EMPD including CK20 and CDX2.1,2
As part of the workup, it is critical to distinguish between primary disease and secondary EMPD.1 Beyond skin and clinical lymph node examination, additional workup should be based on age-appropriate and location-directed malignant neoplasm screenings, including colonoscopy, cystoscopy, prostate examination, mammography, and Papanicolaou test. Advanced imaging such as CT, positron emission tomography, or magnetic resonance imaging can be used to assess for metastatic disease if internal malignant neoplasms are present on initial screening or clinical lymphadenopathy is identified.2 Additionally, it can be helpful in the evaluation for nodal disease in cases of invasive EMPD.
The likelihood of associated underlying carcinomas varies depending on the site of involvement.38,39 For example, vulvar involvement constitutes approximately 65% of EMPD cases, with 11% to 20% of cases being associated with underlying gastrointestinal or genitourinary carcinomas. Involvement of the male genitalia, as in our 2 patients, is rare, accounting for approximately 14% of cases, 11% of which are associated with prostate, testicular, and bladder carcinoma. Perianal involvement comprises 20% of EMPD cases and has the greatest risk for underlying malignancy with an incidence of 33% to 86%, the majority of which are rectal or tubo-ovarian cancers.38,39 Consideration of the frequency and types of underlying carcinoma of respective sites of involvement can be helpful when ruling out secondary EMPD.
In both of our patients, palpable lymphadenopathy at the time of original diagnosis and histologic invasive disease on initial biopsy warranted thorough imaging and laboratory workup; there was no evidence of primary malignancy. Given the absence of an underlying carcinoma, both patients were classified as having metastatic primary EMPD.
Assessment of lymphadenopathy is an essential aspect of disease workup, as it is associated with a statistically higher rate of lymph node metastases. A study by Fujisawa et al20 demonstrated that 80% of patients with lymphadenopathy had regional metastases compared to only 15% of patients without clinical lymphadenopathy. The presence of invasive disease also has been shown to correspond with lymph node metastases.40 Ogata et al40 showed that 0% of cases with in situ EMPD had a positive sentinel lymph node biopsy (SLNB) compared to 4% and 43% in cases that showed evidence of microinvasion and dermal invasion, respectively. Lymph node metastases are associated with poor prognosis, with increasingly worse prognosis when there are multiple lymph nodes affected.41 In our case series, patient 1 had lymphadenopathy and both patients had invasive EMPD; they both later developed metastases and died.
Lymphadenopathy should be further investigated with imaging and biopsy or fine-needle aspiration.42 Recent expert consensus guidelines recommended this method of investigation over routine use of SLNB, as there is no evidence that a positive SLNB affects treatment that changes disease-specific survival.2
Treatment of EMPD—Surgical excision of the primary lesion is the first-line treatment of EMPD,1,2 which can be performed by wide local excision; however, studies have demonstrated higher recurrence-free survival with margin-controlled surgery (complete circumferential peripheral and deep margin assessment) or Mohs micrographic surgery (MMS), especially with CK7 immunostaining.2,37,43 The literature on MMS of invasive EMPD is sparse, accounting for 57 patients.25,37,44 Other reports describe management with surgical excision, wide local excision, regional resection, or vulvectomy, in addition to lymph node dissection, radiation therapy (RT), and/or chemotherapy.1-36,39,43-46 Despite the improved outcomes with MMS, the predominance of other surgical approaches in our search suggests that MMS may be currently underutilized for the treatment of invasive or locally advanced EMPD.
Among patients with unresectable disease or distant metastases, management includes RT with curative intent, chemotherapy, or a combination of both.1,2 In our review, 267 cases were treated using RT and 77 with chemotherapy. Radiation therapy is an effective therapeutic option with a reported response rate of 62% to 100% and can be employed as either primary or adjuvant treatment.3 For patients with lymph node metastasis the combination of RT and lymph node dissection has been shown to have improved outcomes compared to lymph node dissection alone, with 1 study showing a 5-year survival of 75% for patients who received adjuvant RT compared to 0% for lymph node dissection alone.45
There are currently no consensus guidelines on the best chemotherapeutic regimen for metastatic EMPD. Several regimens have been reported, including docetaxel monotherapy; low-dose 5-fluorouracil and cisplatin; combination chemotherapy FECOM (5-fluorouracil, epirubicin, carboplatin, vincristine, mitomycin); or combination therapy with cisplatin, epirubicin, and paclitaxel.1
Prognosis of Metastatic EMPD—Because invasive and metastatic EMPD is rare, its natural history is hard to predict. Poor prognosis is associated with nodule formation, tumor thickness, perianal or vaginal involvement, lymphovascular invasion, nodal metastasis, and distant metastasis. The 5-year survival for metastatic EMPD has been reported to be less than 10%.46 Our cases underscore the poor prognostic risk associated with metastatic EMPD.
For all cases of EMPD, close follow-up is warranted. Guidelines recommend physical examination with lymph node assessment every 3 to 6 months for 3 years and every 6 to 12 months for the subsequent 5 years.2 Specific recommendations for follow-up in invasive disease have not yet been described, though the 20% probability of developing an internal malignancy within 5 years after diagnosis and poor prognostic outcomes associated with invasive and metastatic disease support the need for close monitoring.2
Conclusion
Although in situ EMPD often is a slow-growing tumor with good prognosis, invasive disease has high potential to behave aggressively with high morbidity and mortality. Increased awareness and prompt identification of invasive EMPD, expedited comprehensive workup, and early multidisciplinary management might impact patient outcomes.
Acknowledgment—The authors would like to thank Ellen Aaronson, MLIS, AHIP (Mayo Clinic Libraries [Jacksonville, FL]), for creating and conducting the narrative literature search in the MEDLINE database.
- Hashimoto H, Ito T. Current management and treatment of extramammary Paget’s disease. Curr Treat Options Oncol. 2022;23:818-830. doi:10.1007/s11864-021-00923-3
- Kibbi N, Owen JL, Worley B, et al. Evidence-based clinical practice guidelines for extramammary Paget disease. JAMA Oncol. 2022;8:618-628. doi:10.1001/jamaoncol.2021.7148
- Morris CR, Hurst EA. Extramammary Paget’s disease: a review of the literature part II: treatment and prognosis. Dermatol Surg. 2020;46:305-311. doi:10.1097/DSS.0000000000002240
- Merritt BG, Degesys CA, Brodland DG. Extramammary Paget disease. Dermatol Clin. 2019;37:261-267. doi:10.1016/j.det.2019.02.002
- Aroche Gutierrez LL, Holloway SB, Donthi D, et al. Docetaxel treatment for widely metastatic invasive vulvar extramammary Paget’s disease with multifocal bone metastasis. Gynecol Oncol Rep. 2022;45:101114. doi:10.1016/j.gore.2022.101114
- Ueda M, Omori M, Sakai A. Invasive extramammary Paget’s disease with lymph node metastases and high-grade B-cell lymphoma. An Bras Dermatol. 2023;98:414-418. doi:10.1016/j.abd.2022.04.012
- Rathore R, Yadav D, Agarwal S, et al. Primary extra mammary Paget’s disease of vulva, with apocrine adenocarcinoma, signet ring cell differentiation and distant metastasis. J Family Reprod Health. 2020;14:276-280. doi:10.18502/jfrh.v14i4.5213
- Kawahara Y, Umeda Y, Yamaguchi B, et al. Long-term resolution of invasive extramammary Paget’s disease with multiple regional lymph node metastases solely with regional lymph node dissection. J Dermatol. 2021;48:E452-E453. doi:10.1111/1346-8138.16007
- Hanyu T, Fujitani S, Ito A, et al. Brain metastasis from extramammary Paget’s disease. Nagoya J Med Sci. 2020;82:791-798. doi:10.18999/nagjms.82.4.791
- Waki Y, Nobeyama Y, Ogawa T, et al. Case of extramammary Paget’s disease causing pulmonary tumor embolism. J Dermatol. 2020;47:E133-E134. doi:10.1111/1346-8138.15267
- Li ZG, Qin XJ. Extensive invasive extramammary Paget disease evaluated by F-18 FDG PET/CT: a case report. Medicine (Baltimore). 2015;94:E371. doi:10.1097/MD.0000000000000371
- Kato N, Matsue K, Sotodate A, et al. Extramammary Paget’s disease with distant skin metastasis. J Dermatol. 1996;23:408-414. doi:10.1111/j.1346-8138.1996.tb04043.x
- Hosomi M, Miyake O, Matsumiya K, et al. Extramammary Paget’s disease with a large mass in male genitalia: a case report. Article in Japanese. Hinyokika Kiyo. 1989;35:1981-1984.
- Hardy LE, Baxter L, Wan K, et al. Invasive cervical adenocarcinoma arising from extension of recurrent vulval Paget’s disease. BMJ Case Rep. 2020;13e232424. doi:10.1136/bcr-2019-232424
- Onaiwu CO, Ramirez PT, Kamat A, et al. Invasive extramammary Paget’s disease of the bladder diagnosed 18 years after noninvasive extramammary Paget’s disease of the vulva. Gynecol Oncol Case Rep. 2014;8:27-29. doi:10.1016/j.gynor.2014.03.004
- Yao H, Xie M, Fu S, et al. Survival analysis of patients with invasive extramammary Paget disease: implications of anatomic sites. BMC Cancer. 2018;18:403. doi:10.1186/s12885-018-4257-1
- Kato H, Watanabe S, Kariya K, et al. Efficacy of low-dose 5-fluorouracil/cisplatin therapy for invasive extramammary Paget’s disease. J Dermatol. 2018;45:560-563. doi:10.1111/1346-8138.14247
- Yoshino K, Fujisawa Y, Kiyohara Y, et al. Usefulness of docetaxel as first-line chemotherapy for metastatic extramammary Paget’s disease. J Dermatol. 2016;43:633-637. doi:10.1111/1346-8138.13200
- Shu B, Shen XX, Chen P, et al. Primary invasive extramammary Paget disease on penoscrotum: a clinicopathological analysis of 41 cases. Hum Pathol. 2016;47:70-77. doi:10.1016/j.humpath.2015.09.005References
- Fujisawa Y, Yoshino K, Kiyohara Y, et al. The role of sentinel lymph node biopsy in the management of invasive extramammary Paget’s disease: multi-center, retrospective study of 151 patients. J Dermatol Sci. 2015;79:38-42. doi:10.1016/j.jdermsci.2015.03.014
- Dai B, Kong YY, Chang K, et al. Primary invasive carcinoma associated with penoscrotal extramammary Paget’s disease: a clinicopathological analysis of 56 cases. BJU Int. 2015;115:153-160. doi:10.1111/bju.12776
- Shiomi T, Noguchi T, Nakayama H, et al. Clinicopathological study of invasive extramammary Paget’s disease: subgroup comparison according to invasion depth. J Eur Acad Dermatol Venereol. 2013;27:589-592. doi:10.1111/j.1468-3083.2012.04489.x
- Hatta N, Morita R, Yamada M, et al. Sentinel lymph node biopsy in patients with extramammary Paget’s disease. Dermatol Surg. 2004;30:1329-1334. doi:10.1111/j.1524-4725.2004.30377.x
- Karam A, Dorigo O. Treatment outcomes in a large cohort of patients with invasive extramammary Paget’s disease. Gynecol Oncol. 2012;125:346-351. doi:10.1016/j.ygyno.2012.01.032
- Guo L, Liu X, Li H, et al. Clinicopathological features of extramammary Paget’s disease: a report of 75 cases. Article in Chinese. Zhonghua Yi Xue Za Zhi. 2015;95:1751-1754.
- Kilts TP, Long B, Glasgow AE, et al. Invasive vulvar extramammary Paget’s disease in the United States. Gynecol Oncol. 2020;157:649-655. doi:10.1016/j.ygyno.2020.03.018
- Kusatake K, Harada Y, Mizumoto K, et al. Usefulness of sentinel lymph node biopsy for the detection of metastasis in the early stage of extramammary Paget’s disease. Eur J Dermatol. 2015;25:156-161. doi:10.1684/ejd.2015.2534
- Jeong BK, Kim KR. Invasive extramammary Paget disease of the vulva with signet ring cell morphology in a patient with signet ring cell carcinoma of the stomach: report of a case. Int J Gynecol Pathol. 2018;37:147-151. doi:10.1097/PGP.0000000000000405
- Pagnanelli M, De Nardi P, Martella S, et al. Local excision of a mucinous adenocarcinoma of the anal margin (extramammary Paget’s disease) and reconstruction with a bilateral V-Y flap. Case Rep Surg. 2019;2019:9073982. doi:10.1155/2019/9073982
- Sopracordevole F, Di Giuseppe J, De Piero G, et al. Surgical treatment of Paget disease of the vulva: prognostic significance of stromal invasion and surgical margin status. J Low Genit Tract Dis. 2016;20:184-188. doi:10.1097/LGT.0000000000000191
- Evans AT, Neven P. Invasive adenocarcinoma arising in extramammary Paget’s disease of the vulva. Histopathology. 1991;18:355-360. doi:10.1111/j.1365-2559.1991.tb00857.x
- Kitano A, Izumi M, Tamura K, et al. Brain metastasis from cutaneous squamous cell carcinoma coexistent with extramammary Paget’s disease: a case report. Pathol Int. 2019;69:619-625. doi:10.1111/pin.12846
- Miracco C, Francini E, Torre P, et al. Extramammary invasive Paget’s disease and apocrine angiomatous hamartoma: an unusual association. Eur J Dermatol. 2018;28:853-855. doi:10.1684/ejd.2018.3438
- Kambayashi Y, Fujimura T, Ohuchi K, et al. Advanced invasive extramammary Paget’s disease concomitant with cecal cancer possessing rare variant of TP53 single nucleotide polymorphism. Case Rep Oncol. 2019;12:855-860. doi:10.1159/000504339
- Fujimura T, Furudate S, Kambayashi Y, et al. Potential use of bisphosphonates in invasive extramammary Paget’s disease: an immunohistochemical investigation. Clin Dev Immunol. 2013;2013:164982. doi:10.1155/2013/164982
- Kawamura H, Ogata K, Miura H, et al. Patellar metastases. A report of two cases. Int Orthop. 1993;17:57-59. doi:10.1007/BF00195227
- Damavandy AA, Terushkin V, Zitelli JA, et al. Intraoperative immunostaining for cytokeratin-7 during Mohs micrographic surgery demonstrates low local recurrence rates in extramammary Paget’s disease. Dermatol Surg. 2018;44:354-364. doi:10.1097/DSS.0000000000001355
- Morris CR, Hurst EA. Extramammary Paget disease: a review of the literature-part I: history, epidemiology, pathogenesis, presentation, histopathology, and diagnostic work-up. Dermatol Surg. 2020;46:151-158. doi:10.1097/DSS.0000000000002064
- Simonds RM, Segal RJ, Sharma A. Extramammary Paget’s disease: a review of the literature. Int J Dermatol. 2019;58:871-879. doi:10.1111/ijd.14328
- Ogata D, Kiyohara Y, Yoshikawa S, et al. Usefulness of sentinel lymph node biopsy for prognostic prediction in extramammary Paget’s disease. Eur J Dermatol. 2016;26:254-259. doi:10.1684/ejd.2016.2744
- Ohara K, Fujisawa Y, Yoshino K, et al. A proposal for a TNM staging system for extramammary Paget disease: retrospective analysis of 301 patients with invasive primary tumors. J Dermatol Sci. 2016;83:234-239. doi:10.1016/j.jdermsci.2016.06.004
- Fujisawa Y, Yoshino K, Kiyohara Y, et al. The role of sentinel lymph node biopsy in the management of invasive extramammary Paget’s disease: multi-center, retrospective study of 151 patients. J Dermatol Sci. 2015;79:38-42. doi:10.1016/j.jdermsci.2015.03.014
- Kim SJ, Thompson AK, Zubair AS, et al. Surgical treatment and outcomes of patients with extramammary Paget disease: a cohort study. Dermatol Surg. 2017;43:708-714. doi:10.1097/DSS.0000000000001051
- Wollina U. Extensive invasive extramammary Paget’s disease: surgical treatment. J Cutan Aesthet Surg. 2013;6:41-44. doi:10.4103/0974-2077.110098
- Tsutsui K, Takahashi A, Muto Y, et al. Outcomes of lymph node dissection in the treatment of extramammary Paget’s disease: a single-institution study. J Dermatol. 2020;47:512-517. doi:10.1111/1346-8138.15285
- Guercio BJ, Iyer G, Kidwai WZ, et al. Treatment of metastatic extramammary Paget disease with combination ipilimumab and nivolumab: a case report. Case Rep Oncol. 2021;14:430-438. doi:10.1159/000514345
- Hashimoto H, Ito T. Current management and treatment of extramammary Paget’s disease. Curr Treat Options Oncol. 2022;23:818-830. doi:10.1007/s11864-021-00923-3
- Kibbi N, Owen JL, Worley B, et al. Evidence-based clinical practice guidelines for extramammary Paget disease. JAMA Oncol. 2022;8:618-628. doi:10.1001/jamaoncol.2021.7148
- Morris CR, Hurst EA. Extramammary Paget’s disease: a review of the literature part II: treatment and prognosis. Dermatol Surg. 2020;46:305-311. doi:10.1097/DSS.0000000000002240
- Merritt BG, Degesys CA, Brodland DG. Extramammary Paget disease. Dermatol Clin. 2019;37:261-267. doi:10.1016/j.det.2019.02.002
- Aroche Gutierrez LL, Holloway SB, Donthi D, et al. Docetaxel treatment for widely metastatic invasive vulvar extramammary Paget’s disease with multifocal bone metastasis. Gynecol Oncol Rep. 2022;45:101114. doi:10.1016/j.gore.2022.101114
- Ueda M, Omori M, Sakai A. Invasive extramammary Paget’s disease with lymph node metastases and high-grade B-cell lymphoma. An Bras Dermatol. 2023;98:414-418. doi:10.1016/j.abd.2022.04.012
- Rathore R, Yadav D, Agarwal S, et al. Primary extra mammary Paget’s disease of vulva, with apocrine adenocarcinoma, signet ring cell differentiation and distant metastasis. J Family Reprod Health. 2020;14:276-280. doi:10.18502/jfrh.v14i4.5213
- Kawahara Y, Umeda Y, Yamaguchi B, et al. Long-term resolution of invasive extramammary Paget’s disease with multiple regional lymph node metastases solely with regional lymph node dissection. J Dermatol. 2021;48:E452-E453. doi:10.1111/1346-8138.16007
- Hanyu T, Fujitani S, Ito A, et al. Brain metastasis from extramammary Paget’s disease. Nagoya J Med Sci. 2020;82:791-798. doi:10.18999/nagjms.82.4.791
- Waki Y, Nobeyama Y, Ogawa T, et al. Case of extramammary Paget’s disease causing pulmonary tumor embolism. J Dermatol. 2020;47:E133-E134. doi:10.1111/1346-8138.15267
- Li ZG, Qin XJ. Extensive invasive extramammary Paget disease evaluated by F-18 FDG PET/CT: a case report. Medicine (Baltimore). 2015;94:E371. doi:10.1097/MD.0000000000000371
- Kato N, Matsue K, Sotodate A, et al. Extramammary Paget’s disease with distant skin metastasis. J Dermatol. 1996;23:408-414. doi:10.1111/j.1346-8138.1996.tb04043.x
- Hosomi M, Miyake O, Matsumiya K, et al. Extramammary Paget’s disease with a large mass in male genitalia: a case report. Article in Japanese. Hinyokika Kiyo. 1989;35:1981-1984.
- Hardy LE, Baxter L, Wan K, et al. Invasive cervical adenocarcinoma arising from extension of recurrent vulval Paget’s disease. BMJ Case Rep. 2020;13e232424. doi:10.1136/bcr-2019-232424
- Onaiwu CO, Ramirez PT, Kamat A, et al. Invasive extramammary Paget’s disease of the bladder diagnosed 18 years after noninvasive extramammary Paget’s disease of the vulva. Gynecol Oncol Case Rep. 2014;8:27-29. doi:10.1016/j.gynor.2014.03.004
- Yao H, Xie M, Fu S, et al. Survival analysis of patients with invasive extramammary Paget disease: implications of anatomic sites. BMC Cancer. 2018;18:403. doi:10.1186/s12885-018-4257-1
- Kato H, Watanabe S, Kariya K, et al. Efficacy of low-dose 5-fluorouracil/cisplatin therapy for invasive extramammary Paget’s disease. J Dermatol. 2018;45:560-563. doi:10.1111/1346-8138.14247
- Yoshino K, Fujisawa Y, Kiyohara Y, et al. Usefulness of docetaxel as first-line chemotherapy for metastatic extramammary Paget’s disease. J Dermatol. 2016;43:633-637. doi:10.1111/1346-8138.13200
- Shu B, Shen XX, Chen P, et al. Primary invasive extramammary Paget disease on penoscrotum: a clinicopathological analysis of 41 cases. Hum Pathol. 2016;47:70-77. doi:10.1016/j.humpath.2015.09.005References
- Fujisawa Y, Yoshino K, Kiyohara Y, et al. The role of sentinel lymph node biopsy in the management of invasive extramammary Paget’s disease: multi-center, retrospective study of 151 patients. J Dermatol Sci. 2015;79:38-42. doi:10.1016/j.jdermsci.2015.03.014
- Dai B, Kong YY, Chang K, et al. Primary invasive carcinoma associated with penoscrotal extramammary Paget’s disease: a clinicopathological analysis of 56 cases. BJU Int. 2015;115:153-160. doi:10.1111/bju.12776
- Shiomi T, Noguchi T, Nakayama H, et al. Clinicopathological study of invasive extramammary Paget’s disease: subgroup comparison according to invasion depth. J Eur Acad Dermatol Venereol. 2013;27:589-592. doi:10.1111/j.1468-3083.2012.04489.x
- Hatta N, Morita R, Yamada M, et al. Sentinel lymph node biopsy in patients with extramammary Paget’s disease. Dermatol Surg. 2004;30:1329-1334. doi:10.1111/j.1524-4725.2004.30377.x
- Karam A, Dorigo O. Treatment outcomes in a large cohort of patients with invasive extramammary Paget’s disease. Gynecol Oncol. 2012;125:346-351. doi:10.1016/j.ygyno.2012.01.032
- Guo L, Liu X, Li H, et al. Clinicopathological features of extramammary Paget’s disease: a report of 75 cases. Article in Chinese. Zhonghua Yi Xue Za Zhi. 2015;95:1751-1754.
- Kilts TP, Long B, Glasgow AE, et al. Invasive vulvar extramammary Paget’s disease in the United States. Gynecol Oncol. 2020;157:649-655. doi:10.1016/j.ygyno.2020.03.018
- Kusatake K, Harada Y, Mizumoto K, et al. Usefulness of sentinel lymph node biopsy for the detection of metastasis in the early stage of extramammary Paget’s disease. Eur J Dermatol. 2015;25:156-161. doi:10.1684/ejd.2015.2534
- Jeong BK, Kim KR. Invasive extramammary Paget disease of the vulva with signet ring cell morphology in a patient with signet ring cell carcinoma of the stomach: report of a case. Int J Gynecol Pathol. 2018;37:147-151. doi:10.1097/PGP.0000000000000405
- Pagnanelli M, De Nardi P, Martella S, et al. Local excision of a mucinous adenocarcinoma of the anal margin (extramammary Paget’s disease) and reconstruction with a bilateral V-Y flap. Case Rep Surg. 2019;2019:9073982. doi:10.1155/2019/9073982
- Sopracordevole F, Di Giuseppe J, De Piero G, et al. Surgical treatment of Paget disease of the vulva: prognostic significance of stromal invasion and surgical margin status. J Low Genit Tract Dis. 2016;20:184-188. doi:10.1097/LGT.0000000000000191
- Evans AT, Neven P. Invasive adenocarcinoma arising in extramammary Paget’s disease of the vulva. Histopathology. 1991;18:355-360. doi:10.1111/j.1365-2559.1991.tb00857.x
- Kitano A, Izumi M, Tamura K, et al. Brain metastasis from cutaneous squamous cell carcinoma coexistent with extramammary Paget’s disease: a case report. Pathol Int. 2019;69:619-625. doi:10.1111/pin.12846
- Miracco C, Francini E, Torre P, et al. Extramammary invasive Paget’s disease and apocrine angiomatous hamartoma: an unusual association. Eur J Dermatol. 2018;28:853-855. doi:10.1684/ejd.2018.3438
- Kambayashi Y, Fujimura T, Ohuchi K, et al. Advanced invasive extramammary Paget’s disease concomitant with cecal cancer possessing rare variant of TP53 single nucleotide polymorphism. Case Rep Oncol. 2019;12:855-860. doi:10.1159/000504339
- Fujimura T, Furudate S, Kambayashi Y, et al. Potential use of bisphosphonates in invasive extramammary Paget’s disease: an immunohistochemical investigation. Clin Dev Immunol. 2013;2013:164982. doi:10.1155/2013/164982
- Kawamura H, Ogata K, Miura H, et al. Patellar metastases. A report of two cases. Int Orthop. 1993;17:57-59. doi:10.1007/BF00195227
- Damavandy AA, Terushkin V, Zitelli JA, et al. Intraoperative immunostaining for cytokeratin-7 during Mohs micrographic surgery demonstrates low local recurrence rates in extramammary Paget’s disease. Dermatol Surg. 2018;44:354-364. doi:10.1097/DSS.0000000000001355
- Morris CR, Hurst EA. Extramammary Paget disease: a review of the literature-part I: history, epidemiology, pathogenesis, presentation, histopathology, and diagnostic work-up. Dermatol Surg. 2020;46:151-158. doi:10.1097/DSS.0000000000002064
- Simonds RM, Segal RJ, Sharma A. Extramammary Paget’s disease: a review of the literature. Int J Dermatol. 2019;58:871-879. doi:10.1111/ijd.14328
- Ogata D, Kiyohara Y, Yoshikawa S, et al. Usefulness of sentinel lymph node biopsy for prognostic prediction in extramammary Paget’s disease. Eur J Dermatol. 2016;26:254-259. doi:10.1684/ejd.2016.2744
- Ohara K, Fujisawa Y, Yoshino K, et al. A proposal for a TNM staging system for extramammary Paget disease: retrospective analysis of 301 patients with invasive primary tumors. J Dermatol Sci. 2016;83:234-239. doi:10.1016/j.jdermsci.2016.06.004
- Fujisawa Y, Yoshino K, Kiyohara Y, et al. The role of sentinel lymph node biopsy in the management of invasive extramammary Paget’s disease: multi-center, retrospective study of 151 patients. J Dermatol Sci. 2015;79:38-42. doi:10.1016/j.jdermsci.2015.03.014
- Kim SJ, Thompson AK, Zubair AS, et al. Surgical treatment and outcomes of patients with extramammary Paget disease: a cohort study. Dermatol Surg. 2017;43:708-714. doi:10.1097/DSS.0000000000001051
- Wollina U. Extensive invasive extramammary Paget’s disease: surgical treatment. J Cutan Aesthet Surg. 2013;6:41-44. doi:10.4103/0974-2077.110098
- Tsutsui K, Takahashi A, Muto Y, et al. Outcomes of lymph node dissection in the treatment of extramammary Paget’s disease: a single-institution study. J Dermatol. 2020;47:512-517. doi:10.1111/1346-8138.15285
- Guercio BJ, Iyer G, Kidwai WZ, et al. Treatment of metastatic extramammary Paget disease with combination ipilimumab and nivolumab: a case report. Case Rep Oncol. 2021;14:430-438. doi:10.1159/000514345
Metastatic Primary Extramammary Paget Disease: A Case Series
Metastatic Primary Extramammary Paget Disease: A Case Series
Practice Points
- Invasive primary extramammary Paget disease has a higher risk for lymph node metastasis.
- Consider extramammary Paget disease in patients presenting with erythematous pruritic plaques in apocrine-rich areas that fail to respond to topical steroids or antifungals.
- Prompt diagnosis can expedite comprehensive malignancy work-up and multidisciplinary management, potentially impacting patient outcomes.
Best Practices for Clinical Image Collection and Utilization in Patients With Skin of Color
Clinical images are integral to dermatologic care, research, and education. Studies have highlighted the underrepresentation of images of skin of color (SOC) in educational materials,1 clinical trials,2 and research publications.3 Recognition of this disparity has ignited a call to action by dermatologists and dermatologic organizations to address the gap by improving the collection and use of SOC images.4 It is critical to remind dermatologists of the importance of properly obtaining informed consent and ensuring images are not used without a patient’s permission, as images in journal articles, conference presentations, and educational materials can be widely distributed and shared. Herein, we summarize current practices of clinical image storage and make general recommendations on how dermatologists can better protect patient privacy. Certain cultural and social factors in patients with SOC should be considered when obtaining informed consent and collecting images.
Clinical Image Acquisition
Consenting procedures are crucial components of proper image usage. However, current consenting practices are inconsistent across various platforms, including academic journals, websites, printed text, social media, and educational presentations.5
Current regulations for use of patient health information in the United States are governed by the Health Insurance Portability and Accountability Act (HIPAA)of 1996. Although this act explicitly prohibits use of “full face photographic images and any comparable images” without consent from the patient or the patient’s representative, there is less restriction regarding the use of deidentified images.6 Some clinicians or researchers may consider using a black bar or a masking technique over the eyes or face, but this is not always a sufficient method of anonymizing an image.
One study investigating the different requirements listed by the top 20 dermatology journals (as determined by the Google Scholar h5-index) found that while 95% (19/20) of journals stated that written or signed consent or permission was a requirement for use of patient images, only 20% (4/20) instructed authors to inform the patient or the patient’s representative that images may become available on the internet.5 Once an article is accepted for publication by a medical journal, it eventually may be accessible online; however, patients may not be aware of this factor, which is particularly concerning for those with SOC due to the increased demand for diverse dermatologic resources and images as well as the highly digitalized manner in which we access and share media.
Furthermore, cultural and social factors exist that present challenges to informed decision-making during the consenting process for certain SOC populations such as a lack of trust in the medical and scientific research community, inadequate comprehension of the consent material, health illiteracy, language barriers, or use of complex terminology in consent documentation.7,8 Studies also have shown that patients in ethnic minority groups have greater barriers to health literacy compared to other patient groups, and patients with limited health literacy are less likely to ask questions during their medical visits.9,10 Therefore, when obtaining informed consent for images, it is important that measures are taken to ensure that the patient has full knowledge and understanding of what the consent covers, including the extent to which the images will be used and/or shared and whether the patient’s confidentiality and/or anonymity are at risk.
Recommendations—We propose that dermatologists should follow these recommendations:
1. Encourage influential dermatology organizations such as the American Academy of Dermatology to establish standardized consenting procedures for image acquisition and use, including requirements to provide (a) written consent for all patient images and (b) specific details as to where and how the image may be used and/or shared.
2. Ensure that consent terminology is presented at a sixth-grade reading level or below, minimize the use of medical jargon and complex terms, and provide consent documentation in the patient’s preferred language.
3. Allow patients to take the consent document home so they can have additional time to comprehensively review the material or have it reviewed by family or friends.
4. Employ strategies such as teach-back methods and encourage questions to maximize the level of understanding during the consent process.
Clinical Image Storage
Clinical image storage procedures can have an impact on a patient’s health information remaining anonymous and confidential. In a survey evaluating medical photography use among 153 US board-certified dermatologists, 69.1% of respondents reported emailing or texting images between patients and colleagues. Additionally, 30.3% (46/152) reported having patient photographs stored on their personal phone at the time of the survey, and 39.1% (18/46) of those individuals had images that showed identifiable features, such as the patient’s face or a tattoo.11
Although most providers state that their devices are password protected, it cannot be guaranteed that the device and consequently the images remain secure and inaccessible to unauthorized individuals. As sharing and viewing images continue to play an essential role in assessing disease state, progression, treatment response, and inclusion in research, we must establish and encourage clear guidelines for the storage and retention of such images.
Recommendations—We propose that dermatologists should follow these recommendations:
1. Store clinical images exclusively on password-protected devices and in password-protected files.
2. Use work-related cameras or electronic devices rather than personal devices, unless the personal device is being used to upload directly into the patient’s medical record. In such cases, use a HIPAA-compliant electronic medical record mobile application that does not store images on the application or the device itself.
3. Avoid using text-messaging systems or unencrypted email to share identifying images without clear patient consent.
Clinical Image Use
Once a thorough consenting process has been completed, it is crucial that the use and distribution of the clinical image are in accordance with the terms specified in the original consent. With the current state of technologic advancement, widespread social media usage, and constant sharing of information, adherence to these terms can be challenging. For example, an image initially intended for use in an educational presentation at a professional conference can be shared on social media if an audience member captures a photo of it. In another example, a patient may consent to their image being shown on a dermatologic website but that image can be duplicated and shared on other unauthorized sites and locations. This situation can be particularly distressing to patients whose image may include all or most of their face, an intimate area, or other physical features that they did not wish to share widely.
Individuals identifying as Black/African American, Latino/Hispanic, or Asian have been shown to express less comfort with providing permission for images of a nonidentifiable sensitive area to be taken (or obtained) or for use for teaching irrespective of identifiability compared to their White counterparts,12 which may be due to the aforementioned lack of trust in medical providers and the health care system in general, both of which may contribute to concerns with how a clinical image is used and/or shared. Although consent from a patient or the patient’s representative can be granted, we must ensure that the use of these images adheres to the patient’s initial agreement. Ultimately, medical providers, researchers, and other parties involved in acquiring or sharing patient images have both an ethical and legal responsibility to ensure that anonymity, privacy, and confidentiality are preserved to the greatest extent possible.
Recommendations—We propose that dermatologists should follow these recommendations:
1. Display a message on websites containing patient images stating that the sharing of the images outside the established guidelines and intended use is prohibited.
2. Place a watermark on images to discourage unauthorized duplication.
3. Issue explicit instructions to audiences prohibiting the copying or reproducing of any patient images during teaching events or presentations.
Final Thoughts
The use of clinical images is an essential component of dermatologic care, education, and research. Due to the higher demand for diverse and representative images and the dearth of images in the medical literature, many SOC images have been widely disseminated and utilized by dermatologists, raising concerns of the adequacy of informed consent for the storage and use of such material. Therefore, dermatologists should implement streamlined guidelines and consent procedures to ensure a patient’s informed consent is provided with full knowledge of how and where their images might be used and shared. Additional efforts should be made to protect patients’ privacy and unauthorized use of their images. Furthermore, we encourage our leading dermatology organizations to develop expert consensus on best practices for appropriate clinical image consent, storage, and use.
- Alvarado SM, Feng H. Representation of dark skin images of common dermatologic conditions in educational resources: a cross-sectional analysis [published online June 18, 2020]. J Am Acad Dermatol. 2021;84:1427-1431. doi:10.1016/j.jaad.2020.06.041
- Charrow A, Xia FD, Joyce C, et al. Diversity in dermatology clinical trials: a systematic review. JAMA Dermatol. 2017;153:193-198. doi:10.1001/jamadermatol.2016.4129
- Marroquin NA, Carboni A, Zueger M, et al. Skin of color representation trends in JAAD case reports 2015-2021: content analysis. JMIR Dermatol. 2023;6:e40816. doi:10.2196/40816
- Kim Y, Miller JJ, Hollins LC. Skin of color matters: a call to action. J Am Acad Dermatol. 2021;84:E273-E274. doi:10.1016/j.jaad.2020.11.026
- Nanda JK, Marchetti MA. Consent and deidentification of patient images in dermatology journals: observational study. JMIR Dermatol. 2022;5:E37398. doi:10.2196/37398
- US Department of Health and Human Services. Summary of the HIPAA privacy rule. Updated October 19, 2022. Accessed March 15, 2024. https://www.hhs.gov/hipaa/for-professionals/privacy/laws-regulations/index.html
- Quinn SC, Garza MA, Butler J, et al. Improving informed consent with minority participants: results from researcher and community surveys. J Empir Res Hum Res Ethics. 2012;7:44-55. doi:10.1525/jer.2012.7.5.44
- Hadden KB, Prince LY, Moore TD, et al. Improving readability of informed consents for research at an academic medical institution. J Clin Transl Sci. 2017;1:361-365. doi:10.1017/cts.2017.312
- Muvuka B, Combs RM, Ayangeakaa SD, et al. Health literacy in African-American communities: barriers and strategies. Health Lit Res Pract. 2020;4:E138-E143. doi:10.3928/24748307-20200617-01
- Menendez ME, van Hoorn BT, Mackert M, et al. Patients with limited health literacy ask fewer questions during office visits with hand surgeons. Clin Orthop Relat Res. 2017;475:1291-1297. doi:10.1007/s11999-016-5140-5
- Milam EC, Leger MC. Use of medical photography among dermatologists: a nationwide online survey study. J Eur Acad Dermatol Venereol. 2018;32:1804-1809. doi:10.1111/jdv.14839
- Leger MC, Wu T, Haimovic A, et al. Patient perspectives on medical photography in dermatology. Dermatol Surg. 2014;40:1028-1037. doi:10.1097/01.DSS.0000452632.22081.79
Clinical images are integral to dermatologic care, research, and education. Studies have highlighted the underrepresentation of images of skin of color (SOC) in educational materials,1 clinical trials,2 and research publications.3 Recognition of this disparity has ignited a call to action by dermatologists and dermatologic organizations to address the gap by improving the collection and use of SOC images.4 It is critical to remind dermatologists of the importance of properly obtaining informed consent and ensuring images are not used without a patient’s permission, as images in journal articles, conference presentations, and educational materials can be widely distributed and shared. Herein, we summarize current practices of clinical image storage and make general recommendations on how dermatologists can better protect patient privacy. Certain cultural and social factors in patients with SOC should be considered when obtaining informed consent and collecting images.
Clinical Image Acquisition
Consenting procedures are crucial components of proper image usage. However, current consenting practices are inconsistent across various platforms, including academic journals, websites, printed text, social media, and educational presentations.5
Current regulations for use of patient health information in the United States are governed by the Health Insurance Portability and Accountability Act (HIPAA)of 1996. Although this act explicitly prohibits use of “full face photographic images and any comparable images” without consent from the patient or the patient’s representative, there is less restriction regarding the use of deidentified images.6 Some clinicians or researchers may consider using a black bar or a masking technique over the eyes or face, but this is not always a sufficient method of anonymizing an image.
One study investigating the different requirements listed by the top 20 dermatology journals (as determined by the Google Scholar h5-index) found that while 95% (19/20) of journals stated that written or signed consent or permission was a requirement for use of patient images, only 20% (4/20) instructed authors to inform the patient or the patient’s representative that images may become available on the internet.5 Once an article is accepted for publication by a medical journal, it eventually may be accessible online; however, patients may not be aware of this factor, which is particularly concerning for those with SOC due to the increased demand for diverse dermatologic resources and images as well as the highly digitalized manner in which we access and share media.
Furthermore, cultural and social factors exist that present challenges to informed decision-making during the consenting process for certain SOC populations such as a lack of trust in the medical and scientific research community, inadequate comprehension of the consent material, health illiteracy, language barriers, or use of complex terminology in consent documentation.7,8 Studies also have shown that patients in ethnic minority groups have greater barriers to health literacy compared to other patient groups, and patients with limited health literacy are less likely to ask questions during their medical visits.9,10 Therefore, when obtaining informed consent for images, it is important that measures are taken to ensure that the patient has full knowledge and understanding of what the consent covers, including the extent to which the images will be used and/or shared and whether the patient’s confidentiality and/or anonymity are at risk.
Recommendations—We propose that dermatologists should follow these recommendations:
1. Encourage influential dermatology organizations such as the American Academy of Dermatology to establish standardized consenting procedures for image acquisition and use, including requirements to provide (a) written consent for all patient images and (b) specific details as to where and how the image may be used and/or shared.
2. Ensure that consent terminology is presented at a sixth-grade reading level or below, minimize the use of medical jargon and complex terms, and provide consent documentation in the patient’s preferred language.
3. Allow patients to take the consent document home so they can have additional time to comprehensively review the material or have it reviewed by family or friends.
4. Employ strategies such as teach-back methods and encourage questions to maximize the level of understanding during the consent process.
Clinical Image Storage
Clinical image storage procedures can have an impact on a patient’s health information remaining anonymous and confidential. In a survey evaluating medical photography use among 153 US board-certified dermatologists, 69.1% of respondents reported emailing or texting images between patients and colleagues. Additionally, 30.3% (46/152) reported having patient photographs stored on their personal phone at the time of the survey, and 39.1% (18/46) of those individuals had images that showed identifiable features, such as the patient’s face or a tattoo.11
Although most providers state that their devices are password protected, it cannot be guaranteed that the device and consequently the images remain secure and inaccessible to unauthorized individuals. As sharing and viewing images continue to play an essential role in assessing disease state, progression, treatment response, and inclusion in research, we must establish and encourage clear guidelines for the storage and retention of such images.
Recommendations—We propose that dermatologists should follow these recommendations:
1. Store clinical images exclusively on password-protected devices and in password-protected files.
2. Use work-related cameras or electronic devices rather than personal devices, unless the personal device is being used to upload directly into the patient’s medical record. In such cases, use a HIPAA-compliant electronic medical record mobile application that does not store images on the application or the device itself.
3. Avoid using text-messaging systems or unencrypted email to share identifying images without clear patient consent.
Clinical Image Use
Once a thorough consenting process has been completed, it is crucial that the use and distribution of the clinical image are in accordance with the terms specified in the original consent. With the current state of technologic advancement, widespread social media usage, and constant sharing of information, adherence to these terms can be challenging. For example, an image initially intended for use in an educational presentation at a professional conference can be shared on social media if an audience member captures a photo of it. In another example, a patient may consent to their image being shown on a dermatologic website but that image can be duplicated and shared on other unauthorized sites and locations. This situation can be particularly distressing to patients whose image may include all or most of their face, an intimate area, or other physical features that they did not wish to share widely.
Individuals identifying as Black/African American, Latino/Hispanic, or Asian have been shown to express less comfort with providing permission for images of a nonidentifiable sensitive area to be taken (or obtained) or for use for teaching irrespective of identifiability compared to their White counterparts,12 which may be due to the aforementioned lack of trust in medical providers and the health care system in general, both of which may contribute to concerns with how a clinical image is used and/or shared. Although consent from a patient or the patient’s representative can be granted, we must ensure that the use of these images adheres to the patient’s initial agreement. Ultimately, medical providers, researchers, and other parties involved in acquiring or sharing patient images have both an ethical and legal responsibility to ensure that anonymity, privacy, and confidentiality are preserved to the greatest extent possible.
Recommendations—We propose that dermatologists should follow these recommendations:
1. Display a message on websites containing patient images stating that the sharing of the images outside the established guidelines and intended use is prohibited.
2. Place a watermark on images to discourage unauthorized duplication.
3. Issue explicit instructions to audiences prohibiting the copying or reproducing of any patient images during teaching events or presentations.
Final Thoughts
The use of clinical images is an essential component of dermatologic care, education, and research. Due to the higher demand for diverse and representative images and the dearth of images in the medical literature, many SOC images have been widely disseminated and utilized by dermatologists, raising concerns of the adequacy of informed consent for the storage and use of such material. Therefore, dermatologists should implement streamlined guidelines and consent procedures to ensure a patient’s informed consent is provided with full knowledge of how and where their images might be used and shared. Additional efforts should be made to protect patients’ privacy and unauthorized use of their images. Furthermore, we encourage our leading dermatology organizations to develop expert consensus on best practices for appropriate clinical image consent, storage, and use.
Clinical images are integral to dermatologic care, research, and education. Studies have highlighted the underrepresentation of images of skin of color (SOC) in educational materials,1 clinical trials,2 and research publications.3 Recognition of this disparity has ignited a call to action by dermatologists and dermatologic organizations to address the gap by improving the collection and use of SOC images.4 It is critical to remind dermatologists of the importance of properly obtaining informed consent and ensuring images are not used without a patient’s permission, as images in journal articles, conference presentations, and educational materials can be widely distributed and shared. Herein, we summarize current practices of clinical image storage and make general recommendations on how dermatologists can better protect patient privacy. Certain cultural and social factors in patients with SOC should be considered when obtaining informed consent and collecting images.
Clinical Image Acquisition
Consenting procedures are crucial components of proper image usage. However, current consenting practices are inconsistent across various platforms, including academic journals, websites, printed text, social media, and educational presentations.5
Current regulations for use of patient health information in the United States are governed by the Health Insurance Portability and Accountability Act (HIPAA)of 1996. Although this act explicitly prohibits use of “full face photographic images and any comparable images” without consent from the patient or the patient’s representative, there is less restriction regarding the use of deidentified images.6 Some clinicians or researchers may consider using a black bar or a masking technique over the eyes or face, but this is not always a sufficient method of anonymizing an image.
One study investigating the different requirements listed by the top 20 dermatology journals (as determined by the Google Scholar h5-index) found that while 95% (19/20) of journals stated that written or signed consent or permission was a requirement for use of patient images, only 20% (4/20) instructed authors to inform the patient or the patient’s representative that images may become available on the internet.5 Once an article is accepted for publication by a medical journal, it eventually may be accessible online; however, patients may not be aware of this factor, which is particularly concerning for those with SOC due to the increased demand for diverse dermatologic resources and images as well as the highly digitalized manner in which we access and share media.
Furthermore, cultural and social factors exist that present challenges to informed decision-making during the consenting process for certain SOC populations such as a lack of trust in the medical and scientific research community, inadequate comprehension of the consent material, health illiteracy, language barriers, or use of complex terminology in consent documentation.7,8 Studies also have shown that patients in ethnic minority groups have greater barriers to health literacy compared to other patient groups, and patients with limited health literacy are less likely to ask questions during their medical visits.9,10 Therefore, when obtaining informed consent for images, it is important that measures are taken to ensure that the patient has full knowledge and understanding of what the consent covers, including the extent to which the images will be used and/or shared and whether the patient’s confidentiality and/or anonymity are at risk.
Recommendations—We propose that dermatologists should follow these recommendations:
1. Encourage influential dermatology organizations such as the American Academy of Dermatology to establish standardized consenting procedures for image acquisition and use, including requirements to provide (a) written consent for all patient images and (b) specific details as to where and how the image may be used and/or shared.
2. Ensure that consent terminology is presented at a sixth-grade reading level or below, minimize the use of medical jargon and complex terms, and provide consent documentation in the patient’s preferred language.
3. Allow patients to take the consent document home so they can have additional time to comprehensively review the material or have it reviewed by family or friends.
4. Employ strategies such as teach-back methods and encourage questions to maximize the level of understanding during the consent process.
Clinical Image Storage
Clinical image storage procedures can have an impact on a patient’s health information remaining anonymous and confidential. In a survey evaluating medical photography use among 153 US board-certified dermatologists, 69.1% of respondents reported emailing or texting images between patients and colleagues. Additionally, 30.3% (46/152) reported having patient photographs stored on their personal phone at the time of the survey, and 39.1% (18/46) of those individuals had images that showed identifiable features, such as the patient’s face or a tattoo.11
Although most providers state that their devices are password protected, it cannot be guaranteed that the device and consequently the images remain secure and inaccessible to unauthorized individuals. As sharing and viewing images continue to play an essential role in assessing disease state, progression, treatment response, and inclusion in research, we must establish and encourage clear guidelines for the storage and retention of such images.
Recommendations—We propose that dermatologists should follow these recommendations:
1. Store clinical images exclusively on password-protected devices and in password-protected files.
2. Use work-related cameras or electronic devices rather than personal devices, unless the personal device is being used to upload directly into the patient’s medical record. In such cases, use a HIPAA-compliant electronic medical record mobile application that does not store images on the application or the device itself.
3. Avoid using text-messaging systems or unencrypted email to share identifying images without clear patient consent.
Clinical Image Use
Once a thorough consenting process has been completed, it is crucial that the use and distribution of the clinical image are in accordance with the terms specified in the original consent. With the current state of technologic advancement, widespread social media usage, and constant sharing of information, adherence to these terms can be challenging. For example, an image initially intended for use in an educational presentation at a professional conference can be shared on social media if an audience member captures a photo of it. In another example, a patient may consent to their image being shown on a dermatologic website but that image can be duplicated and shared on other unauthorized sites and locations. This situation can be particularly distressing to patients whose image may include all or most of their face, an intimate area, or other physical features that they did not wish to share widely.
Individuals identifying as Black/African American, Latino/Hispanic, or Asian have been shown to express less comfort with providing permission for images of a nonidentifiable sensitive area to be taken (or obtained) or for use for teaching irrespective of identifiability compared to their White counterparts,12 which may be due to the aforementioned lack of trust in medical providers and the health care system in general, both of which may contribute to concerns with how a clinical image is used and/or shared. Although consent from a patient or the patient’s representative can be granted, we must ensure that the use of these images adheres to the patient’s initial agreement. Ultimately, medical providers, researchers, and other parties involved in acquiring or sharing patient images have both an ethical and legal responsibility to ensure that anonymity, privacy, and confidentiality are preserved to the greatest extent possible.
Recommendations—We propose that dermatologists should follow these recommendations:
1. Display a message on websites containing patient images stating that the sharing of the images outside the established guidelines and intended use is prohibited.
2. Place a watermark on images to discourage unauthorized duplication.
3. Issue explicit instructions to audiences prohibiting the copying or reproducing of any patient images during teaching events or presentations.
Final Thoughts
The use of clinical images is an essential component of dermatologic care, education, and research. Due to the higher demand for diverse and representative images and the dearth of images in the medical literature, many SOC images have been widely disseminated and utilized by dermatologists, raising concerns of the adequacy of informed consent for the storage and use of such material. Therefore, dermatologists should implement streamlined guidelines and consent procedures to ensure a patient’s informed consent is provided with full knowledge of how and where their images might be used and shared. Additional efforts should be made to protect patients’ privacy and unauthorized use of their images. Furthermore, we encourage our leading dermatology organizations to develop expert consensus on best practices for appropriate clinical image consent, storage, and use.
- Alvarado SM, Feng H. Representation of dark skin images of common dermatologic conditions in educational resources: a cross-sectional analysis [published online June 18, 2020]. J Am Acad Dermatol. 2021;84:1427-1431. doi:10.1016/j.jaad.2020.06.041
- Charrow A, Xia FD, Joyce C, et al. Diversity in dermatology clinical trials: a systematic review. JAMA Dermatol. 2017;153:193-198. doi:10.1001/jamadermatol.2016.4129
- Marroquin NA, Carboni A, Zueger M, et al. Skin of color representation trends in JAAD case reports 2015-2021: content analysis. JMIR Dermatol. 2023;6:e40816. doi:10.2196/40816
- Kim Y, Miller JJ, Hollins LC. Skin of color matters: a call to action. J Am Acad Dermatol. 2021;84:E273-E274. doi:10.1016/j.jaad.2020.11.026
- Nanda JK, Marchetti MA. Consent and deidentification of patient images in dermatology journals: observational study. JMIR Dermatol. 2022;5:E37398. doi:10.2196/37398
- US Department of Health and Human Services. Summary of the HIPAA privacy rule. Updated October 19, 2022. Accessed March 15, 2024. https://www.hhs.gov/hipaa/for-professionals/privacy/laws-regulations/index.html
- Quinn SC, Garza MA, Butler J, et al. Improving informed consent with minority participants: results from researcher and community surveys. J Empir Res Hum Res Ethics. 2012;7:44-55. doi:10.1525/jer.2012.7.5.44
- Hadden KB, Prince LY, Moore TD, et al. Improving readability of informed consents for research at an academic medical institution. J Clin Transl Sci. 2017;1:361-365. doi:10.1017/cts.2017.312
- Muvuka B, Combs RM, Ayangeakaa SD, et al. Health literacy in African-American communities: barriers and strategies. Health Lit Res Pract. 2020;4:E138-E143. doi:10.3928/24748307-20200617-01
- Menendez ME, van Hoorn BT, Mackert M, et al. Patients with limited health literacy ask fewer questions during office visits with hand surgeons. Clin Orthop Relat Res. 2017;475:1291-1297. doi:10.1007/s11999-016-5140-5
- Milam EC, Leger MC. Use of medical photography among dermatologists: a nationwide online survey study. J Eur Acad Dermatol Venereol. 2018;32:1804-1809. doi:10.1111/jdv.14839
- Leger MC, Wu T, Haimovic A, et al. Patient perspectives on medical photography in dermatology. Dermatol Surg. 2014;40:1028-1037. doi:10.1097/01.DSS.0000452632.22081.79
- Alvarado SM, Feng H. Representation of dark skin images of common dermatologic conditions in educational resources: a cross-sectional analysis [published online June 18, 2020]. J Am Acad Dermatol. 2021;84:1427-1431. doi:10.1016/j.jaad.2020.06.041
- Charrow A, Xia FD, Joyce C, et al. Diversity in dermatology clinical trials: a systematic review. JAMA Dermatol. 2017;153:193-198. doi:10.1001/jamadermatol.2016.4129
- Marroquin NA, Carboni A, Zueger M, et al. Skin of color representation trends in JAAD case reports 2015-2021: content analysis. JMIR Dermatol. 2023;6:e40816. doi:10.2196/40816
- Kim Y, Miller JJ, Hollins LC. Skin of color matters: a call to action. J Am Acad Dermatol. 2021;84:E273-E274. doi:10.1016/j.jaad.2020.11.026
- Nanda JK, Marchetti MA. Consent and deidentification of patient images in dermatology journals: observational study. JMIR Dermatol. 2022;5:E37398. doi:10.2196/37398
- US Department of Health and Human Services. Summary of the HIPAA privacy rule. Updated October 19, 2022. Accessed March 15, 2024. https://www.hhs.gov/hipaa/for-professionals/privacy/laws-regulations/index.html
- Quinn SC, Garza MA, Butler J, et al. Improving informed consent with minority participants: results from researcher and community surveys. J Empir Res Hum Res Ethics. 2012;7:44-55. doi:10.1525/jer.2012.7.5.44
- Hadden KB, Prince LY, Moore TD, et al. Improving readability of informed consents for research at an academic medical institution. J Clin Transl Sci. 2017;1:361-365. doi:10.1017/cts.2017.312
- Muvuka B, Combs RM, Ayangeakaa SD, et al. Health literacy in African-American communities: barriers and strategies. Health Lit Res Pract. 2020;4:E138-E143. doi:10.3928/24748307-20200617-01
- Menendez ME, van Hoorn BT, Mackert M, et al. Patients with limited health literacy ask fewer questions during office visits with hand surgeons. Clin Orthop Relat Res. 2017;475:1291-1297. doi:10.1007/s11999-016-5140-5
- Milam EC, Leger MC. Use of medical photography among dermatologists: a nationwide online survey study. J Eur Acad Dermatol Venereol. 2018;32:1804-1809. doi:10.1111/jdv.14839
- Leger MC, Wu T, Haimovic A, et al. Patient perspectives on medical photography in dermatology. Dermatol Surg. 2014;40:1028-1037. doi:10.1097/01.DSS.0000452632.22081.79
Underrepresented Minority Students Applying to Dermatology Residency in the COVID-19 Era: Challenges and Considerations
The COVID-19 pandemic has markedly changed the dermatology residency application process. As medical students head into this application cycle, the impacts of systemic racism and deeply rooted structural barriers continue to be exacerbated for students who identify as an underrepresented minority (URM) in medicine—historically defined as those who self-identify as Hispanic or Latinx; Black or African American; American Indian or Alaska Native; or Native Hawaiian or Pacific Islander. The Association of American Medical Colleges (AAMC) defines URMs as racial and ethnic populations that are underrepresented in medicine relative to their numbers in the general population.1 Although these groups account for approximately 34% of the population of the United States, they constitute only 11% of the country’s physician workforce.2,3
Of the total physician workforce in the United States, Black and African American physicians account for 5% of practicing physicians; Hispanic physicians, 5.8%; American Indian and Alaska Native physicians, 0.3%; and Native Hawaiian and Pacific Islander physicians, 0.1%.2 In competitive medical specialties, the disproportionality of these numbers compared to our current demographics in the United States as shown above is even more staggering. In 2018, for example, 10% of practicing dermatologists identified as female URM physicians; 6%, as male URM physicians.2 In this article, we discuss some of the challenges and considerations for URM students applying to dermatology residency in the era of the COVID-19 pandemic.
Barriers for URM Students in Dermatology
Multiple studies have attempted to identify some of the barriers faced by URM students in medicine that might explain the lack of diversity in competitive specialties. Vasquez and colleagues4 identified 4 major factors that play a role in dermatology: lack of equitable resources, lack of support, financial limitations, and the lack of group identity. More than half of URM students surveyed (1) identified lack of support as a barrier and (2) reported having been encouraged to seek a specialty more reflective of their community.4
Soliman et al5 reported that URM barriers in dermatology extend to include lack of diversity in the field, socioeconomic factors, lack of mentorship, and a negative perception of minority students by residency programs. Dermatology is the second least diverse specialty in medicine after orthopedic surgery, which, in and of itself, might further discourage URM students from applying to dermatology.5
With the minimal exposure that URM students have to the field of dermatology, the lack of pipeline programs, and reports that URMs often are encouraged to pursue primary care, the current diversity deficiency in dermatology comes as no surprise. In addition, the substantial disadvantage for URM students is perpetuated by the traditional highly selective process that favors grades, board scores, and honor society status over holistic assessment of the individual student and their unique experiences and potential for contribution.
Looking Beyond Test Scores
The US Medical Licensing Examination (USMLE) traditionally has been used to select dermatology residency applicants, with high cutoff scores often excluding outstanding URM students. Research has suggested that the use of USMLE examination test scores for residency recruitment lacks validity because it has poor predictability of residency performance.6 Although the USMLE Step 1 examination is transitioning to pass/fail scoring, applicants for the next cycle will still have a 3-digit numerical score.
We strongly recommend that dermatology programs transition from emphasizing scores of residency candidates to reviewing each candidate holistically. The AAMC defines “holistic review” as a “flexible, individualized way of assessing an applicant’s capabilities, by which balanced consideration is given to experiences, attributes, competencies, and academic or scholarly metrics and, when considered in combination, how the individual might contribute value to the institution’s mission.”7 Furthermore, we recommend that dermatology residency programs have multiple faculty members review each application, including a representative of the diversity, inclusion, and equity committee.
Applying to Residency in the COVID-19 Virtual Environment
In the COVID-19 era, dermatology externship opportunities that would have allowed URM students to work directly with potential residency programs, showcase their abilities, and network have been limited. Virtual residency interviews could make it more challenging to evaluate candidates, especially URM students from less prestigious programs or unusual socioeconomic backgrounds, or with lower board scores. In addition, virtual interviews can more easily become one-dimensional, depriving URM students of the opportunity to gauge their personal fit in a specific dermatology residency program and its community. Questions and concerns of URM students might include: Will I be appropriately supported and mentored? Will my cultural preferences, religion, sexual preference, hairstyle, and beliefs be accepted? Can I advocate for minorities and support antiracism and diversity and inclusion initiatives? To that end, we recommend that dermatology programs continue to host virtual meet-and-greet events for potential students to meet faculty and learn more about the program. In addition, programs should consider having current residents interact virtually with candidates to allow students to better understand the culture of the department and residents’ experiences as trainees in such an environment. For URM students, this is highly important because diversity, inclusion, and antiracism policies and initiatives might not be explicitly available on the institution’s website or residency information page.
Organizations Championing Diversity
Recently, multiple dermatology societies and organizations have been emphasizing the need for diversity and inclusion as well as promoting holistic application review. The American Academy of Dermatology pioneered the Diversity Champion Workshop in 2019 and continues to offer the Diversity Mentorship program, connecting URM students to mentors nationally. The Skin of Color Society offers yearly grants and awards to medical students to develop mentorship and research, and recently hosted webinars to guide medical students and residency programs on diversity and inclusion, residency application and review, and COVID-19 virtual interviews. Other national societies, such as the Student National Medical Association and Latino Medical Student Association, have been promoting workshops and interview mentoring for URM students, including dermatology-specific events. Although it is estimated that more than 90% of medical schools in the United States already perform holistic application review and that such review has been adopted by many dermatology programs nationwide, data regarding dermatology residency programs’ implementation of holistic application review are lacking.8
In addition, we encourage continuation of the proposed coordinated interview invite release from the Association of Professors of Dermatology, which was implemented in the 2020-2021 cycle. In light of the recent AAMC letter9 on the maldistribution of interview invitations to highest-tier applicants, coordination of interview release dates and other similar initiatives to prevent programs from offering more invites than their available slots and improve transparency about interview days are needed. Furthermore, continuing to offer optional virtual interviews for applicants in future cycles could make the process less cost-prohibitive for many URM students.4,5
Final Thoughts
Dermatology residency programs must intentionally guard against falling back to traditional standards of assessment as the only means of student evaluation, especially in this virtual era. It is our responsibility to remove artificial barriers that continue to stall progress in diversity, inclusion, equity, and belonging in dermatology.
- Underrepresented in medicine definition. Association of American Medical Colleges website. Accessed September 27, 2021. https://www.aamc.org/what-we-do/mission-areas/diversity-inclusion/underrepresented-in-medicine
- Diversity in medicine: facts and figures 2019. table 13. practice specialty, males by race/ethnicity, 2018. Association of American Medical Colleges website. Accessed September 27, 2021. https://www.aamc.org/data-reports/workforce/data/table-13-practice-specialty-males-race/ethnicity-2018 1B
- US Census Bureau. Quick facts: United States. Updated July 1, 2019. Accessed September 20, 2021. https://www.census.gov/quickfacts/fact/table/US/PST045219
- Vasquez R, Jeong H, Florez-Pollack S, et al. What are the barriers faced by underrepresented minorities applying to dermatology? a qualitative cross-sectional study of applicants applying to a large dermatology residency program. J Am Acad Dermatol. 2020;83:1770-1773. doi:10.1016/j.jaad.2020.03.067
- Soliman YS, Rzepecki AK, Guzman AK, et al. Understanding perceived barriers of minority medical students pursuing a career in dermatology. JAMA Dermatol. 2019;155:252-254. doi:10.1001/jamadermatol.2018.4813
- Williams C, Kwan B, Pereira A, et al. A call to improve conditions for conducting holistic review in graduate medical education recruitment. MedEdPublish. 2019;8:6. https://doi.org/10.15694/mep.2019.000076.1
- Holistic principles in resident selection: an introduction. Association of American Medical Colleges website. Accessed September 27, 2021. https://www.aamc.org/system/files/2020-08/aa-member-capacity-building-holistic-review-transcript-activities-GME-081420.pdf
- Luke J, Cornelius L, Lim H. Dermatology resident selection: shifting toward holistic review? J Am Acad Dermatol. 2020;84:1208-1209. doi:10.1016/j.jaad.2020.11.025
- Open letter on residency interviews from Alison Whelan, MD, AAMC Chief Medical Education Officer. Association of American Medical Colleges website. Published December 18, 2020. Accessed September 27, 2021. https://www.aamc.org/media/50291/download
The COVID-19 pandemic has markedly changed the dermatology residency application process. As medical students head into this application cycle, the impacts of systemic racism and deeply rooted structural barriers continue to be exacerbated for students who identify as an underrepresented minority (URM) in medicine—historically defined as those who self-identify as Hispanic or Latinx; Black or African American; American Indian or Alaska Native; or Native Hawaiian or Pacific Islander. The Association of American Medical Colleges (AAMC) defines URMs as racial and ethnic populations that are underrepresented in medicine relative to their numbers in the general population.1 Although these groups account for approximately 34% of the population of the United States, they constitute only 11% of the country’s physician workforce.2,3
Of the total physician workforce in the United States, Black and African American physicians account for 5% of practicing physicians; Hispanic physicians, 5.8%; American Indian and Alaska Native physicians, 0.3%; and Native Hawaiian and Pacific Islander physicians, 0.1%.2 In competitive medical specialties, the disproportionality of these numbers compared to our current demographics in the United States as shown above is even more staggering. In 2018, for example, 10% of practicing dermatologists identified as female URM physicians; 6%, as male URM physicians.2 In this article, we discuss some of the challenges and considerations for URM students applying to dermatology residency in the era of the COVID-19 pandemic.
Barriers for URM Students in Dermatology
Multiple studies have attempted to identify some of the barriers faced by URM students in medicine that might explain the lack of diversity in competitive specialties. Vasquez and colleagues4 identified 4 major factors that play a role in dermatology: lack of equitable resources, lack of support, financial limitations, and the lack of group identity. More than half of URM students surveyed (1) identified lack of support as a barrier and (2) reported having been encouraged to seek a specialty more reflective of their community.4
Soliman et al5 reported that URM barriers in dermatology extend to include lack of diversity in the field, socioeconomic factors, lack of mentorship, and a negative perception of minority students by residency programs. Dermatology is the second least diverse specialty in medicine after orthopedic surgery, which, in and of itself, might further discourage URM students from applying to dermatology.5
With the minimal exposure that URM students have to the field of dermatology, the lack of pipeline programs, and reports that URMs often are encouraged to pursue primary care, the current diversity deficiency in dermatology comes as no surprise. In addition, the substantial disadvantage for URM students is perpetuated by the traditional highly selective process that favors grades, board scores, and honor society status over holistic assessment of the individual student and their unique experiences and potential for contribution.
Looking Beyond Test Scores
The US Medical Licensing Examination (USMLE) traditionally has been used to select dermatology residency applicants, with high cutoff scores often excluding outstanding URM students. Research has suggested that the use of USMLE examination test scores for residency recruitment lacks validity because it has poor predictability of residency performance.6 Although the USMLE Step 1 examination is transitioning to pass/fail scoring, applicants for the next cycle will still have a 3-digit numerical score.
We strongly recommend that dermatology programs transition from emphasizing scores of residency candidates to reviewing each candidate holistically. The AAMC defines “holistic review” as a “flexible, individualized way of assessing an applicant’s capabilities, by which balanced consideration is given to experiences, attributes, competencies, and academic or scholarly metrics and, when considered in combination, how the individual might contribute value to the institution’s mission.”7 Furthermore, we recommend that dermatology residency programs have multiple faculty members review each application, including a representative of the diversity, inclusion, and equity committee.
Applying to Residency in the COVID-19 Virtual Environment
In the COVID-19 era, dermatology externship opportunities that would have allowed URM students to work directly with potential residency programs, showcase their abilities, and network have been limited. Virtual residency interviews could make it more challenging to evaluate candidates, especially URM students from less prestigious programs or unusual socioeconomic backgrounds, or with lower board scores. In addition, virtual interviews can more easily become one-dimensional, depriving URM students of the opportunity to gauge their personal fit in a specific dermatology residency program and its community. Questions and concerns of URM students might include: Will I be appropriately supported and mentored? Will my cultural preferences, religion, sexual preference, hairstyle, and beliefs be accepted? Can I advocate for minorities and support antiracism and diversity and inclusion initiatives? To that end, we recommend that dermatology programs continue to host virtual meet-and-greet events for potential students to meet faculty and learn more about the program. In addition, programs should consider having current residents interact virtually with candidates to allow students to better understand the culture of the department and residents’ experiences as trainees in such an environment. For URM students, this is highly important because diversity, inclusion, and antiracism policies and initiatives might not be explicitly available on the institution’s website or residency information page.
Organizations Championing Diversity
Recently, multiple dermatology societies and organizations have been emphasizing the need for diversity and inclusion as well as promoting holistic application review. The American Academy of Dermatology pioneered the Diversity Champion Workshop in 2019 and continues to offer the Diversity Mentorship program, connecting URM students to mentors nationally. The Skin of Color Society offers yearly grants and awards to medical students to develop mentorship and research, and recently hosted webinars to guide medical students and residency programs on diversity and inclusion, residency application and review, and COVID-19 virtual interviews. Other national societies, such as the Student National Medical Association and Latino Medical Student Association, have been promoting workshops and interview mentoring for URM students, including dermatology-specific events. Although it is estimated that more than 90% of medical schools in the United States already perform holistic application review and that such review has been adopted by many dermatology programs nationwide, data regarding dermatology residency programs’ implementation of holistic application review are lacking.8
In addition, we encourage continuation of the proposed coordinated interview invite release from the Association of Professors of Dermatology, which was implemented in the 2020-2021 cycle. In light of the recent AAMC letter9 on the maldistribution of interview invitations to highest-tier applicants, coordination of interview release dates and other similar initiatives to prevent programs from offering more invites than their available slots and improve transparency about interview days are needed. Furthermore, continuing to offer optional virtual interviews for applicants in future cycles could make the process less cost-prohibitive for many URM students.4,5
Final Thoughts
Dermatology residency programs must intentionally guard against falling back to traditional standards of assessment as the only means of student evaluation, especially in this virtual era. It is our responsibility to remove artificial barriers that continue to stall progress in diversity, inclusion, equity, and belonging in dermatology.
The COVID-19 pandemic has markedly changed the dermatology residency application process. As medical students head into this application cycle, the impacts of systemic racism and deeply rooted structural barriers continue to be exacerbated for students who identify as an underrepresented minority (URM) in medicine—historically defined as those who self-identify as Hispanic or Latinx; Black or African American; American Indian or Alaska Native; or Native Hawaiian or Pacific Islander. The Association of American Medical Colleges (AAMC) defines URMs as racial and ethnic populations that are underrepresented in medicine relative to their numbers in the general population.1 Although these groups account for approximately 34% of the population of the United States, they constitute only 11% of the country’s physician workforce.2,3
Of the total physician workforce in the United States, Black and African American physicians account for 5% of practicing physicians; Hispanic physicians, 5.8%; American Indian and Alaska Native physicians, 0.3%; and Native Hawaiian and Pacific Islander physicians, 0.1%.2 In competitive medical specialties, the disproportionality of these numbers compared to our current demographics in the United States as shown above is even more staggering. In 2018, for example, 10% of practicing dermatologists identified as female URM physicians; 6%, as male URM physicians.2 In this article, we discuss some of the challenges and considerations for URM students applying to dermatology residency in the era of the COVID-19 pandemic.
Barriers for URM Students in Dermatology
Multiple studies have attempted to identify some of the barriers faced by URM students in medicine that might explain the lack of diversity in competitive specialties. Vasquez and colleagues4 identified 4 major factors that play a role in dermatology: lack of equitable resources, lack of support, financial limitations, and the lack of group identity. More than half of URM students surveyed (1) identified lack of support as a barrier and (2) reported having been encouraged to seek a specialty more reflective of their community.4
Soliman et al5 reported that URM barriers in dermatology extend to include lack of diversity in the field, socioeconomic factors, lack of mentorship, and a negative perception of minority students by residency programs. Dermatology is the second least diverse specialty in medicine after orthopedic surgery, which, in and of itself, might further discourage URM students from applying to dermatology.5
With the minimal exposure that URM students have to the field of dermatology, the lack of pipeline programs, and reports that URMs often are encouraged to pursue primary care, the current diversity deficiency in dermatology comes as no surprise. In addition, the substantial disadvantage for URM students is perpetuated by the traditional highly selective process that favors grades, board scores, and honor society status over holistic assessment of the individual student and their unique experiences and potential for contribution.
Looking Beyond Test Scores
The US Medical Licensing Examination (USMLE) traditionally has been used to select dermatology residency applicants, with high cutoff scores often excluding outstanding URM students. Research has suggested that the use of USMLE examination test scores for residency recruitment lacks validity because it has poor predictability of residency performance.6 Although the USMLE Step 1 examination is transitioning to pass/fail scoring, applicants for the next cycle will still have a 3-digit numerical score.
We strongly recommend that dermatology programs transition from emphasizing scores of residency candidates to reviewing each candidate holistically. The AAMC defines “holistic review” as a “flexible, individualized way of assessing an applicant’s capabilities, by which balanced consideration is given to experiences, attributes, competencies, and academic or scholarly metrics and, when considered in combination, how the individual might contribute value to the institution’s mission.”7 Furthermore, we recommend that dermatology residency programs have multiple faculty members review each application, including a representative of the diversity, inclusion, and equity committee.
Applying to Residency in the COVID-19 Virtual Environment
In the COVID-19 era, dermatology externship opportunities that would have allowed URM students to work directly with potential residency programs, showcase their abilities, and network have been limited. Virtual residency interviews could make it more challenging to evaluate candidates, especially URM students from less prestigious programs or unusual socioeconomic backgrounds, or with lower board scores. In addition, virtual interviews can more easily become one-dimensional, depriving URM students of the opportunity to gauge their personal fit in a specific dermatology residency program and its community. Questions and concerns of URM students might include: Will I be appropriately supported and mentored? Will my cultural preferences, religion, sexual preference, hairstyle, and beliefs be accepted? Can I advocate for minorities and support antiracism and diversity and inclusion initiatives? To that end, we recommend that dermatology programs continue to host virtual meet-and-greet events for potential students to meet faculty and learn more about the program. In addition, programs should consider having current residents interact virtually with candidates to allow students to better understand the culture of the department and residents’ experiences as trainees in such an environment. For URM students, this is highly important because diversity, inclusion, and antiracism policies and initiatives might not be explicitly available on the institution’s website or residency information page.
Organizations Championing Diversity
Recently, multiple dermatology societies and organizations have been emphasizing the need for diversity and inclusion as well as promoting holistic application review. The American Academy of Dermatology pioneered the Diversity Champion Workshop in 2019 and continues to offer the Diversity Mentorship program, connecting URM students to mentors nationally. The Skin of Color Society offers yearly grants and awards to medical students to develop mentorship and research, and recently hosted webinars to guide medical students and residency programs on diversity and inclusion, residency application and review, and COVID-19 virtual interviews. Other national societies, such as the Student National Medical Association and Latino Medical Student Association, have been promoting workshops and interview mentoring for URM students, including dermatology-specific events. Although it is estimated that more than 90% of medical schools in the United States already perform holistic application review and that such review has been adopted by many dermatology programs nationwide, data regarding dermatology residency programs’ implementation of holistic application review are lacking.8
In addition, we encourage continuation of the proposed coordinated interview invite release from the Association of Professors of Dermatology, which was implemented in the 2020-2021 cycle. In light of the recent AAMC letter9 on the maldistribution of interview invitations to highest-tier applicants, coordination of interview release dates and other similar initiatives to prevent programs from offering more invites than their available slots and improve transparency about interview days are needed. Furthermore, continuing to offer optional virtual interviews for applicants in future cycles could make the process less cost-prohibitive for many URM students.4,5
Final Thoughts
Dermatology residency programs must intentionally guard against falling back to traditional standards of assessment as the only means of student evaluation, especially in this virtual era. It is our responsibility to remove artificial barriers that continue to stall progress in diversity, inclusion, equity, and belonging in dermatology.
- Underrepresented in medicine definition. Association of American Medical Colleges website. Accessed September 27, 2021. https://www.aamc.org/what-we-do/mission-areas/diversity-inclusion/underrepresented-in-medicine
- Diversity in medicine: facts and figures 2019. table 13. practice specialty, males by race/ethnicity, 2018. Association of American Medical Colleges website. Accessed September 27, 2021. https://www.aamc.org/data-reports/workforce/data/table-13-practice-specialty-males-race/ethnicity-2018 1B
- US Census Bureau. Quick facts: United States. Updated July 1, 2019. Accessed September 20, 2021. https://www.census.gov/quickfacts/fact/table/US/PST045219
- Vasquez R, Jeong H, Florez-Pollack S, et al. What are the barriers faced by underrepresented minorities applying to dermatology? a qualitative cross-sectional study of applicants applying to a large dermatology residency program. J Am Acad Dermatol. 2020;83:1770-1773. doi:10.1016/j.jaad.2020.03.067
- Soliman YS, Rzepecki AK, Guzman AK, et al. Understanding perceived barriers of minority medical students pursuing a career in dermatology. JAMA Dermatol. 2019;155:252-254. doi:10.1001/jamadermatol.2018.4813
- Williams C, Kwan B, Pereira A, et al. A call to improve conditions for conducting holistic review in graduate medical education recruitment. MedEdPublish. 2019;8:6. https://doi.org/10.15694/mep.2019.000076.1
- Holistic principles in resident selection: an introduction. Association of American Medical Colleges website. Accessed September 27, 2021. https://www.aamc.org/system/files/2020-08/aa-member-capacity-building-holistic-review-transcript-activities-GME-081420.pdf
- Luke J, Cornelius L, Lim H. Dermatology resident selection: shifting toward holistic review? J Am Acad Dermatol. 2020;84:1208-1209. doi:10.1016/j.jaad.2020.11.025
- Open letter on residency interviews from Alison Whelan, MD, AAMC Chief Medical Education Officer. Association of American Medical Colleges website. Published December 18, 2020. Accessed September 27, 2021. https://www.aamc.org/media/50291/download
- Underrepresented in medicine definition. Association of American Medical Colleges website. Accessed September 27, 2021. https://www.aamc.org/what-we-do/mission-areas/diversity-inclusion/underrepresented-in-medicine
- Diversity in medicine: facts and figures 2019. table 13. practice specialty, males by race/ethnicity, 2018. Association of American Medical Colleges website. Accessed September 27, 2021. https://www.aamc.org/data-reports/workforce/data/table-13-practice-specialty-males-race/ethnicity-2018 1B
- US Census Bureau. Quick facts: United States. Updated July 1, 2019. Accessed September 20, 2021. https://www.census.gov/quickfacts/fact/table/US/PST045219
- Vasquez R, Jeong H, Florez-Pollack S, et al. What are the barriers faced by underrepresented minorities applying to dermatology? a qualitative cross-sectional study of applicants applying to a large dermatology residency program. J Am Acad Dermatol. 2020;83:1770-1773. doi:10.1016/j.jaad.2020.03.067
- Soliman YS, Rzepecki AK, Guzman AK, et al. Understanding perceived barriers of minority medical students pursuing a career in dermatology. JAMA Dermatol. 2019;155:252-254. doi:10.1001/jamadermatol.2018.4813
- Williams C, Kwan B, Pereira A, et al. A call to improve conditions for conducting holistic review in graduate medical education recruitment. MedEdPublish. 2019;8:6. https://doi.org/10.15694/mep.2019.000076.1
- Holistic principles in resident selection: an introduction. Association of American Medical Colleges website. Accessed September 27, 2021. https://www.aamc.org/system/files/2020-08/aa-member-capacity-building-holistic-review-transcript-activities-GME-081420.pdf
- Luke J, Cornelius L, Lim H. Dermatology resident selection: shifting toward holistic review? J Am Acad Dermatol. 2020;84:1208-1209. doi:10.1016/j.jaad.2020.11.025
- Open letter on residency interviews from Alison Whelan, MD, AAMC Chief Medical Education Officer. Association of American Medical Colleges website. Published December 18, 2020. Accessed September 27, 2021. https://www.aamc.org/media/50291/download
Practice Points
- Dermatology remains one of the least diverse medical specialties.
- Underrepresented minority (URM) in medicine residency applicants might be negatively affected by the COVID-19 pandemic.
- The implementation of holistic review, diversity and inclusion initiatives, and virtual opportunities might mitigate some of the barriers faced by URM applicants.
Mohs Micrographic Surgery During the COVID-19 Pandemic: Considering the Patient Perspective
Guidelines on Skin Cancer Surgeries During the COVID-19 Pandemic
At the start of the COVID-19 pandemic, the Centers for Disease Control and Prevention issued recommendations to decrease the spread of SARS-CoV-2 and optimize the use of personal protective equipment (PPE) for frontline workers.1 In the field of dermatologic surgery, the American College of Mohs Surgery, the National Comprehensive Cancer Network, the American Society for Dermatologic Surgery, and the American Academy of Dermatology made recommendations to postpone nonessential and nonurgent procedures.2-4 The initial guidelines of the American College of Mohs Surgery advised cancellation of all elective surgeries and deferred treatment of most cases of basal cell carcinoma for as long as 3 months; low-risk squamous cell carcinoma (SCC) and melanoma in situ treatment was deferred for as long as 2 or 3 months.3 Additional recommendations were made to reserve inpatient visits for suspicious lesions and high-risk cancers, postpone other nonessential and nonurgent appointments, and utilize telemedicine whenever possible.5
These recommendations led to great uncertainty and stress for patients and providers. Although numerous important variables, such as patient risk factors, severity of disease, availability of PPE and staff, and patient-to-provider transmission were considered when creating these guidelines, the patient’s experience likely was not a contributing factor.
COVID-19 Transmission During Mohs Surgery
There have been concerns that surgeons performing Mohs micrographic surgery (MMS) might be at an increased risk for COVID-19, given their close contact with high-risk sites (ie, nose, mouth) and cautery-generated aerosols; most of the estimated transmission risk associated with MMS has been based on head and neck surgery experience and publications.6-8 Tee and colleagues9 recently published their institution’s MMS COVID-19 preventive measures, which, to their knowledge, have prevented all intraoperative transmission of SARS-CoV-2, even in disease-positive patients. Currently, evidence is lacking to support a high risk for SARS-CoV-2 transmission during MMS when proper PPE and personal hygiene measures as well as strict infection control protocols—presurgical COVID-19 testing in high-risk cases, COVID-19 screening optimization, visitor restrictions, and appropriate disinfection between patients—are in place.
The Impact of Postponing Treatment on Patients
Although studies have focused on the effects of the COVID-19 pandemic on physicians practicing MMS,10 little is known about the effects of delays in skin cancer treatment on patients. A survey conducted in the United Kingdom investigating the patient’s perspective found that patients expressed worry and concern about the possibility that their MMS would be postponed and greatly appreciated continuation of treatment during the pandemic.11
Other medical specialties have reported their patient experiences during the pandemic. In a study examining patient perception of postponed surgical treatment of pelvic floor disorders due to COVID-19, nearly half of survey respondents were unhappy with the delay in receiving care. Furthermore, patients who reported being unhappy were more likely to report feelings of isolation and anxiety because their surgery was postponed.12 In another study involving patients with lung cancer, 9.1% (N=15) of patients postponed their treatment during the COVID-19 pandemic because of pandemic-related anxiety.13
With the goal of improving care at our institution, we conducted a brief institutional review board–approved survey to evaluate how postponing MMS treatment due to the COVID-19 pandemic affected patients. All MMS patients undergoing surgery in June 2020 and July 2020 (N=99) were asked to complete our voluntary and anonymous 23-question survey in person during their procedure. We obtained 88 responses (response rate, 89%). Twenty percent of surveyed patients (n=18) reported that their MMS had been postponed; 78% of those whose MMS was postponed (n=14) indicated some level of anxiety during the waiting period. It was unclear which patients had their treatment postponed based on national guidelines and which ones elected to postpone surgery.
Tips for Health Care Providers
Patient-provider communication highlighting specific skin cancer risk and the risk vs benefit of postponing treatment might reduce anxiety and stress during the waiting period.14 A study found that COVID-19 posed a bigger threat than most noninvasive skin cancers; therefore, the authors of that study concluded that treatment for most skin cancers could be safely postponed.15 Specifically, those authors recommended prioritizing treatment for Merkel cell carcinoma, invasive SCC, and melanoma with positive margins or macroscopic residual disease. They proposed that all other skin cancers, including basal cell carcinoma, SCC in situ, and melanoma with negative margins and no macroscopic residual disease, could be safely delayed for as long as 3 months.15
For patients with multiple risk factors for COVID-19–related morbidity or mortality, delaying skin cancer treatment likely has less risk than contracting the virus.15 This information should be communicated with patients. Investigation of specific patient concerns is warranted, and case-by-case evaluation of patients’ risk factors and skin cancer risk should be considered.
Based on the current, though limited, literature, delaying medical treatment can have a negative impact on the patient experience. Furthermore, proper precautions have been shown to limit intraoperative transmission of SARS-CoV-2 during MMS, but research is lacking. Practitioners should utilize shared decision-making and evaluate a given patient’s risk factors and concerns when deciding whether to postpone treatment. We encourage other institutions to evaluate the effects that delaying MMS has had on their patients, as further studies would improve understanding of patients’ experiences during a pandemic and potentially influence future dermatology guidelines.
- Center for Disease Control and Prevention. COVID-19. Accessed April 20, 2021. https://www.cdc.gov/coronavirus/2019-ncov/index.html
- American College of Mohs Surgery. Mohs surgery ambulatory protocol during COVID pandemic (version 6-3-20). June 4, 2020. Accessed April 20, 2021. http://staging.mohscollege.org/UserFiles/AM20/Member%20Alert/MohsSurgeryAmbulatoryProtocolDuringCOVIDPandemicFinal.pdf
- COVID-19 resources. National Comprehensive Cancer Network website. Accessed April 20, 2021. https://www.nccn.org/covid-19
- Narla S, Alam M, Ozog DM, et al. American Society of Dermatologic Surgery Association (ASDSA) and American Society for Laser Medicine & Surgery (ASLMS) guidance for cosmetic dermatology practices during COVID-19. Updated January 11, 2021. Accessed April 10, 2021. https://www.asds.net/Portals/0/PDF/asdsa/asdsa-aslms-cosmetic-reopening-guidance.pdf
- Geskin LJ, Trager MH, Aasi SZ, et al. Perspectives on the recommendations for skin cancer management during the COVID-19 pandemic.J Am Acad Dermatol. 2020;83:295-296. doi:10.1016/j.jaad.2020.05.002
- Yuan JT, Jiang SIB. Urgent safety considerations for dermatologic surgeons in the COVID-19 pandemic. Dermatol Online J. 2020;26:1. Accessed April 20, 2021. http://escholarship.org/uc/item/2qr3w771
- Otolaryngologists may contract COVID-19 during surgery. ENTtoday. March 20, 2020. Accessed April 20, 2021. https://www.enttoday.org/article/otolaryngologists-may-contract-covid-19-during-surgery/
- Howard BE. High-risk aerosol-generating procedures in COVID-19: respiratory protective equipment considerations. Otolaryngol Head Neck Surg. 2020;163:98-103. doi:10.1177/0194599820927335
- Tee MW, Stewart C, Aliessa S, et al. Dermatological surgery during the COVID-19 pandemic: experience of a large academic center. J Am Acad Dermatol. 2021;84:1094-1096. doi:10.1016/j.jaad.2020.12.003
- Hooper J, Feng H. The impact of COVID-19 on micrographic surgery and dermatologic oncology fellows. Dermatol Surg. 2020;46:1762-1763. doi:10.1097/DSS.0000000000002766
- Nicholson P, Ali FR, Patalay R, et al. Patient perceptions of Mohs micrographic surgery during the COVID-19 pandemic and lessons for the next outbreak. Clin Exp Dermatol. 2021;46:179-180. doi:10.1111/ced.14423
- Mou T, Brown O, Gillingham A, et al. Patients’ perceptions on surgical care suspension for pelvic floor disorders during the COVID-19 pandemic. Female Pelvic Med Reconstr Surg. 2020;26:477-482. doi:10.1097/SPV.0000000000000918
- Fujita K, Ito T, Saito Z, et al. Impact of COVID-19 pandemic on lung cancer treatment scheduling. Thorac Cancer. 2020;11:2983-2986. doi:10.1111/1759-7714.13615
- Nikumb VB, Banerjee A, Kaur G, et al. Impact of doctor-patient communication on preoperative anxiety: study at industrial township, Pimpri, Pune. Ind Psychiatry J. 2009;18:19-21. doi:10.4103/0972-6748.57852
- Baumann BC, MacArthur KM, Brewer JD, et al. Management of primary skin cancer during a pandemic: multidisciplinary recommendations. Cancer. 2020;126:3900-3906. doi:10.1002/cncr.32969
Guidelines on Skin Cancer Surgeries During the COVID-19 Pandemic
At the start of the COVID-19 pandemic, the Centers for Disease Control and Prevention issued recommendations to decrease the spread of SARS-CoV-2 and optimize the use of personal protective equipment (PPE) for frontline workers.1 In the field of dermatologic surgery, the American College of Mohs Surgery, the National Comprehensive Cancer Network, the American Society for Dermatologic Surgery, and the American Academy of Dermatology made recommendations to postpone nonessential and nonurgent procedures.2-4 The initial guidelines of the American College of Mohs Surgery advised cancellation of all elective surgeries and deferred treatment of most cases of basal cell carcinoma for as long as 3 months; low-risk squamous cell carcinoma (SCC) and melanoma in situ treatment was deferred for as long as 2 or 3 months.3 Additional recommendations were made to reserve inpatient visits for suspicious lesions and high-risk cancers, postpone other nonessential and nonurgent appointments, and utilize telemedicine whenever possible.5
These recommendations led to great uncertainty and stress for patients and providers. Although numerous important variables, such as patient risk factors, severity of disease, availability of PPE and staff, and patient-to-provider transmission were considered when creating these guidelines, the patient’s experience likely was not a contributing factor.
COVID-19 Transmission During Mohs Surgery
There have been concerns that surgeons performing Mohs micrographic surgery (MMS) might be at an increased risk for COVID-19, given their close contact with high-risk sites (ie, nose, mouth) and cautery-generated aerosols; most of the estimated transmission risk associated with MMS has been based on head and neck surgery experience and publications.6-8 Tee and colleagues9 recently published their institution’s MMS COVID-19 preventive measures, which, to their knowledge, have prevented all intraoperative transmission of SARS-CoV-2, even in disease-positive patients. Currently, evidence is lacking to support a high risk for SARS-CoV-2 transmission during MMS when proper PPE and personal hygiene measures as well as strict infection control protocols—presurgical COVID-19 testing in high-risk cases, COVID-19 screening optimization, visitor restrictions, and appropriate disinfection between patients—are in place.
The Impact of Postponing Treatment on Patients
Although studies have focused on the effects of the COVID-19 pandemic on physicians practicing MMS,10 little is known about the effects of delays in skin cancer treatment on patients. A survey conducted in the United Kingdom investigating the patient’s perspective found that patients expressed worry and concern about the possibility that their MMS would be postponed and greatly appreciated continuation of treatment during the pandemic.11
Other medical specialties have reported their patient experiences during the pandemic. In a study examining patient perception of postponed surgical treatment of pelvic floor disorders due to COVID-19, nearly half of survey respondents were unhappy with the delay in receiving care. Furthermore, patients who reported being unhappy were more likely to report feelings of isolation and anxiety because their surgery was postponed.12 In another study involving patients with lung cancer, 9.1% (N=15) of patients postponed their treatment during the COVID-19 pandemic because of pandemic-related anxiety.13
With the goal of improving care at our institution, we conducted a brief institutional review board–approved survey to evaluate how postponing MMS treatment due to the COVID-19 pandemic affected patients. All MMS patients undergoing surgery in June 2020 and July 2020 (N=99) were asked to complete our voluntary and anonymous 23-question survey in person during their procedure. We obtained 88 responses (response rate, 89%). Twenty percent of surveyed patients (n=18) reported that their MMS had been postponed; 78% of those whose MMS was postponed (n=14) indicated some level of anxiety during the waiting period. It was unclear which patients had their treatment postponed based on national guidelines and which ones elected to postpone surgery.
Tips for Health Care Providers
Patient-provider communication highlighting specific skin cancer risk and the risk vs benefit of postponing treatment might reduce anxiety and stress during the waiting period.14 A study found that COVID-19 posed a bigger threat than most noninvasive skin cancers; therefore, the authors of that study concluded that treatment for most skin cancers could be safely postponed.15 Specifically, those authors recommended prioritizing treatment for Merkel cell carcinoma, invasive SCC, and melanoma with positive margins or macroscopic residual disease. They proposed that all other skin cancers, including basal cell carcinoma, SCC in situ, and melanoma with negative margins and no macroscopic residual disease, could be safely delayed for as long as 3 months.15
For patients with multiple risk factors for COVID-19–related morbidity or mortality, delaying skin cancer treatment likely has less risk than contracting the virus.15 This information should be communicated with patients. Investigation of specific patient concerns is warranted, and case-by-case evaluation of patients’ risk factors and skin cancer risk should be considered.
Based on the current, though limited, literature, delaying medical treatment can have a negative impact on the patient experience. Furthermore, proper precautions have been shown to limit intraoperative transmission of SARS-CoV-2 during MMS, but research is lacking. Practitioners should utilize shared decision-making and evaluate a given patient’s risk factors and concerns when deciding whether to postpone treatment. We encourage other institutions to evaluate the effects that delaying MMS has had on their patients, as further studies would improve understanding of patients’ experiences during a pandemic and potentially influence future dermatology guidelines.
Guidelines on Skin Cancer Surgeries During the COVID-19 Pandemic
At the start of the COVID-19 pandemic, the Centers for Disease Control and Prevention issued recommendations to decrease the spread of SARS-CoV-2 and optimize the use of personal protective equipment (PPE) for frontline workers.1 In the field of dermatologic surgery, the American College of Mohs Surgery, the National Comprehensive Cancer Network, the American Society for Dermatologic Surgery, and the American Academy of Dermatology made recommendations to postpone nonessential and nonurgent procedures.2-4 The initial guidelines of the American College of Mohs Surgery advised cancellation of all elective surgeries and deferred treatment of most cases of basal cell carcinoma for as long as 3 months; low-risk squamous cell carcinoma (SCC) and melanoma in situ treatment was deferred for as long as 2 or 3 months.3 Additional recommendations were made to reserve inpatient visits for suspicious lesions and high-risk cancers, postpone other nonessential and nonurgent appointments, and utilize telemedicine whenever possible.5
These recommendations led to great uncertainty and stress for patients and providers. Although numerous important variables, such as patient risk factors, severity of disease, availability of PPE and staff, and patient-to-provider transmission were considered when creating these guidelines, the patient’s experience likely was not a contributing factor.
COVID-19 Transmission During Mohs Surgery
There have been concerns that surgeons performing Mohs micrographic surgery (MMS) might be at an increased risk for COVID-19, given their close contact with high-risk sites (ie, nose, mouth) and cautery-generated aerosols; most of the estimated transmission risk associated with MMS has been based on head and neck surgery experience and publications.6-8 Tee and colleagues9 recently published their institution’s MMS COVID-19 preventive measures, which, to their knowledge, have prevented all intraoperative transmission of SARS-CoV-2, even in disease-positive patients. Currently, evidence is lacking to support a high risk for SARS-CoV-2 transmission during MMS when proper PPE and personal hygiene measures as well as strict infection control protocols—presurgical COVID-19 testing in high-risk cases, COVID-19 screening optimization, visitor restrictions, and appropriate disinfection between patients—are in place.
The Impact of Postponing Treatment on Patients
Although studies have focused on the effects of the COVID-19 pandemic on physicians practicing MMS,10 little is known about the effects of delays in skin cancer treatment on patients. A survey conducted in the United Kingdom investigating the patient’s perspective found that patients expressed worry and concern about the possibility that their MMS would be postponed and greatly appreciated continuation of treatment during the pandemic.11
Other medical specialties have reported their patient experiences during the pandemic. In a study examining patient perception of postponed surgical treatment of pelvic floor disorders due to COVID-19, nearly half of survey respondents were unhappy with the delay in receiving care. Furthermore, patients who reported being unhappy were more likely to report feelings of isolation and anxiety because their surgery was postponed.12 In another study involving patients with lung cancer, 9.1% (N=15) of patients postponed their treatment during the COVID-19 pandemic because of pandemic-related anxiety.13
With the goal of improving care at our institution, we conducted a brief institutional review board–approved survey to evaluate how postponing MMS treatment due to the COVID-19 pandemic affected patients. All MMS patients undergoing surgery in June 2020 and July 2020 (N=99) were asked to complete our voluntary and anonymous 23-question survey in person during their procedure. We obtained 88 responses (response rate, 89%). Twenty percent of surveyed patients (n=18) reported that their MMS had been postponed; 78% of those whose MMS was postponed (n=14) indicated some level of anxiety during the waiting period. It was unclear which patients had their treatment postponed based on national guidelines and which ones elected to postpone surgery.
Tips for Health Care Providers
Patient-provider communication highlighting specific skin cancer risk and the risk vs benefit of postponing treatment might reduce anxiety and stress during the waiting period.14 A study found that COVID-19 posed a bigger threat than most noninvasive skin cancers; therefore, the authors of that study concluded that treatment for most skin cancers could be safely postponed.15 Specifically, those authors recommended prioritizing treatment for Merkel cell carcinoma, invasive SCC, and melanoma with positive margins or macroscopic residual disease. They proposed that all other skin cancers, including basal cell carcinoma, SCC in situ, and melanoma with negative margins and no macroscopic residual disease, could be safely delayed for as long as 3 months.15
For patients with multiple risk factors for COVID-19–related morbidity or mortality, delaying skin cancer treatment likely has less risk than contracting the virus.15 This information should be communicated with patients. Investigation of specific patient concerns is warranted, and case-by-case evaluation of patients’ risk factors and skin cancer risk should be considered.
Based on the current, though limited, literature, delaying medical treatment can have a negative impact on the patient experience. Furthermore, proper precautions have been shown to limit intraoperative transmission of SARS-CoV-2 during MMS, but research is lacking. Practitioners should utilize shared decision-making and evaluate a given patient’s risk factors and concerns when deciding whether to postpone treatment. We encourage other institutions to evaluate the effects that delaying MMS has had on their patients, as further studies would improve understanding of patients’ experiences during a pandemic and potentially influence future dermatology guidelines.
- Center for Disease Control and Prevention. COVID-19. Accessed April 20, 2021. https://www.cdc.gov/coronavirus/2019-ncov/index.html
- American College of Mohs Surgery. Mohs surgery ambulatory protocol during COVID pandemic (version 6-3-20). June 4, 2020. Accessed April 20, 2021. http://staging.mohscollege.org/UserFiles/AM20/Member%20Alert/MohsSurgeryAmbulatoryProtocolDuringCOVIDPandemicFinal.pdf
- COVID-19 resources. National Comprehensive Cancer Network website. Accessed April 20, 2021. https://www.nccn.org/covid-19
- Narla S, Alam M, Ozog DM, et al. American Society of Dermatologic Surgery Association (ASDSA) and American Society for Laser Medicine & Surgery (ASLMS) guidance for cosmetic dermatology practices during COVID-19. Updated January 11, 2021. Accessed April 10, 2021. https://www.asds.net/Portals/0/PDF/asdsa/asdsa-aslms-cosmetic-reopening-guidance.pdf
- Geskin LJ, Trager MH, Aasi SZ, et al. Perspectives on the recommendations for skin cancer management during the COVID-19 pandemic.J Am Acad Dermatol. 2020;83:295-296. doi:10.1016/j.jaad.2020.05.002
- Yuan JT, Jiang SIB. Urgent safety considerations for dermatologic surgeons in the COVID-19 pandemic. Dermatol Online J. 2020;26:1. Accessed April 20, 2021. http://escholarship.org/uc/item/2qr3w771
- Otolaryngologists may contract COVID-19 during surgery. ENTtoday. March 20, 2020. Accessed April 20, 2021. https://www.enttoday.org/article/otolaryngologists-may-contract-covid-19-during-surgery/
- Howard BE. High-risk aerosol-generating procedures in COVID-19: respiratory protective equipment considerations. Otolaryngol Head Neck Surg. 2020;163:98-103. doi:10.1177/0194599820927335
- Tee MW, Stewart C, Aliessa S, et al. Dermatological surgery during the COVID-19 pandemic: experience of a large academic center. J Am Acad Dermatol. 2021;84:1094-1096. doi:10.1016/j.jaad.2020.12.003
- Hooper J, Feng H. The impact of COVID-19 on micrographic surgery and dermatologic oncology fellows. Dermatol Surg. 2020;46:1762-1763. doi:10.1097/DSS.0000000000002766
- Nicholson P, Ali FR, Patalay R, et al. Patient perceptions of Mohs micrographic surgery during the COVID-19 pandemic and lessons for the next outbreak. Clin Exp Dermatol. 2021;46:179-180. doi:10.1111/ced.14423
- Mou T, Brown O, Gillingham A, et al. Patients’ perceptions on surgical care suspension for pelvic floor disorders during the COVID-19 pandemic. Female Pelvic Med Reconstr Surg. 2020;26:477-482. doi:10.1097/SPV.0000000000000918
- Fujita K, Ito T, Saito Z, et al. Impact of COVID-19 pandemic on lung cancer treatment scheduling. Thorac Cancer. 2020;11:2983-2986. doi:10.1111/1759-7714.13615
- Nikumb VB, Banerjee A, Kaur G, et al. Impact of doctor-patient communication on preoperative anxiety: study at industrial township, Pimpri, Pune. Ind Psychiatry J. 2009;18:19-21. doi:10.4103/0972-6748.57852
- Baumann BC, MacArthur KM, Brewer JD, et al. Management of primary skin cancer during a pandemic: multidisciplinary recommendations. Cancer. 2020;126:3900-3906. doi:10.1002/cncr.32969
- Center for Disease Control and Prevention. COVID-19. Accessed April 20, 2021. https://www.cdc.gov/coronavirus/2019-ncov/index.html
- American College of Mohs Surgery. Mohs surgery ambulatory protocol during COVID pandemic (version 6-3-20). June 4, 2020. Accessed April 20, 2021. http://staging.mohscollege.org/UserFiles/AM20/Member%20Alert/MohsSurgeryAmbulatoryProtocolDuringCOVIDPandemicFinal.pdf
- COVID-19 resources. National Comprehensive Cancer Network website. Accessed April 20, 2021. https://www.nccn.org/covid-19
- Narla S, Alam M, Ozog DM, et al. American Society of Dermatologic Surgery Association (ASDSA) and American Society for Laser Medicine & Surgery (ASLMS) guidance for cosmetic dermatology practices during COVID-19. Updated January 11, 2021. Accessed April 10, 2021. https://www.asds.net/Portals/0/PDF/asdsa/asdsa-aslms-cosmetic-reopening-guidance.pdf
- Geskin LJ, Trager MH, Aasi SZ, et al. Perspectives on the recommendations for skin cancer management during the COVID-19 pandemic.J Am Acad Dermatol. 2020;83:295-296. doi:10.1016/j.jaad.2020.05.002
- Yuan JT, Jiang SIB. Urgent safety considerations for dermatologic surgeons in the COVID-19 pandemic. Dermatol Online J. 2020;26:1. Accessed April 20, 2021. http://escholarship.org/uc/item/2qr3w771
- Otolaryngologists may contract COVID-19 during surgery. ENTtoday. March 20, 2020. Accessed April 20, 2021. https://www.enttoday.org/article/otolaryngologists-may-contract-covid-19-during-surgery/
- Howard BE. High-risk aerosol-generating procedures in COVID-19: respiratory protective equipment considerations. Otolaryngol Head Neck Surg. 2020;163:98-103. doi:10.1177/0194599820927335
- Tee MW, Stewart C, Aliessa S, et al. Dermatological surgery during the COVID-19 pandemic: experience of a large academic center. J Am Acad Dermatol. 2021;84:1094-1096. doi:10.1016/j.jaad.2020.12.003
- Hooper J, Feng H. The impact of COVID-19 on micrographic surgery and dermatologic oncology fellows. Dermatol Surg. 2020;46:1762-1763. doi:10.1097/DSS.0000000000002766
- Nicholson P, Ali FR, Patalay R, et al. Patient perceptions of Mohs micrographic surgery during the COVID-19 pandemic and lessons for the next outbreak. Clin Exp Dermatol. 2021;46:179-180. doi:10.1111/ced.14423
- Mou T, Brown O, Gillingham A, et al. Patients’ perceptions on surgical care suspension for pelvic floor disorders during the COVID-19 pandemic. Female Pelvic Med Reconstr Surg. 2020;26:477-482. doi:10.1097/SPV.0000000000000918
- Fujita K, Ito T, Saito Z, et al. Impact of COVID-19 pandemic on lung cancer treatment scheduling. Thorac Cancer. 2020;11:2983-2986. doi:10.1111/1759-7714.13615
- Nikumb VB, Banerjee A, Kaur G, et al. Impact of doctor-patient communication on preoperative anxiety: study at industrial township, Pimpri, Pune. Ind Psychiatry J. 2009;18:19-21. doi:10.4103/0972-6748.57852
- Baumann BC, MacArthur KM, Brewer JD, et al. Management of primary skin cancer during a pandemic: multidisciplinary recommendations. Cancer. 2020;126:3900-3906. doi:10.1002/cncr.32969
Practice Points
- There is little evidence that supports a high risk for SARS-CoV-2 transmission during Mohs micrographic surgery when proper personal protective equipment and strict infection control protocols are in place.
- The effects of treatment delays due to COVID-19 on the patient experience have not been well studied, but the limited literature suggests a negative association.
- Shared decision-making and evaluation of individual patient risk factors and concerns should be considered when deciding whether to postpone skin cancer treatment.