Case Letter

To the Editor:

A 33-year-old woman who was otherwise healthy presented with itchy wheals that developed within 15 to 20 minutes of removing leg hair with an electric epilator. Furthermore, she reported that small hives often developed after waxing the legs with warm wax. All lesions spontaneously disappeared within 3 hours; depilatory creams and shaving did not trigger urticarial lesions. She had no history of atopy or prior episodes of spontaneous urticaria. Symptomatic dermographism also was not reported. Classic physical stimuli that could be associated with the use of an electric epilator, such as heat, vibration, and pressure, did not elicit lesions.

Physical examination showed no active lesions. Dermographism was not inducible by stroking the patient’s skin with a blunt object. She brought personal photographs that showed erythematous follicular hives measuring 1 to 3 mm in diameter located on the distal legs (Figure). In accordance with these findings, she was diagnosed with an unusual form of physical urticaria likely resulting from hair traction and was prescribed oral H1 antihistamines to be taken a few days before and after hair removal.

Özkaya and Yazganog˘lu¹ proposed that follicular dermographism should be differentiated from physical urticaria. This variant of dermographism is characterized by discrete urticarial papules appearing at the location of hair follicles after having stroked the skin with a blunt object.^1,8 These lesions usually disappear within 30 minutes.⁸ Given that none of the reported cases presented dermographism on examination tests, we agree with Özkaya and Yazganog˘lu¹ that this phenomenon of traction urticaria likely is a different condition than follicular dermographism, even though intraindividual variability sometimes can be seen in dermographism skin tests.⁷

We present a unique form of urticaria that easily can be misdiagnosed as pseudofolliculitis, which tends to be more commonly associated with the use of electric epilators.

To the Editor:

A 33-year-old woman who was otherwise healthy presented with itchy wheals that developed within 15 to 20 minutes of removing leg hair with an electric epilator. Furthermore, she reported that small hives often developed after waxing the legs with warm wax. All lesions spontaneously disappeared within 3 hours; depilatory creams and shaving did not trigger urticarial lesions. She had no history of atopy or prior episodes of spontaneous urticaria. Symptomatic dermographism also was not reported. Classic physical stimuli that could be associated with the use of an electric epilator, such as heat, vibration, and pressure, did not elicit lesions.

Physical examination showed no active lesions. Dermographism was not inducible by stroking the patient’s skin with a blunt object. She brought personal photographs that showed erythematous follicular hives measuring 1 to 3 mm in diameter located on the distal legs (Figure). In accordance with these findings, she was diagnosed with an unusual form of physical urticaria likely resulting from hair traction and was prescribed oral H1 antihistamines to be taken a few days before and after hair removal.

Özkaya and Yazganog˘lu¹ proposed that follicular dermographism should be differentiated from physical urticaria. This variant of dermographism is characterized by discrete urticarial papules appearing at the location of hair follicles after having stroked the skin with a blunt object.^1,8 These lesions usually disappear within 30 minutes.⁸ Given that none of the reported cases presented dermographism on examination tests, we agree with Özkaya and Yazganog˘lu¹ that this phenomenon of traction urticaria likely is a different condition than follicular dermographism, even though intraindividual variability sometimes can be seen in dermographism skin tests.⁷

We present a unique form of urticaria that easily can be misdiagnosed as pseudofolliculitis, which tends to be more commonly associated with the use of electric epilators.

To the Editor:

A 33-year-old woman who was otherwise healthy presented with itchy wheals that developed within 15 to 20 minutes of removing leg hair with an electric epilator. Furthermore, she reported that small hives often developed after waxing the legs with warm wax. All lesions spontaneously disappeared within 3 hours; depilatory creams and shaving did not trigger urticarial lesions. She had no history of atopy or prior episodes of spontaneous urticaria. Symptomatic dermographism also was not reported. Classic physical stimuli that could be associated with the use of an electric epilator, such as heat, vibration, and pressure, did not elicit lesions.

Physical examination showed no active lesions. Dermographism was not inducible by stroking the patient’s skin with a blunt object. She brought personal photographs that showed erythematous follicular hives measuring 1 to 3 mm in diameter located on the distal legs (Figure). In accordance with these findings, she was diagnosed with an unusual form of physical urticaria likely resulting from hair traction and was prescribed oral H1 antihistamines to be taken a few days before and after hair removal.

Özkaya and Yazganog˘lu¹ proposed that follicular dermographism should be differentiated from physical urticaria. This variant of dermographism is characterized by discrete urticarial papules appearing at the location of hair follicles after having stroked the skin with a blunt object.^1,8 These lesions usually disappear within 30 minutes.⁸ Given that none of the reported cases presented dermographism on examination tests, we agree with Özkaya and Yazganog˘lu¹ that this phenomenon of traction urticaria likely is a different condition than follicular dermographism, even though intraindividual variability sometimes can be seen in dermographism skin tests.⁷

We present a unique form of urticaria that easily can be misdiagnosed as pseudofolliculitis, which tends to be more commonly associated with the use of electric epilators.

To the Editor:

Cutaneous metastases occur more often in the setting of breast carcinoma than other malignancies in women.¹ Although interventions are aimed at halting disease progression, cutaneous metastases indicate widespread disease and are associated with poor prognosis. We present the case of a patient with metastatic breast adenocarcinoma who developed cutaneous metastasis on the trunk after a double mastectomy. The widespread distribution and wide range of clinical manifestations are unique.

An 81-year-old woman presented to the dermatology office for evaluation of a skin eruption that started along a mastectomy scar on the left breast a few months postoperatively. She had a history of stage IV breast adenocarcinoma metastatic to the chest wall that was treated with a double mastectomy 2 years prior. The patient denied associated pain or pruritus and mainly was concerned with the cosmetic appearance. At the time of the initial diagnosis of breast adenocarcinoma, the patient was offered chemotherapy, which she did not tolerate. The patient opted against radiation therapy, as she preferred a more natural approach, such as anticancer shakes, which she was taking from a homeopathic source. She was unaware of the ingredients used in the shakes.

Physical examination revealed multiple grouped, firm, purpuric papules, nodules, and pseudovesicles on a background of violaceous erythema on the chest, abdomen, and flank (Figure 1). The background erythema had a mosaic pattern that extended toward the central back (Figure 2). A scoop shave biopsy of one of the purpuric nodules revealed highly atypical cells with abundant cytoplasm, large nuclei, and prominent nucleoli (Figure 3). Focally, the cells appeared to form glandular structures. Numerous atypical mitotic figures were present. Lymphatic invasion and microcalcifications were identified (Figure 3 [inset]). Immunohistochemical staining for cytokeratin 7 and gross cystic disease fluid protein 15 were strongly positive (Figure 4). Based on the histopathologic and immunohistochemical findings, a diagnosis of cutaneous metastatic breast adenocarcinoma was made. The patient opted to continue the homeopathic anticancer shakes and was subsequently lost to follow-up.

Cutaneous metastases of internal malignancies make up only 2% of all skin tumors,¹ making them relatively uncommon in the dermatologic setting. However, cutaneous metastasis occurs in 23.9% of patients with breast carcinoma, making it the most common tumor after malignant melanoma to metastasize to the skin.² The most common sites for breast carcinoma cutaneous metastasis (BCCM) are the chest wall and abdomen; other sites include the head/neck region and the extremities. The clinical presentation of BCCM varies depending on the mode of dissemination—lymphatic, hematogenous, contiguous growth, or iatrogenic implantation. The most common presentation is nodular carcinoma (47%–80%).^2,3 Other presentations include carcinoma telangiectoides (8%–11%),^2,3 alopecia neoplastica (2%–12%),^2,3 and carcinoma erysipeloides (3%–6%).^2,3 Carcinoma en cuirasse is rare.³

Nodular BCCM may present as firm solitary or grouped papules and nodules that are painless and range in color from flesh colored or pink to red-brown. Histologically, they are composed of atypical neoplastic cells arranged in small nests and cords, usually in a single-file line within the collagen bundles of the dermis.⁴ Carcinoma telangiectoides is characterized by its violaceous hue due to the dilated vascular channels. The lesions are purpuric papules and pseudovesicles appearing on an erythematous base, most commonly contiguous with the surgical scar. Histologically, collections of atypical tumor cells and erythrocytes are present along with dilated blood vessels in the papillary dermis.² Alopecia neoplastica presents as singular or grouped cicatricial patches of hair loss. Lesions of carcinoma erysipeloides present as warm, erythematous, tender, well-defined patches or plaques. Carcinoma en cuirasse is characterized by an erythematous sclerodermoid plaque on the chest wall.²

Our patient’s presentation was unique due to the widespread distribution, unusual pattern, and variable clinical morphologies of the cutaneous metastases. Our patient had findings of both carcinoma telangiectoides and nodular carcinoma. The mosaic violaceous erythema extending toward the mid-back rarely is reported in the literature and indicates extensive intravascular spread of tumor cells in the dermis.

Metastatic breast cancer is associated with a poor prognosis because it typically occurs in advanced stages and often does not respond to treatment.⁵ Although chemotherapy, hormonal therapy, and/or radiation therapy may improve survival, the choice in regimen is guided by cancer histology as well as prior treatments. In our case, the patient chose to continue her homeopathic therapy.

To the Editor:

Cutaneous metastases occur more often in the setting of breast carcinoma than other malignancies in women.¹ Although interventions are aimed at halting disease progression, cutaneous metastases indicate widespread disease and are associated with poor prognosis. We present the case of a patient with metastatic breast adenocarcinoma who developed cutaneous metastasis on the trunk after a double mastectomy. The widespread distribution and wide range of clinical manifestations are unique.

An 81-year-old woman presented to the dermatology office for evaluation of a skin eruption that started along a mastectomy scar on the left breast a few months postoperatively. She had a history of stage IV breast adenocarcinoma metastatic to the chest wall that was treated with a double mastectomy 2 years prior. The patient denied associated pain or pruritus and mainly was concerned with the cosmetic appearance. At the time of the initial diagnosis of breast adenocarcinoma, the patient was offered chemotherapy, which she did not tolerate. The patient opted against radiation therapy, as she preferred a more natural approach, such as anticancer shakes, which she was taking from a homeopathic source. She was unaware of the ingredients used in the shakes.

Physical examination revealed multiple grouped, firm, purpuric papules, nodules, and pseudovesicles on a background of violaceous erythema on the chest, abdomen, and flank (Figure 1). The background erythema had a mosaic pattern that extended toward the central back (Figure 2). A scoop shave biopsy of one of the purpuric nodules revealed highly atypical cells with abundant cytoplasm, large nuclei, and prominent nucleoli (Figure 3). Focally, the cells appeared to form glandular structures. Numerous atypical mitotic figures were present. Lymphatic invasion and microcalcifications were identified (Figure 3 [inset]). Immunohistochemical staining for cytokeratin 7 and gross cystic disease fluid protein 15 were strongly positive (Figure 4). Based on the histopathologic and immunohistochemical findings, a diagnosis of cutaneous metastatic breast adenocarcinoma was made. The patient opted to continue the homeopathic anticancer shakes and was subsequently lost to follow-up.

Cutaneous metastases of internal malignancies make up only 2% of all skin tumors,¹ making them relatively uncommon in the dermatologic setting. However, cutaneous metastasis occurs in 23.9% of patients with breast carcinoma, making it the most common tumor after malignant melanoma to metastasize to the skin.² The most common sites for breast carcinoma cutaneous metastasis (BCCM) are the chest wall and abdomen; other sites include the head/neck region and the extremities. The clinical presentation of BCCM varies depending on the mode of dissemination—lymphatic, hematogenous, contiguous growth, or iatrogenic implantation. The most common presentation is nodular carcinoma (47%–80%).^2,3 Other presentations include carcinoma telangiectoides (8%–11%),^2,3 alopecia neoplastica (2%–12%),^2,3 and carcinoma erysipeloides (3%–6%).^2,3 Carcinoma en cuirasse is rare.³

Nodular BCCM may present as firm solitary or grouped papules and nodules that are painless and range in color from flesh colored or pink to red-brown. Histologically, they are composed of atypical neoplastic cells arranged in small nests and cords, usually in a single-file line within the collagen bundles of the dermis.⁴ Carcinoma telangiectoides is characterized by its violaceous hue due to the dilated vascular channels. The lesions are purpuric papules and pseudovesicles appearing on an erythematous base, most commonly contiguous with the surgical scar. Histologically, collections of atypical tumor cells and erythrocytes are present along with dilated blood vessels in the papillary dermis.² Alopecia neoplastica presents as singular or grouped cicatricial patches of hair loss. Lesions of carcinoma erysipeloides present as warm, erythematous, tender, well-defined patches or plaques. Carcinoma en cuirasse is characterized by an erythematous sclerodermoid plaque on the chest wall.²

Our patient’s presentation was unique due to the widespread distribution, unusual pattern, and variable clinical morphologies of the cutaneous metastases. Our patient had findings of both carcinoma telangiectoides and nodular carcinoma. The mosaic violaceous erythema extending toward the mid-back rarely is reported in the literature and indicates extensive intravascular spread of tumor cells in the dermis.

Metastatic breast cancer is associated with a poor prognosis because it typically occurs in advanced stages and often does not respond to treatment.⁵ Although chemotherapy, hormonal therapy, and/or radiation therapy may improve survival, the choice in regimen is guided by cancer histology as well as prior treatments. In our case, the patient chose to continue her homeopathic therapy.

To the Editor:

Cutaneous metastases occur more often in the setting of breast carcinoma than other malignancies in women.¹ Although interventions are aimed at halting disease progression, cutaneous metastases indicate widespread disease and are associated with poor prognosis. We present the case of a patient with metastatic breast adenocarcinoma who developed cutaneous metastasis on the trunk after a double mastectomy. The widespread distribution and wide range of clinical manifestations are unique.

An 81-year-old woman presented to the dermatology office for evaluation of a skin eruption that started along a mastectomy scar on the left breast a few months postoperatively. She had a history of stage IV breast adenocarcinoma metastatic to the chest wall that was treated with a double mastectomy 2 years prior. The patient denied associated pain or pruritus and mainly was concerned with the cosmetic appearance. At the time of the initial diagnosis of breast adenocarcinoma, the patient was offered chemotherapy, which she did not tolerate. The patient opted against radiation therapy, as she preferred a more natural approach, such as anticancer shakes, which she was taking from a homeopathic source. She was unaware of the ingredients used in the shakes.

Physical examination revealed multiple grouped, firm, purpuric papules, nodules, and pseudovesicles on a background of violaceous erythema on the chest, abdomen, and flank (Figure 1). The background erythema had a mosaic pattern that extended toward the central back (Figure 2). A scoop shave biopsy of one of the purpuric nodules revealed highly atypical cells with abundant cytoplasm, large nuclei, and prominent nucleoli (Figure 3). Focally, the cells appeared to form glandular structures. Numerous atypical mitotic figures were present. Lymphatic invasion and microcalcifications were identified (Figure 3 [inset]). Immunohistochemical staining for cytokeratin 7 and gross cystic disease fluid protein 15 were strongly positive (Figure 4). Based on the histopathologic and immunohistochemical findings, a diagnosis of cutaneous metastatic breast adenocarcinoma was made. The patient opted to continue the homeopathic anticancer shakes and was subsequently lost to follow-up.

Cutaneous metastases of internal malignancies make up only 2% of all skin tumors,¹ making them relatively uncommon in the dermatologic setting. However, cutaneous metastasis occurs in 23.9% of patients with breast carcinoma, making it the most common tumor after malignant melanoma to metastasize to the skin.² The most common sites for breast carcinoma cutaneous metastasis (BCCM) are the chest wall and abdomen; other sites include the head/neck region and the extremities. The clinical presentation of BCCM varies depending on the mode of dissemination—lymphatic, hematogenous, contiguous growth, or iatrogenic implantation. The most common presentation is nodular carcinoma (47%–80%).^2,3 Other presentations include carcinoma telangiectoides (8%–11%),^2,3 alopecia neoplastica (2%–12%),^2,3 and carcinoma erysipeloides (3%–6%).^2,3 Carcinoma en cuirasse is rare.³

Nodular BCCM may present as firm solitary or grouped papules and nodules that are painless and range in color from flesh colored or pink to red-brown. Histologically, they are composed of atypical neoplastic cells arranged in small nests and cords, usually in a single-file line within the collagen bundles of the dermis.⁴ Carcinoma telangiectoides is characterized by its violaceous hue due to the dilated vascular channels. The lesions are purpuric papules and pseudovesicles appearing on an erythematous base, most commonly contiguous with the surgical scar. Histologically, collections of atypical tumor cells and erythrocytes are present along with dilated blood vessels in the papillary dermis.² Alopecia neoplastica presents as singular or grouped cicatricial patches of hair loss. Lesions of carcinoma erysipeloides present as warm, erythematous, tender, well-defined patches or plaques. Carcinoma en cuirasse is characterized by an erythematous sclerodermoid plaque on the chest wall.²

Our patient’s presentation was unique due to the widespread distribution, unusual pattern, and variable clinical morphologies of the cutaneous metastases. Our patient had findings of both carcinoma telangiectoides and nodular carcinoma. The mosaic violaceous erythema extending toward the mid-back rarely is reported in the literature and indicates extensive intravascular spread of tumor cells in the dermis.

Metastatic breast cancer is associated with a poor prognosis because it typically occurs in advanced stages and often does not respond to treatment.⁵ Although chemotherapy, hormonal therapy, and/or radiation therapy may improve survival, the choice in regimen is guided by cancer histology as well as prior treatments. In our case, the patient chose to continue her homeopathic therapy.

To the Editor:

The term paraffinoma refers to a chronic granulomatous response to injection of paraffin, silicone, or other mineral oils into skin and soft tissue. Paraffinomas develop when the material is injected into the skin for cosmetic purposes to augment or enhance one’s appearance. Although they may occur in any location, the most common sites include the breasts and buttocks. The penis is a rare but emerging site for paraffinomas.^1-3 We present a rare case of recurrence of a penile paraffinoma following surgical resection.

A 26-year-old uncircumcised Trinidadian man presented with a 5-cm, exquisitely tender tumor involving the penile shaft and median raphe that rapidly evolved over the course of 3 weeks (Figure 1). He presented with inability to urinate, attain an erection, or ambulate without notable tenderness. Additionally, he developed swelling of the penis and surrounding tissue. He had no other medical comorbidities; however, 1 year prior he presented to a urologist with a 1-cm nodule involving the median raphe that was surgically resected and required circumcision. Biopsy at the time of his surgical procedure revealed an exuberant foreign body giant cell reaction with surrounding empty spaces in the dermis resembling Swiss cheese, consistent with a paraffinoma (Figure 2). The recurrent tumor, which was 5 times the size of the initial nodule, was biopsied. Again, histopathologic findings were consistent with a paraffinoma with extensive dermal fibrosis and absence of polarizable material.

In 1899, mineral oil was first injected into male genitalia to restore architecture in a patient’s testicles following bilateral orchiectomy. After the success of this endeavor, mineral oil injections were used as filler for other defects.³ However, by 1906 the complications of these injections became public knowledge when 2 patients developed subcutaneous nodules after receiving injections for facial wrinkles.² Despite public knowledge of these complications, penile paraffin injections continued to occur both in medical and eventually nonmedical settings.

In 1947, Quérnu and Pérol⁶ described 6 penile paraffinoma cases outside the United States. Patients had petroleum jelly injections that eventuated in penile paraffinomas, and all of them lost the ability to attain an erection.⁶ Four years later, Bradley and Ehrgott⁷ described a case of penile paraffinoma likely caused by application of paraffin in association with occupational exposure. In 1956, May and Pickering⁸ cited a case of penile paraffinoma affecting the entire penile shaft in which the patient had undergone paraffin injection 7 years prior to treat premature ejaculation. Unfortunately, the injection resulted in a painful and unsatisfactory erection without resolution of premature ejaculation.⁸ Lee et al⁹ analyzed 26 cases of penile paraffinomas that occurred from 1981 to 1993. They found that all patients underwent injections of paraffin or petroleum jelly performed by nonmedical personnel with the predominant goal of enhancing penis size. Within 18.5 months of injection, 19 patients already experienced tenderness at the injection site. The remaining 7 patients experienced penile skin discoloration and abnormal contouring of the penis. Biopsy specimens revealed hyaline necrosis of subcutaneous adipose septa, cystlike spaces throughout involved tissue, and macrophages engulfing adipose tissue were found near blood vessels.⁹ In 2007, Eandi et al⁴ reported a case of penile paraffinoma with a 40-year delay of onset. Four years later, Manny et al¹⁰ reported penile paraffinomas in 3 Laotian men who injected a mineral oil.

Currently, paraffin injections are uncommon but still are being performed in some countries in Eastern Europe and the Far East¹¹; they rarely are reported in the United States. Injections can occur in unusual sites such as the knee, and paraffinomas can develop many years after the procedure.¹² Additionally, paraffinomas can obscure proper diagnosis of carcinomas, as described by Lee et al¹³ in a case in which a cervical paraffin injection confounded the diagnosis of a thyroid tumor. Furthermore, these injections usually are performed by nonmedical personnel and typically are repeated multiple times to reach cosmetic goals, rendering the patient vulnerable to early complications including allergic reactions, paraphimosis, infection, and inflammation.³

The clinical presentation of a penile paraffinoma may be a mimicker of several different entities, which are important to consider in the evaluation of a presenting patient. Infectious etiologies must be considered including lymphogranuloma venereum, granuloma inguinale, atypical mycobacteria, lupus vulgaris, and sexually transmitted infections. Importantly, neoplasms must be ruled out including squamous cell carcinoma, soft tissue sarcomas, melanoma, adenocarcinoma, or metastasis. Lymphedema, prior surgical procedures, trauma, and inflammatory etiologies also are in the differential diagnosis.¹⁴ Nonetheless, physicians must have a high clinical suspicion in the evaluation of a possible paraffinoma, as patients may not be forthcoming with relevant clinical history regarding a prior injection to the affected site, particularly if the injection occurred many years ago. As such, the patient may not consider this history relevant or may not even remember the event occurred, as was observed in our case. Furthermore, embarrassment, social taboo, and stigma may be associated with the behavior of undergoing injections in nonclinical settings without medical supervision.¹⁵

Patients may be motivated to undergo dangerous procedures to potentially alter their appearance due to perceived enhanced sexual ability, influence by loved ones, cultural rituals, and societal pressure.^15,16 Furthermore, patients may not be aware of the material being injected or the volume. Given that these injections often are used with the goal of cosmetic enhancement, biopsies in cosmetically sensitive areas must be given careful consideration, and a thorough clinical history must support the decision to pursue a biopsy to obtain a definitive diagnosis.

The definitive diagnosis of a paraffinoma is determined by histopathology. However, the use of imaging modalities such as magnetic resonance imaging and computed tomography have been employed to delineate the extent of involvement. Imaging studies allow for surgical planning and may assist in narrowing a differential diagnosis.¹⁷ Currently, wide and complete surgical resection is the only definitive treatment of paraffinomas, including penile paraffinomas, as there is no evidence of spontaneous regression.³ A report of a reconstructive surgery involving penile resurfacing without T-style anastomosis has been found effective at preventing necrosis of the ventral penile skin. Not all paraffinomas behave similarly, and there is no reliable method to determine which paraffinoma may possess a more aggressive clinical course compared to those which have a more indolent course.¹⁸ As such, early detection is critical in the management of paraffinomas, especially in anatomic locations where tissue preservation is of utmost importance. In the case of a large penile paraffinoma with the ability to destroy vital urologic and reproductive function, physicians must consider prevention of recurrent episodes through suppression of inflammation and fibrosis with doxycycline and nicotinamide.¹⁹ Other medical treatments reported with varying success include corticosteroids, imiquimod, and isotretinoin.^19-24 Employing adjunctive medical treatment may decrease the size of the mass, reducing the surgical defect size and preserving tissue vitality. Ultimately, the most crucial aspect in treatment is prevention, as injection of foreign materials elicits a foreign body response and can lead to notable morbidity.

To the Editor:

The term paraffinoma refers to a chronic granulomatous response to injection of paraffin, silicone, or other mineral oils into skin and soft tissue. Paraffinomas develop when the material is injected into the skin for cosmetic purposes to augment or enhance one’s appearance. Although they may occur in any location, the most common sites include the breasts and buttocks. The penis is a rare but emerging site for paraffinomas.^1-3 We present a rare case of recurrence of a penile paraffinoma following surgical resection.

A 26-year-old uncircumcised Trinidadian man presented with a 5-cm, exquisitely tender tumor involving the penile shaft and median raphe that rapidly evolved over the course of 3 weeks (Figure 1). He presented with inability to urinate, attain an erection, or ambulate without notable tenderness. Additionally, he developed swelling of the penis and surrounding tissue. He had no other medical comorbidities; however, 1 year prior he presented to a urologist with a 1-cm nodule involving the median raphe that was surgically resected and required circumcision. Biopsy at the time of his surgical procedure revealed an exuberant foreign body giant cell reaction with surrounding empty spaces in the dermis resembling Swiss cheese, consistent with a paraffinoma (Figure 2). The recurrent tumor, which was 5 times the size of the initial nodule, was biopsied. Again, histopathologic findings were consistent with a paraffinoma with extensive dermal fibrosis and absence of polarizable material.

In 1899, mineral oil was first injected into male genitalia to restore architecture in a patient’s testicles following bilateral orchiectomy. After the success of this endeavor, mineral oil injections were used as filler for other defects.³ However, by 1906 the complications of these injections became public knowledge when 2 patients developed subcutaneous nodules after receiving injections for facial wrinkles.² Despite public knowledge of these complications, penile paraffin injections continued to occur both in medical and eventually nonmedical settings.

In 1947, Quérnu and Pérol⁶ described 6 penile paraffinoma cases outside the United States. Patients had petroleum jelly injections that eventuated in penile paraffinomas, and all of them lost the ability to attain an erection.⁶ Four years later, Bradley and Ehrgott⁷ described a case of penile paraffinoma likely caused by application of paraffin in association with occupational exposure. In 1956, May and Pickering⁸ cited a case of penile paraffinoma affecting the entire penile shaft in which the patient had undergone paraffin injection 7 years prior to treat premature ejaculation. Unfortunately, the injection resulted in a painful and unsatisfactory erection without resolution of premature ejaculation.⁸ Lee et al⁹ analyzed 26 cases of penile paraffinomas that occurred from 1981 to 1993. They found that all patients underwent injections of paraffin or petroleum jelly performed by nonmedical personnel with the predominant goal of enhancing penis size. Within 18.5 months of injection, 19 patients already experienced tenderness at the injection site. The remaining 7 patients experienced penile skin discoloration and abnormal contouring of the penis. Biopsy specimens revealed hyaline necrosis of subcutaneous adipose septa, cystlike spaces throughout involved tissue, and macrophages engulfing adipose tissue were found near blood vessels.⁹ In 2007, Eandi et al⁴ reported a case of penile paraffinoma with a 40-year delay of onset. Four years later, Manny et al¹⁰ reported penile paraffinomas in 3 Laotian men who injected a mineral oil.

Currently, paraffin injections are uncommon but still are being performed in some countries in Eastern Europe and the Far East¹¹; they rarely are reported in the United States. Injections can occur in unusual sites such as the knee, and paraffinomas can develop many years after the procedure.¹² Additionally, paraffinomas can obscure proper diagnosis of carcinomas, as described by Lee et al¹³ in a case in which a cervical paraffin injection confounded the diagnosis of a thyroid tumor. Furthermore, these injections usually are performed by nonmedical personnel and typically are repeated multiple times to reach cosmetic goals, rendering the patient vulnerable to early complications including allergic reactions, paraphimosis, infection, and inflammation.³

The clinical presentation of a penile paraffinoma may be a mimicker of several different entities, which are important to consider in the evaluation of a presenting patient. Infectious etiologies must be considered including lymphogranuloma venereum, granuloma inguinale, atypical mycobacteria, lupus vulgaris, and sexually transmitted infections. Importantly, neoplasms must be ruled out including squamous cell carcinoma, soft tissue sarcomas, melanoma, adenocarcinoma, or metastasis. Lymphedema, prior surgical procedures, trauma, and inflammatory etiologies also are in the differential diagnosis.¹⁴ Nonetheless, physicians must have a high clinical suspicion in the evaluation of a possible paraffinoma, as patients may not be forthcoming with relevant clinical history regarding a prior injection to the affected site, particularly if the injection occurred many years ago. As such, the patient may not consider this history relevant or may not even remember the event occurred, as was observed in our case. Furthermore, embarrassment, social taboo, and stigma may be associated with the behavior of undergoing injections in nonclinical settings without medical supervision.¹⁵

Patients may be motivated to undergo dangerous procedures to potentially alter their appearance due to perceived enhanced sexual ability, influence by loved ones, cultural rituals, and societal pressure.^15,16 Furthermore, patients may not be aware of the material being injected or the volume. Given that these injections often are used with the goal of cosmetic enhancement, biopsies in cosmetically sensitive areas must be given careful consideration, and a thorough clinical history must support the decision to pursue a biopsy to obtain a definitive diagnosis.

The definitive diagnosis of a paraffinoma is determined by histopathology. However, the use of imaging modalities such as magnetic resonance imaging and computed tomography have been employed to delineate the extent of involvement. Imaging studies allow for surgical planning and may assist in narrowing a differential diagnosis.¹⁷ Currently, wide and complete surgical resection is the only definitive treatment of paraffinomas, including penile paraffinomas, as there is no evidence of spontaneous regression.³ A report of a reconstructive surgery involving penile resurfacing without T-style anastomosis has been found effective at preventing necrosis of the ventral penile skin. Not all paraffinomas behave similarly, and there is no reliable method to determine which paraffinoma may possess a more aggressive clinical course compared to those which have a more indolent course.¹⁸ As such, early detection is critical in the management of paraffinomas, especially in anatomic locations where tissue preservation is of utmost importance. In the case of a large penile paraffinoma with the ability to destroy vital urologic and reproductive function, physicians must consider prevention of recurrent episodes through suppression of inflammation and fibrosis with doxycycline and nicotinamide.¹⁹ Other medical treatments reported with varying success include corticosteroids, imiquimod, and isotretinoin.^19-24 Employing adjunctive medical treatment may decrease the size of the mass, reducing the surgical defect size and preserving tissue vitality. Ultimately, the most crucial aspect in treatment is prevention, as injection of foreign materials elicits a foreign body response and can lead to notable morbidity.

To the Editor:

The term paraffinoma refers to a chronic granulomatous response to injection of paraffin, silicone, or other mineral oils into skin and soft tissue. Paraffinomas develop when the material is injected into the skin for cosmetic purposes to augment or enhance one’s appearance. Although they may occur in any location, the most common sites include the breasts and buttocks. The penis is a rare but emerging site for paraffinomas.^1-3 We present a rare case of recurrence of a penile paraffinoma following surgical resection.

A 26-year-old uncircumcised Trinidadian man presented with a 5-cm, exquisitely tender tumor involving the penile shaft and median raphe that rapidly evolved over the course of 3 weeks (Figure 1). He presented with inability to urinate, attain an erection, or ambulate without notable tenderness. Additionally, he developed swelling of the penis and surrounding tissue. He had no other medical comorbidities; however, 1 year prior he presented to a urologist with a 1-cm nodule involving the median raphe that was surgically resected and required circumcision. Biopsy at the time of his surgical procedure revealed an exuberant foreign body giant cell reaction with surrounding empty spaces in the dermis resembling Swiss cheese, consistent with a paraffinoma (Figure 2). The recurrent tumor, which was 5 times the size of the initial nodule, was biopsied. Again, histopathologic findings were consistent with a paraffinoma with extensive dermal fibrosis and absence of polarizable material.

In 1899, mineral oil was first injected into male genitalia to restore architecture in a patient’s testicles following bilateral orchiectomy. After the success of this endeavor, mineral oil injections were used as filler for other defects.³ However, by 1906 the complications of these injections became public knowledge when 2 patients developed subcutaneous nodules after receiving injections for facial wrinkles.² Despite public knowledge of these complications, penile paraffin injections continued to occur both in medical and eventually nonmedical settings.

In 1947, Quérnu and Pérol⁶ described 6 penile paraffinoma cases outside the United States. Patients had petroleum jelly injections that eventuated in penile paraffinomas, and all of them lost the ability to attain an erection.⁶ Four years later, Bradley and Ehrgott⁷ described a case of penile paraffinoma likely caused by application of paraffin in association with occupational exposure. In 1956, May and Pickering⁸ cited a case of penile paraffinoma affecting the entire penile shaft in which the patient had undergone paraffin injection 7 years prior to treat premature ejaculation. Unfortunately, the injection resulted in a painful and unsatisfactory erection without resolution of premature ejaculation.⁸ Lee et al⁹ analyzed 26 cases of penile paraffinomas that occurred from 1981 to 1993. They found that all patients underwent injections of paraffin or petroleum jelly performed by nonmedical personnel with the predominant goal of enhancing penis size. Within 18.5 months of injection, 19 patients already experienced tenderness at the injection site. The remaining 7 patients experienced penile skin discoloration and abnormal contouring of the penis. Biopsy specimens revealed hyaline necrosis of subcutaneous adipose septa, cystlike spaces throughout involved tissue, and macrophages engulfing adipose tissue were found near blood vessels.⁹ In 2007, Eandi et al⁴ reported a case of penile paraffinoma with a 40-year delay of onset. Four years later, Manny et al¹⁰ reported penile paraffinomas in 3 Laotian men who injected a mineral oil.

Currently, paraffin injections are uncommon but still are being performed in some countries in Eastern Europe and the Far East¹¹; they rarely are reported in the United States. Injections can occur in unusual sites such as the knee, and paraffinomas can develop many years after the procedure.¹² Additionally, paraffinomas can obscure proper diagnosis of carcinomas, as described by Lee et al¹³ in a case in which a cervical paraffin injection confounded the diagnosis of a thyroid tumor. Furthermore, these injections usually are performed by nonmedical personnel and typically are repeated multiple times to reach cosmetic goals, rendering the patient vulnerable to early complications including allergic reactions, paraphimosis, infection, and inflammation.³

The clinical presentation of a penile paraffinoma may be a mimicker of several different entities, which are important to consider in the evaluation of a presenting patient. Infectious etiologies must be considered including lymphogranuloma venereum, granuloma inguinale, atypical mycobacteria, lupus vulgaris, and sexually transmitted infections. Importantly, neoplasms must be ruled out including squamous cell carcinoma, soft tissue sarcomas, melanoma, adenocarcinoma, or metastasis. Lymphedema, prior surgical procedures, trauma, and inflammatory etiologies also are in the differential diagnosis.¹⁴ Nonetheless, physicians must have a high clinical suspicion in the evaluation of a possible paraffinoma, as patients may not be forthcoming with relevant clinical history regarding a prior injection to the affected site, particularly if the injection occurred many years ago. As such, the patient may not consider this history relevant or may not even remember the event occurred, as was observed in our case. Furthermore, embarrassment, social taboo, and stigma may be associated with the behavior of undergoing injections in nonclinical settings without medical supervision.¹⁵

Patients may be motivated to undergo dangerous procedures to potentially alter their appearance due to perceived enhanced sexual ability, influence by loved ones, cultural rituals, and societal pressure.^15,16 Furthermore, patients may not be aware of the material being injected or the volume. Given that these injections often are used with the goal of cosmetic enhancement, biopsies in cosmetically sensitive areas must be given careful consideration, and a thorough clinical history must support the decision to pursue a biopsy to obtain a definitive diagnosis.

The definitive diagnosis of a paraffinoma is determined by histopathology. However, the use of imaging modalities such as magnetic resonance imaging and computed tomography have been employed to delineate the extent of involvement. Imaging studies allow for surgical planning and may assist in narrowing a differential diagnosis.¹⁷ Currently, wide and complete surgical resection is the only definitive treatment of paraffinomas, including penile paraffinomas, as there is no evidence of spontaneous regression.³ A report of a reconstructive surgery involving penile resurfacing without T-style anastomosis has been found effective at preventing necrosis of the ventral penile skin. Not all paraffinomas behave similarly, and there is no reliable method to determine which paraffinoma may possess a more aggressive clinical course compared to those which have a more indolent course.¹⁸ As such, early detection is critical in the management of paraffinomas, especially in anatomic locations where tissue preservation is of utmost importance. In the case of a large penile paraffinoma with the ability to destroy vital urologic and reproductive function, physicians must consider prevention of recurrent episodes through suppression of inflammation and fibrosis with doxycycline and nicotinamide.¹⁹ Other medical treatments reported with varying success include corticosteroids, imiquimod, and isotretinoin.^19-24 Employing adjunctive medical treatment may decrease the size of the mass, reducing the surgical defect size and preserving tissue vitality. Ultimately, the most crucial aspect in treatment is prevention, as injection of foreign materials elicits a foreign body response and can lead to notable morbidity.

To the Editor:

A 62-year-old man presented with an atrophied painful left arm of 17 years’ duration that began when he was hit by a car as a pedestrian. He sustained severe multisystem injuries from the accident, including left brachial plexus and C6 nerve root avulsion injury. When he regained consciousness after 6 weeks in the intensive care unit, he immediately noted diffuse pain throughout the body, especially in the left arm. Since the accident, the patient continued to have diminished sensation to touch and temperature in the left arm. He also had burning, throbbing, and electrical pain in the left arm with light touch as well as spontaneously. He was thoroughly evaluated by a neurologist and was diagnosed with complex regional pain syndrome (CRPS) type II. For the treatment of pain, dorsal column stimulation and hemilaminectomy with exploration of the avulsed nerve root were attempted, both of which had minimal effect. He was maintained on hydromorphone, methadone, and oxazepam. He reported that for many years he was unable move out of bed due to the unbearable pain. With pain medications, he was able to regain most of his independence in his daily life, though the pain and other clinical aspects of CRPS still completely limited his use of the left arm.

Physical examination revealed glossy, cold, hairless skin with hypohidrosis of the left arm, forearm, and hand (Figures 1 and 2A). The left arm was conspicuously atrophied, with the forearm and hand erythematous. The fingers were taut, contracted, and edematous (Figure 2B), and the skin was unable to be pinched. The fingernails on the left hand had dystrophic changes including yellow color and brittleness with longitudinal ridges (Figure 3). The patient could activate the left bicep and tricep muscles against gravity but had minimal function of the deltoid muscle. He also had minimal movement of the left index finger and was unable to move any other digits of the left hand. The patient was continued on pain management treatments and physical therapy for his condition.

Complex regional pain syndrome is a neuropathic disorder of the extremities characterized by pain and a variety of autonomic and motor disturbances such as local edema, limited active range of motion, and vasomotor and trophic skin changes. There are 2 types of CRPS: type II is marked by explicit nerve injury and type I is not. The pathophysiology of CRPS is unknown.^1-3

There is no definite set of diagnostic criteria for CRPS. The lack of any gold-standard diagnostic test for CRPS has made arriving at one valid, widely accepted set of diagnostic criteria impossible.¹ There are 4 widely used sets of diagnostic criteria. One is the International Association for the Study of Pain diagnostic criteria defined in 1994.⁴ However, the criteria rely entirely on subjective symptoms and have been under great scrutiny due to their questionable validity.² Veldman et al⁵ presented other widely used CRPS diagnostic criteria in their prospective study of 829 reflex sympathetic dystrophy patients, which paid particular attention to the early clinical manifestations of CRPS. In 1999, Bruehl et al² proposed their own modified diagnostic criteria, which required physician-assessed signs in 2 of 4 categories to avoid the practice of exclusively relying on subjective symptoms. In addition, during a consensus meeting in Budapest, Hungary, a modified version of the Bruehl criteria was proposed.⁶ All 4 criteria rely solely on detailed history and physical examination, and the choice of diagnostic criteria remains subjective.

The pathophysiology of CRPS also remains unclear. There are several proposed mechanisms such as sympathetic nervous system dysfunction, abnormal inflammatory response, and central nervous system involvement.¹ Psychologic factors, sequelae of nerve injury, and genetic predisposition also have been implicated in the pathophysiology of CRPS.¹ It is likely that several mechanisms variably contribute to each presentation of CRPS.

Many dermatologic findings, in addition to neuromuscular symptoms, accompany CRPS and serve as important clues to making the clinical diagnosis. Complex regional pain syndrome has been thought to have 3 distinct sequential stages of CRPS.^1,3,7 Stage 1—the acute stage—is marked by hyperalgesia, allodynia, sudomotor disturbances, and prominent edema. Stage 2—the dystrophic stage—is characterized by more marked pain and sensory dysfunction, vasomotor dysfunction, development of motor dysfunction, soft tissue edema, skin and articular soft tissue thickening, and development of dystrophic nail changes. Stage 3—the atrophic stage—is marked by decreased pain and sensory disturbances, markedly increased motor dysfunction, waxy atrophic skin changes, progression of dystrophic nail changes, and skeletal cystic and subchondral erosions with diffuse osteoporosis.^1,3,7

The staging model, however, has been called into question.³ In a cluster analysis, Bruehl et al³ arrived at 3 relatively consistent CRPS patient subgroups that did not have notably different pain duration, suggesting the existence of 3 CRPS subtypes, not stages. Their study found that one of the subgroups best represented the clinical presentation of CRPS type II. This subgroup had the greatest pain and sensory abnormalities and the least vasomotor dysfunction of all 3 subgroups. Nonetheless, this study has not settled the discussion, as it only included 113 patients.³ Thus, with future studies, our understanding of CRPS in stages may change, which likely will impact how the clinical diagnosis is made.

There is a lack of high-quality evidence for most treatment interventions for CRPS⁸; however, the current practice is to use an interdisciplinary approach.^1,9,10 The main therapeutic arm of this approach is rehabilitation; physical and occupational therapy can help improve range of motion, contracture, and atrophy. The other 2 arms of the approach are psychologic therapy to improve quality of life and pain management with pharmacologic therapy and/or invasive interventions. The choice of therapy remains empirical; trial and error should be expected in developing an adequate treatment plan for each individual patient.

Many aspects of CRPS remain unclear, and even our current understanding of the disease will inevitably change over time. The syndrome can cause life-changing morbidities in patients, and late diagnosis and treatment are associated with poor prognosis. Because there are many dermatologic findings associated with the disorder, it is crucial for dermatologists to clinically recognize the disorder and to refer patients to appropriate channels so that treatment can be started as soon as possible.

To the Editor:

A 62-year-old man presented with an atrophied painful left arm of 17 years’ duration that began when he was hit by a car as a pedestrian. He sustained severe multisystem injuries from the accident, including left brachial plexus and C6 nerve root avulsion injury. When he regained consciousness after 6 weeks in the intensive care unit, he immediately noted diffuse pain throughout the body, especially in the left arm. Since the accident, the patient continued to have diminished sensation to touch and temperature in the left arm. He also had burning, throbbing, and electrical pain in the left arm with light touch as well as spontaneously. He was thoroughly evaluated by a neurologist and was diagnosed with complex regional pain syndrome (CRPS) type II. For the treatment of pain, dorsal column stimulation and hemilaminectomy with exploration of the avulsed nerve root were attempted, both of which had minimal effect. He was maintained on hydromorphone, methadone, and oxazepam. He reported that for many years he was unable move out of bed due to the unbearable pain. With pain medications, he was able to regain most of his independence in his daily life, though the pain and other clinical aspects of CRPS still completely limited his use of the left arm.

Physical examination revealed glossy, cold, hairless skin with hypohidrosis of the left arm, forearm, and hand (Figures 1 and 2A). The left arm was conspicuously atrophied, with the forearm and hand erythematous. The fingers were taut, contracted, and edematous (Figure 2B), and the skin was unable to be pinched. The fingernails on the left hand had dystrophic changes including yellow color and brittleness with longitudinal ridges (Figure 3). The patient could activate the left bicep and tricep muscles against gravity but had minimal function of the deltoid muscle. He also had minimal movement of the left index finger and was unable to move any other digits of the left hand. The patient was continued on pain management treatments and physical therapy for his condition.

Complex regional pain syndrome is a neuropathic disorder of the extremities characterized by pain and a variety of autonomic and motor disturbances such as local edema, limited active range of motion, and vasomotor and trophic skin changes. There are 2 types of CRPS: type II is marked by explicit nerve injury and type I is not. The pathophysiology of CRPS is unknown.^1-3

There is no definite set of diagnostic criteria for CRPS. The lack of any gold-standard diagnostic test for CRPS has made arriving at one valid, widely accepted set of diagnostic criteria impossible.¹ There are 4 widely used sets of diagnostic criteria. One is the International Association for the Study of Pain diagnostic criteria defined in 1994.⁴ However, the criteria rely entirely on subjective symptoms and have been under great scrutiny due to their questionable validity.² Veldman et al⁵ presented other widely used CRPS diagnostic criteria in their prospective study of 829 reflex sympathetic dystrophy patients, which paid particular attention to the early clinical manifestations of CRPS. In 1999, Bruehl et al² proposed their own modified diagnostic criteria, which required physician-assessed signs in 2 of 4 categories to avoid the practice of exclusively relying on subjective symptoms. In addition, during a consensus meeting in Budapest, Hungary, a modified version of the Bruehl criteria was proposed.⁶ All 4 criteria rely solely on detailed history and physical examination, and the choice of diagnostic criteria remains subjective.

The pathophysiology of CRPS also remains unclear. There are several proposed mechanisms such as sympathetic nervous system dysfunction, abnormal inflammatory response, and central nervous system involvement.¹ Psychologic factors, sequelae of nerve injury, and genetic predisposition also have been implicated in the pathophysiology of CRPS.¹ It is likely that several mechanisms variably contribute to each presentation of CRPS.

Many dermatologic findings, in addition to neuromuscular symptoms, accompany CRPS and serve as important clues to making the clinical diagnosis. Complex regional pain syndrome has been thought to have 3 distinct sequential stages of CRPS.^1,3,7 Stage 1—the acute stage—is marked by hyperalgesia, allodynia, sudomotor disturbances, and prominent edema. Stage 2—the dystrophic stage—is characterized by more marked pain and sensory dysfunction, vasomotor dysfunction, development of motor dysfunction, soft tissue edema, skin and articular soft tissue thickening, and development of dystrophic nail changes. Stage 3—the atrophic stage—is marked by decreased pain and sensory disturbances, markedly increased motor dysfunction, waxy atrophic skin changes, progression of dystrophic nail changes, and skeletal cystic and subchondral erosions with diffuse osteoporosis.^1,3,7

The staging model, however, has been called into question.³ In a cluster analysis, Bruehl et al³ arrived at 3 relatively consistent CRPS patient subgroups that did not have notably different pain duration, suggesting the existence of 3 CRPS subtypes, not stages. Their study found that one of the subgroups best represented the clinical presentation of CRPS type II. This subgroup had the greatest pain and sensory abnormalities and the least vasomotor dysfunction of all 3 subgroups. Nonetheless, this study has not settled the discussion, as it only included 113 patients.³ Thus, with future studies, our understanding of CRPS in stages may change, which likely will impact how the clinical diagnosis is made.

There is a lack of high-quality evidence for most treatment interventions for CRPS⁸; however, the current practice is to use an interdisciplinary approach.^1,9,10 The main therapeutic arm of this approach is rehabilitation; physical and occupational therapy can help improve range of motion, contracture, and atrophy. The other 2 arms of the approach are psychologic therapy to improve quality of life and pain management with pharmacologic therapy and/or invasive interventions. The choice of therapy remains empirical; trial and error should be expected in developing an adequate treatment plan for each individual patient.

Many aspects of CRPS remain unclear, and even our current understanding of the disease will inevitably change over time. The syndrome can cause life-changing morbidities in patients, and late diagnosis and treatment are associated with poor prognosis. Because there are many dermatologic findings associated with the disorder, it is crucial for dermatologists to clinically recognize the disorder and to refer patients to appropriate channels so that treatment can be started as soon as possible.

To the Editor:

A 62-year-old man presented with an atrophied painful left arm of 17 years’ duration that began when he was hit by a car as a pedestrian. He sustained severe multisystem injuries from the accident, including left brachial plexus and C6 nerve root avulsion injury. When he regained consciousness after 6 weeks in the intensive care unit, he immediately noted diffuse pain throughout the body, especially in the left arm. Since the accident, the patient continued to have diminished sensation to touch and temperature in the left arm. He also had burning, throbbing, and electrical pain in the left arm with light touch as well as spontaneously. He was thoroughly evaluated by a neurologist and was diagnosed with complex regional pain syndrome (CRPS) type II. For the treatment of pain, dorsal column stimulation and hemilaminectomy with exploration of the avulsed nerve root were attempted, both of which had minimal effect. He was maintained on hydromorphone, methadone, and oxazepam. He reported that for many years he was unable move out of bed due to the unbearable pain. With pain medications, he was able to regain most of his independence in his daily life, though the pain and other clinical aspects of CRPS still completely limited his use of the left arm.

Physical examination revealed glossy, cold, hairless skin with hypohidrosis of the left arm, forearm, and hand (Figures 1 and 2A). The left arm was conspicuously atrophied, with the forearm and hand erythematous. The fingers were taut, contracted, and edematous (Figure 2B), and the skin was unable to be pinched. The fingernails on the left hand had dystrophic changes including yellow color and brittleness with longitudinal ridges (Figure 3). The patient could activate the left bicep and tricep muscles against gravity but had minimal function of the deltoid muscle. He also had minimal movement of the left index finger and was unable to move any other digits of the left hand. The patient was continued on pain management treatments and physical therapy for his condition.

Complex regional pain syndrome is a neuropathic disorder of the extremities characterized by pain and a variety of autonomic and motor disturbances such as local edema, limited active range of motion, and vasomotor and trophic skin changes. There are 2 types of CRPS: type II is marked by explicit nerve injury and type I is not. The pathophysiology of CRPS is unknown.^1-3

There is no definite set of diagnostic criteria for CRPS. The lack of any gold-standard diagnostic test for CRPS has made arriving at one valid, widely accepted set of diagnostic criteria impossible.¹ There are 4 widely used sets of diagnostic criteria. One is the International Association for the Study of Pain diagnostic criteria defined in 1994.⁴ However, the criteria rely entirely on subjective symptoms and have been under great scrutiny due to their questionable validity.² Veldman et al⁵ presented other widely used CRPS diagnostic criteria in their prospective study of 829 reflex sympathetic dystrophy patients, which paid particular attention to the early clinical manifestations of CRPS. In 1999, Bruehl et al² proposed their own modified diagnostic criteria, which required physician-assessed signs in 2 of 4 categories to avoid the practice of exclusively relying on subjective symptoms. In addition, during a consensus meeting in Budapest, Hungary, a modified version of the Bruehl criteria was proposed.⁶ All 4 criteria rely solely on detailed history and physical examination, and the choice of diagnostic criteria remains subjective.

The pathophysiology of CRPS also remains unclear. There are several proposed mechanisms such as sympathetic nervous system dysfunction, abnormal inflammatory response, and central nervous system involvement.¹ Psychologic factors, sequelae of nerve injury, and genetic predisposition also have been implicated in the pathophysiology of CRPS.¹ It is likely that several mechanisms variably contribute to each presentation of CRPS.

Many dermatologic findings, in addition to neuromuscular symptoms, accompany CRPS and serve as important clues to making the clinical diagnosis. Complex regional pain syndrome has been thought to have 3 distinct sequential stages of CRPS.^1,3,7 Stage 1—the acute stage—is marked by hyperalgesia, allodynia, sudomotor disturbances, and prominent edema. Stage 2—the dystrophic stage—is characterized by more marked pain and sensory dysfunction, vasomotor dysfunction, development of motor dysfunction, soft tissue edema, skin and articular soft tissue thickening, and development of dystrophic nail changes. Stage 3—the atrophic stage—is marked by decreased pain and sensory disturbances, markedly increased motor dysfunction, waxy atrophic skin changes, progression of dystrophic nail changes, and skeletal cystic and subchondral erosions with diffuse osteoporosis.^1,3,7

The staging model, however, has been called into question.³ In a cluster analysis, Bruehl et al³ arrived at 3 relatively consistent CRPS patient subgroups that did not have notably different pain duration, suggesting the existence of 3 CRPS subtypes, not stages. Their study found that one of the subgroups best represented the clinical presentation of CRPS type II. This subgroup had the greatest pain and sensory abnormalities and the least vasomotor dysfunction of all 3 subgroups. Nonetheless, this study has not settled the discussion, as it only included 113 patients.³ Thus, with future studies, our understanding of CRPS in stages may change, which likely will impact how the clinical diagnosis is made.

There is a lack of high-quality evidence for most treatment interventions for CRPS⁸; however, the current practice is to use an interdisciplinary approach.^1,9,10 The main therapeutic arm of this approach is rehabilitation; physical and occupational therapy can help improve range of motion, contracture, and atrophy. The other 2 arms of the approach are psychologic therapy to improve quality of life and pain management with pharmacologic therapy and/or invasive interventions. The choice of therapy remains empirical; trial and error should be expected in developing an adequate treatment plan for each individual patient.

Many aspects of CRPS remain unclear, and even our current understanding of the disease will inevitably change over time. The syndrome can cause life-changing morbidities in patients, and late diagnosis and treatment are associated with poor prognosis. Because there are many dermatologic findings associated with the disorder, it is crucial for dermatologists to clinically recognize the disorder and to refer patients to appropriate channels so that treatment can be started as soon as possible.

To the Editor:

Syringomas are common benign tumors of the eccrine sweat glands that usually manifest clinically as multiple flesh-colored papules. They are most commonly seen on the face, neck, and chest of adolescent girls. Syringomas may appear at any site of the body but are rare in the vulva. We present a case of a 51-year-old woman who was referred to the Division of Gynecologic Oncology at the University of Alabama at Birmingham for further management of a tumor carrying a differential diagnosis of vulvar syringoma vs microcystic adnexal carcinoma (MAC).

A 51-year-old woman presented to dermatology (G.G.) and was referred to the Division of Gynecologic Oncology at the University of Alabama at Birmingham for further management of possible vulvar syringoma vs MAC. The patient previously had been evaluated at an outside community practice due to dyspareunia, vulvar discomfort, and vulvar irregularities of 1 month’s duration. At that time, a small biopsy was performed, and the histologic differential diagnosis included syringoma vs an adnexal carcinoma. Consequently, she was referred to gynecologic oncology for further management.

Pelvic examination revealed multilobular nodular areas overlying the clitoral hood that extended down to the labia majora. The nodular processes did not involve the clitoris, labia minora, or perineum. A mobile isolated lymph node measuring 2.0×1.0 cm in the right inguinal area also was noted. The patient’s clinical history was notable for right breast carcinoma treated with a right mastectomy with axillary lymph node dissection that showed metastatic disease. She also underwent adjuvant chemotherapy with paclitaxel and doxorubicin for breast carcinoma.

After discussing the diagnostic differential and treatment options, the patient elected to undergo a bilateral partial radical vulvectomy with reconstruction and resection of the right inguinal lymph node. Gross examination of the vulvectomy specimen showed multiple flesh-colored papules (Figure 1). Histologic examination revealed a neoplasm with sweat gland differentiation that was broad and poorly circumscribed but confined to the dermis (Figures 2A and 2B). The neoplasm was composed of epithelial cells that formed ductlike structures, lined by 2 layers of cuboidal epithelium within a fibrous stroma (Figure 2C). A toluidine blue special stain was performed and demonstrated an increased amount of mast cells in the tissue (Figure 3). Immunohistochemical stains for gross cystic disease fluid protein, estrogen receptor (ER), and progesterone receptor (PR) were negative in the tumor cells. The lack of cytologic atypia, perineural invasion, and deep infiltration into the subcutis favored a syringoma. One month later, the case was presented at the Tumor Board Conference at the University of Alabama at Birmingham where a final diagnosis of vulvar syringoma was agreed upon and discussed with the patient. At that time, no recurrence was evident and follow-up was recommended.

Syringomas may occur at any site on the body but are prone to occur on the periorbital area, especially the eyelids.¹ Some of the atypical locations for a syringoma include the anterior neck, chest, abdomen, genitals, axillae, groin, and buttocks.² Vulvar syringomas were first reported by Carneiro³ in 1971 as usually affecting adolescent girls and middle-aged women. There have been approximately 40 reported cases affecting women aged 8 to 78 years.^4,5 Vulvar syringomas classically appear as firm or soft, flesh-colored to transparent, papular lesions. The 2 other clinical variants are miliumlike, whitish, cystic papules as well as lichenoid papules.⁶ Pérez-Bustillo et al⁵ reported a case of the lichenoid papule variant on the labia majora of a 78-year-old woman who presented with intermittent vulvar pruritus of 4 years’ duration. Due to this patient’s 9-year history of urinary incontinence, the lesions had been misdiagnosed as irritant dermatitis and associated lichen simplex chronicus (LSC). This case is a reminder to consider vulvar syringoma in patients with LSC who respond poorly to oral antihistamines and topical steroids.⁵ Rarely, multiple clinical variants may coexist. In a case reported by Dereli et al,⁷ a 19-year-old woman presented with concurrent classical and miliumlike forms of vulvar syringoma.

Vulvar syringomas usually present as multiple lesions involving both sides of the labia majora; however, Blasdale and McLelland⁸ reported a single isolated syringoma of the vulva on the anterior right labia minora that measured 1.0×0.5 cm, leading the lesion to be described as a giant syringoma.

Vulvar syringomas usually are asymptomatic and noticed during routine gynecologic examination. Therefore, it is believed that they likely are underdiagnosed.⁵ When symptomatic, they commonly present with constant⁹ or intermittent⁵ pruritus, which may intensify during menstruation, pregnancy, and summertime.^6,10-12 Gerdsen et al¹⁰ documented a 27-year-old woman who presented with a 2-year history of pruritic vulvar skin lesions that became exacerbated during menstruation, which raised the possibility of cyclical hormonal changes being responsible for periodic exacerbation of vulvar pruritus during menstruation. In addition, patients may experience an increase in size and number of the lesions during pregnancy. Bal et al¹¹ reported a 24-year-old primigravida with vulvar papular lesions that intensified during pregnancy. She had experienced intermittent vulvar pruritus for 12 years but had no change in symptoms during menstruation.¹¹ Few studies have attempted to evaluate the presence of ER and PR in the syringomas. A study of 9 nonvulvar syringomas by Wallace and Smoller¹³ showed ER positivity in 1 case and PR positivity in 8 cases, lending support to the hormonal theory; however, in another case series of 15 vulvar syringomas, Huang et al⁶ failed to show ER and PR expression by immunohistochemical staining. A case report published 3 years earlier documented the first case of PR positivity on a vulvar syringoma.¹⁴ Our patient also was negative for ER and PR, which suggested that hormonal status is important in some but not all syringomas.

Patients with vulgar syringomas also might have coexisting extragenital syringomas in the neck,⁴ eyelids,^6,7,10 and periorbital area,⁶ and thorough examination of the body is essential. If an extragenital syringoma is diagnosed, a vulvar syringoma should be considered, especially when the patient presents with unexplained genital symptoms. Although no proven hereditary transmission pattern has been established, family history of syringomas has been established in several cases.¹⁵ In a case series reported by Huang et al,⁶ 4 of 18 patients reported a family history of periorbital syringomas. In our case, the patient did not report a family history of syringomas.

The differential diagnosis of vulvar lesions with pruritus is broad and includes Fox-Fordyce disease, lichen planus, LSC, epidermal cysts, senile angiomas, dystrophic calcinosis, xanthomas, steatocytomas, soft fibromas, condyloma acuminatum, and candidiasis. Vulvar syringomas might have a nonspecific appearance, and histologic examination is essential to confirm the diagnosis and rule out any malignant process such as MAC, vulvar intraepithelial neoplasia, extramammary Paget disease, or other glandular neoplasms of the vulva.

Microcystic adnexal carcinoma was first reported in 1982 by Goldstein et al¹⁶ as a locally aggressive neoplasm that can be confused with benign adnexal neoplasms, particularly desmoplastic trichoepithelioma, trichoadenoma, and syringoma. Microcystic adnexal carcinomas present as slow-growing, flesh-colored papules that may resemble syringomas and appear in similar body sites. Histologic examination is essential to differentiate between these two entities. Syringomas are tumors confined to the dermis and are composed of multiple small ducts lined by 2 layers of cuboidal epithelium within a dense fibrous stroma. Unlike syringomas, MACs usually infiltrate diffusely into the dermis and subcutis and may extend into the underlying muscle. Although bland cytologic features predominate, perineural invasion frequently is present in MACs. A potential pitfall of misdiagnosis can be caused by a superficial biopsy that may reveal benign histologic appearance, particularly in the upper level of the tumor where it may be confused with a syringoma or a benign follicular neoplasm.¹⁷

The initial biopsy performed on our patient was possibly not deep enough to render an unequivocal diagnosis and therefore bilateral partial radical vulvectomy was considered. After surgery, histologic examination of the resection specimen revealed a poorly circumscribed tumor confined to the dermis. The tumor was broad and the lack of deep infiltration into the subcutis and perineural invasion favored a syringoma (Figures 2A and 2B). These findings were consistent with case reports that documented syringomas as being more wide than deep on microscopic examination, whereas the opposite pertained to MAC.¹⁸ Cases of plaque-type syringomas that initially were misdiagnosed as MACs also have been reported.¹⁹ Because misdiagnosis may affect the treatment plan and potentially result in unnecessary surgery, caution should be taken when differentiating between these two entities. When a definitive diagnosis cannot be rendered on a superficial biopsy, a recommendation should be made for a deeper biopsy sampling the subcutis.

For the majority of the patients with vulvar syringomas, treatment is seldom required due to their asymptomatic nature; however, patients who present with symptoms usually report pruritus of variable intensities and patterns. A standardized treatment does not exist for vulvar syringomas, and oral or topical treatment might be used as an initial approach. Commonly prescribed medications with variable results include topical corticosteroids, oral antihistamines, and topical retinoids. In a case reported by Iwao et al,²⁰ vulvar syringomas were successfully treated with tranilast, which has anti-inflammatory and immunomodulatory effects. This medication could have a possible dual action—inhibiting the release of chemical mediators from the mast cells and inhibiting the release of IL-1β from the eccrine duct, which could suppress the proliferation of stromal connective tissue. Our case was stained with toluidine blue and showed an increased number of mast cells in the tissue (Figure 3). Patients who are unresponsive to tranilast or have extensive disease resulting in cosmetic disfigurement might benefit from more invasive treatment methods including a variety of lasers, cryotherapy, electrosurgery, and excision. Excisions should include the entire tumor to avoid recurrence. In a case reported by Garman and Metry,²¹ the lesions were surgically excised using small 2- to 3-mm punches; however, several weeks later the lesions recurred. Our patient presented with a 1-month evolution of dyspareunia, vulvar discomfort, and vulvar irregularities that were probably not treated with oral or topical medications before being referred for surgery.

We report a case of a vulvar syringoma that presented diagnostic challenges in the initial biopsy, which prevented the exclusion of an MAC. After partial radical vulvectomy, histologic examination was more definitive, showing lack of deep infiltration into the subcutis or perineural invasion that are commonly seen in MAC. This case is an example of a notable pitfall in the diagnosis of vulvar syringoma on a limited biopsy leading to overtreatment. Raising awareness of this entity is the only modality to prevent misdiagnosis. We encourage reporting of further cases of syringomas, particularly those with atypical locations or patterns that may cause diagnostic problems.

To the Editor:

Syringomas are common benign tumors of the eccrine sweat glands that usually manifest clinically as multiple flesh-colored papules. They are most commonly seen on the face, neck, and chest of adolescent girls. Syringomas may appear at any site of the body but are rare in the vulva. We present a case of a 51-year-old woman who was referred to the Division of Gynecologic Oncology at the University of Alabama at Birmingham for further management of a tumor carrying a differential diagnosis of vulvar syringoma vs microcystic adnexal carcinoma (MAC).

A 51-year-old woman presented to dermatology (G.G.) and was referred to the Division of Gynecologic Oncology at the University of Alabama at Birmingham for further management of possible vulvar syringoma vs MAC. The patient previously had been evaluated at an outside community practice due to dyspareunia, vulvar discomfort, and vulvar irregularities of 1 month’s duration. At that time, a small biopsy was performed, and the histologic differential diagnosis included syringoma vs an adnexal carcinoma. Consequently, she was referred to gynecologic oncology for further management.

Pelvic examination revealed multilobular nodular areas overlying the clitoral hood that extended down to the labia majora. The nodular processes did not involve the clitoris, labia minora, or perineum. A mobile isolated lymph node measuring 2.0×1.0 cm in the right inguinal area also was noted. The patient’s clinical history was notable for right breast carcinoma treated with a right mastectomy with axillary lymph node dissection that showed metastatic disease. She also underwent adjuvant chemotherapy with paclitaxel and doxorubicin for breast carcinoma.

After discussing the diagnostic differential and treatment options, the patient elected to undergo a bilateral partial radical vulvectomy with reconstruction and resection of the right inguinal lymph node. Gross examination of the vulvectomy specimen showed multiple flesh-colored papules (Figure 1). Histologic examination revealed a neoplasm with sweat gland differentiation that was broad and poorly circumscribed but confined to the dermis (Figures 2A and 2B). The neoplasm was composed of epithelial cells that formed ductlike structures, lined by 2 layers of cuboidal epithelium within a fibrous stroma (Figure 2C). A toluidine blue special stain was performed and demonstrated an increased amount of mast cells in the tissue (Figure 3). Immunohistochemical stains for gross cystic disease fluid protein, estrogen receptor (ER), and progesterone receptor (PR) were negative in the tumor cells. The lack of cytologic atypia, perineural invasion, and deep infiltration into the subcutis favored a syringoma. One month later, the case was presented at the Tumor Board Conference at the University of Alabama at Birmingham where a final diagnosis of vulvar syringoma was agreed upon and discussed with the patient. At that time, no recurrence was evident and follow-up was recommended.

Syringomas may occur at any site on the body but are prone to occur on the periorbital area, especially the eyelids.¹ Some of the atypical locations for a syringoma include the anterior neck, chest, abdomen, genitals, axillae, groin, and buttocks.² Vulvar syringomas were first reported by Carneiro³ in 1971 as usually affecting adolescent girls and middle-aged women. There have been approximately 40 reported cases affecting women aged 8 to 78 years.^4,5 Vulvar syringomas classically appear as firm or soft, flesh-colored to transparent, papular lesions. The 2 other clinical variants are miliumlike, whitish, cystic papules as well as lichenoid papules.⁶ Pérez-Bustillo et al⁵ reported a case of the lichenoid papule variant on the labia majora of a 78-year-old woman who presented with intermittent vulvar pruritus of 4 years’ duration. Due to this patient’s 9-year history of urinary incontinence, the lesions had been misdiagnosed as irritant dermatitis and associated lichen simplex chronicus (LSC). This case is a reminder to consider vulvar syringoma in patients with LSC who respond poorly to oral antihistamines and topical steroids.⁵ Rarely, multiple clinical variants may coexist. In a case reported by Dereli et al,⁷ a 19-year-old woman presented with concurrent classical and miliumlike forms of vulvar syringoma.

Vulvar syringomas usually present as multiple lesions involving both sides of the labia majora; however, Blasdale and McLelland⁸ reported a single isolated syringoma of the vulva on the anterior right labia minora that measured 1.0×0.5 cm, leading the lesion to be described as a giant syringoma.

Vulvar syringomas usually are asymptomatic and noticed during routine gynecologic examination. Therefore, it is believed that they likely are underdiagnosed.⁵ When symptomatic, they commonly present with constant⁹ or intermittent⁵ pruritus, which may intensify during menstruation, pregnancy, and summertime.^6,10-12 Gerdsen et al¹⁰ documented a 27-year-old woman who presented with a 2-year history of pruritic vulvar skin lesions that became exacerbated during menstruation, which raised the possibility of cyclical hormonal changes being responsible for periodic exacerbation of vulvar pruritus during menstruation. In addition, patients may experience an increase in size and number of the lesions during pregnancy. Bal et al¹¹ reported a 24-year-old primigravida with vulvar papular lesions that intensified during pregnancy. She had experienced intermittent vulvar pruritus for 12 years but had no change in symptoms during menstruation.¹¹ Few studies have attempted to evaluate the presence of ER and PR in the syringomas. A study of 9 nonvulvar syringomas by Wallace and Smoller¹³ showed ER positivity in 1 case and PR positivity in 8 cases, lending support to the hormonal theory; however, in another case series of 15 vulvar syringomas, Huang et al⁶ failed to show ER and PR expression by immunohistochemical staining. A case report published 3 years earlier documented the first case of PR positivity on a vulvar syringoma.¹⁴ Our patient also was negative for ER and PR, which suggested that hormonal status is important in some but not all syringomas.

Patients with vulgar syringomas also might have coexisting extragenital syringomas in the neck,⁴ eyelids,^6,7,10 and periorbital area,⁶ and thorough examination of the body is essential. If an extragenital syringoma is diagnosed, a vulvar syringoma should be considered, especially when the patient presents with unexplained genital symptoms. Although no proven hereditary transmission pattern has been established, family history of syringomas has been established in several cases.¹⁵ In a case series reported by Huang et al,⁶ 4 of 18 patients reported a family history of periorbital syringomas. In our case, the patient did not report a family history of syringomas.

The differential diagnosis of vulvar lesions with pruritus is broad and includes Fox-Fordyce disease, lichen planus, LSC, epidermal cysts, senile angiomas, dystrophic calcinosis, xanthomas, steatocytomas, soft fibromas, condyloma acuminatum, and candidiasis. Vulvar syringomas might have a nonspecific appearance, and histologic examination is essential to confirm the diagnosis and rule out any malignant process such as MAC, vulvar intraepithelial neoplasia, extramammary Paget disease, or other glandular neoplasms of the vulva.

Microcystic adnexal carcinoma was first reported in 1982 by Goldstein et al¹⁶ as a locally aggressive neoplasm that can be confused with benign adnexal neoplasms, particularly desmoplastic trichoepithelioma, trichoadenoma, and syringoma. Microcystic adnexal carcinomas present as slow-growing, flesh-colored papules that may resemble syringomas and appear in similar body sites. Histologic examination is essential to differentiate between these two entities. Syringomas are tumors confined to the dermis and are composed of multiple small ducts lined by 2 layers of cuboidal epithelium within a dense fibrous stroma. Unlike syringomas, MACs usually infiltrate diffusely into the dermis and subcutis and may extend into the underlying muscle. Although bland cytologic features predominate, perineural invasion frequently is present in MACs. A potential pitfall of misdiagnosis can be caused by a superficial biopsy that may reveal benign histologic appearance, particularly in the upper level of the tumor where it may be confused with a syringoma or a benign follicular neoplasm.¹⁷

The initial biopsy performed on our patient was possibly not deep enough to render an unequivocal diagnosis and therefore bilateral partial radical vulvectomy was considered. After surgery, histologic examination of the resection specimen revealed a poorly circumscribed tumor confined to the dermis. The tumor was broad and the lack of deep infiltration into the subcutis and perineural invasion favored a syringoma (Figures 2A and 2B). These findings were consistent with case reports that documented syringomas as being more wide than deep on microscopic examination, whereas the opposite pertained to MAC.¹⁸ Cases of plaque-type syringomas that initially were misdiagnosed as MACs also have been reported.¹⁹ Because misdiagnosis may affect the treatment plan and potentially result in unnecessary surgery, caution should be taken when differentiating between these two entities. When a definitive diagnosis cannot be rendered on a superficial biopsy, a recommendation should be made for a deeper biopsy sampling the subcutis.

For the majority of the patients with vulvar syringomas, treatment is seldom required due to their asymptomatic nature; however, patients who present with symptoms usually report pruritus of variable intensities and patterns. A standardized treatment does not exist for vulvar syringomas, and oral or topical treatment might be used as an initial approach. Commonly prescribed medications with variable results include topical corticosteroids, oral antihistamines, and topical retinoids. In a case reported by Iwao et al,²⁰ vulvar syringomas were successfully treated with tranilast, which has anti-inflammatory and immunomodulatory effects. This medication could have a possible dual action—inhibiting the release of chemical mediators from the mast cells and inhibiting the release of IL-1β from the eccrine duct, which could suppress the proliferation of stromal connective tissue. Our case was stained with toluidine blue and showed an increased number of mast cells in the tissue (Figure 3). Patients who are unresponsive to tranilast or have extensive disease resulting in cosmetic disfigurement might benefit from more invasive treatment methods including a variety of lasers, cryotherapy, electrosurgery, and excision. Excisions should include the entire tumor to avoid recurrence. In a case reported by Garman and Metry,²¹ the lesions were surgically excised using small 2- to 3-mm punches; however, several weeks later the lesions recurred. Our patient presented with a 1-month evolution of dyspareunia, vulvar discomfort, and vulvar irregularities that were probably not treated with oral or topical medications before being referred for surgery.

We report a case of a vulvar syringoma that presented diagnostic challenges in the initial biopsy, which prevented the exclusion of an MAC. After partial radical vulvectomy, histologic examination was more definitive, showing lack of deep infiltration into the subcutis or perineural invasion that are commonly seen in MAC. This case is an example of a notable pitfall in the diagnosis of vulvar syringoma on a limited biopsy leading to overtreatment. Raising awareness of this entity is the only modality to prevent misdiagnosis. We encourage reporting of further cases of syringomas, particularly those with atypical locations or patterns that may cause diagnostic problems.

To the Editor:

Syringomas are common benign tumors of the eccrine sweat glands that usually manifest clinically as multiple flesh-colored papules. They are most commonly seen on the face, neck, and chest of adolescent girls. Syringomas may appear at any site of the body but are rare in the vulva. We present a case of a 51-year-old woman who was referred to the Division of Gynecologic Oncology at the University of Alabama at Birmingham for further management of a tumor carrying a differential diagnosis of vulvar syringoma vs microcystic adnexal carcinoma (MAC).

A 51-year-old woman presented to dermatology (G.G.) and was referred to the Division of Gynecologic Oncology at the University of Alabama at Birmingham for further management of possible vulvar syringoma vs MAC. The patient previously had been evaluated at an outside community practice due to dyspareunia, vulvar discomfort, and vulvar irregularities of 1 month’s duration. At that time, a small biopsy was performed, and the histologic differential diagnosis included syringoma vs an adnexal carcinoma. Consequently, she was referred to gynecologic oncology for further management.

Pelvic examination revealed multilobular nodular areas overlying the clitoral hood that extended down to the labia majora. The nodular processes did not involve the clitoris, labia minora, or perineum. A mobile isolated lymph node measuring 2.0×1.0 cm in the right inguinal area also was noted. The patient’s clinical history was notable for right breast carcinoma treated with a right mastectomy with axillary lymph node dissection that showed metastatic disease. She also underwent adjuvant chemotherapy with paclitaxel and doxorubicin for breast carcinoma.

After discussing the diagnostic differential and treatment options, the patient elected to undergo a bilateral partial radical vulvectomy with reconstruction and resection of the right inguinal lymph node. Gross examination of the vulvectomy specimen showed multiple flesh-colored papules (Figure 1). Histologic examination revealed a neoplasm with sweat gland differentiation that was broad and poorly circumscribed but confined to the dermis (Figures 2A and 2B). The neoplasm was composed of epithelial cells that formed ductlike structures, lined by 2 layers of cuboidal epithelium within a fibrous stroma (Figure 2C). A toluidine blue special stain was performed and demonstrated an increased amount of mast cells in the tissue (Figure 3). Immunohistochemical stains for gross cystic disease fluid protein, estrogen receptor (ER), and progesterone receptor (PR) were negative in the tumor cells. The lack of cytologic atypia, perineural invasion, and deep infiltration into the subcutis favored a syringoma. One month later, the case was presented at the Tumor Board Conference at the University of Alabama at Birmingham where a final diagnosis of vulvar syringoma was agreed upon and discussed with the patient. At that time, no recurrence was evident and follow-up was recommended.

Syringomas may occur at any site on the body but are prone to occur on the periorbital area, especially the eyelids.¹ Some of the atypical locations for a syringoma include the anterior neck, chest, abdomen, genitals, axillae, groin, and buttocks.² Vulvar syringomas were first reported by Carneiro³ in 1971 as usually affecting adolescent girls and middle-aged women. There have been approximately 40 reported cases affecting women aged 8 to 78 years.^4,5 Vulvar syringomas classically appear as firm or soft, flesh-colored to transparent, papular lesions. The 2 other clinical variants are miliumlike, whitish, cystic papules as well as lichenoid papules.⁶ Pérez-Bustillo et al⁵ reported a case of the lichenoid papule variant on the labia majora of a 78-year-old woman who presented with intermittent vulvar pruritus of 4 years’ duration. Due to this patient’s 9-year history of urinary incontinence, the lesions had been misdiagnosed as irritant dermatitis and associated lichen simplex chronicus (LSC). This case is a reminder to consider vulvar syringoma in patients with LSC who respond poorly to oral antihistamines and topical steroids.⁵ Rarely, multiple clinical variants may coexist. In a case reported by Dereli et al,⁷ a 19-year-old woman presented with concurrent classical and miliumlike forms of vulvar syringoma.

Vulvar syringomas usually present as multiple lesions involving both sides of the labia majora; however, Blasdale and McLelland⁸ reported a single isolated syringoma of the vulva on the anterior right labia minora that measured 1.0×0.5 cm, leading the lesion to be described as a giant syringoma.

Vulvar syringomas usually are asymptomatic and noticed during routine gynecologic examination. Therefore, it is believed that they likely are underdiagnosed.⁵ When symptomatic, they commonly present with constant⁹ or intermittent⁵ pruritus, which may intensify during menstruation, pregnancy, and summertime.^6,10-12 Gerdsen et al¹⁰ documented a 27-year-old woman who presented with a 2-year history of pruritic vulvar skin lesions that became exacerbated during menstruation, which raised the possibility of cyclical hormonal changes being responsible for periodic exacerbation of vulvar pruritus during menstruation. In addition, patients may experience an increase in size and number of the lesions during pregnancy. Bal et al¹¹ reported a 24-year-old primigravida with vulvar papular lesions that intensified during pregnancy. She had experienced intermittent vulvar pruritus for 12 years but had no change in symptoms during menstruation.¹¹ Few studies have attempted to evaluate the presence of ER and PR in the syringomas. A study of 9 nonvulvar syringomas by Wallace and Smoller¹³ showed ER positivity in 1 case and PR positivity in 8 cases, lending support to the hormonal theory; however, in another case series of 15 vulvar syringomas, Huang et al⁶ failed to show ER and PR expression by immunohistochemical staining. A case report published 3 years earlier documented the first case of PR positivity on a vulvar syringoma.¹⁴ Our patient also was negative for ER and PR, which suggested that hormonal status is important in some but not all syringomas.

Patients with vulgar syringomas also might have coexisting extragenital syringomas in the neck,⁴ eyelids,^6,7,10 and periorbital area,⁶ and thorough examination of the body is essential. If an extragenital syringoma is diagnosed, a vulvar syringoma should be considered, especially when the patient presents with unexplained genital symptoms. Although no proven hereditary transmission pattern has been established, family history of syringomas has been established in several cases.¹⁵ In a case series reported by Huang et al,⁶ 4 of 18 patients reported a family history of periorbital syringomas. In our case, the patient did not report a family history of syringomas.

The differential diagnosis of vulvar lesions with pruritus is broad and includes Fox-Fordyce disease, lichen planus, LSC, epidermal cysts, senile angiomas, dystrophic calcinosis, xanthomas, steatocytomas, soft fibromas, condyloma acuminatum, and candidiasis. Vulvar syringomas might have a nonspecific appearance, and histologic examination is essential to confirm the diagnosis and rule out any malignant process such as MAC, vulvar intraepithelial neoplasia, extramammary Paget disease, or other glandular neoplasms of the vulva.

Microcystic adnexal carcinoma was first reported in 1982 by Goldstein et al¹⁶ as a locally aggressive neoplasm that can be confused with benign adnexal neoplasms, particularly desmoplastic trichoepithelioma, trichoadenoma, and syringoma. Microcystic adnexal carcinomas present as slow-growing, flesh-colored papules that may resemble syringomas and appear in similar body sites. Histologic examination is essential to differentiate between these two entities. Syringomas are tumors confined to the dermis and are composed of multiple small ducts lined by 2 layers of cuboidal epithelium within a dense fibrous stroma. Unlike syringomas, MACs usually infiltrate diffusely into the dermis and subcutis and may extend into the underlying muscle. Although bland cytologic features predominate, perineural invasion frequently is present in MACs. A potential pitfall of misdiagnosis can be caused by a superficial biopsy that may reveal benign histologic appearance, particularly in the upper level of the tumor where it may be confused with a syringoma or a benign follicular neoplasm.¹⁷

The initial biopsy performed on our patient was possibly not deep enough to render an unequivocal diagnosis and therefore bilateral partial radical vulvectomy was considered. After surgery, histologic examination of the resection specimen revealed a poorly circumscribed tumor confined to the dermis. The tumor was broad and the lack of deep infiltration into the subcutis and perineural invasion favored a syringoma (Figures 2A and 2B). These findings were consistent with case reports that documented syringomas as being more wide than deep on microscopic examination, whereas the opposite pertained to MAC.¹⁸ Cases of plaque-type syringomas that initially were misdiagnosed as MACs also have been reported.¹⁹ Because misdiagnosis may affect the treatment plan and potentially result in unnecessary surgery, caution should be taken when differentiating between these two entities. When a definitive diagnosis cannot be rendered on a superficial biopsy, a recommendation should be made for a deeper biopsy sampling the subcutis.

For the majority of the patients with vulvar syringomas, treatment is seldom required due to their asymptomatic nature; however, patients who present with symptoms usually report pruritus of variable intensities and patterns. A standardized treatment does not exist for vulvar syringomas, and oral or topical treatment might be used as an initial approach. Commonly prescribed medications with variable results include topical corticosteroids, oral antihistamines, and topical retinoids. In a case reported by Iwao et al,²⁰ vulvar syringomas were successfully treated with tranilast, which has anti-inflammatory and immunomodulatory effects. This medication could have a possible dual action—inhibiting the release of chemical mediators from the mast cells and inhibiting the release of IL-1β from the eccrine duct, which could suppress the proliferation of stromal connective tissue. Our case was stained with toluidine blue and showed an increased number of mast cells in the tissue (Figure 3). Patients who are unresponsive to tranilast or have extensive disease resulting in cosmetic disfigurement might benefit from more invasive treatment methods including a variety of lasers, cryotherapy, electrosurgery, and excision. Excisions should include the entire tumor to avoid recurrence. In a case reported by Garman and Metry,²¹ the lesions were surgically excised using small 2- to 3-mm punches; however, several weeks later the lesions recurred. Our patient presented with a 1-month evolution of dyspareunia, vulvar discomfort, and vulvar irregularities that were probably not treated with oral or topical medications before being referred for surgery.

We report a case of a vulvar syringoma that presented diagnostic challenges in the initial biopsy, which prevented the exclusion of an MAC. After partial radical vulvectomy, histologic examination was more definitive, showing lack of deep infiltration into the subcutis or perineural invasion that are commonly seen in MAC. This case is an example of a notable pitfall in the diagnosis of vulvar syringoma on a limited biopsy leading to overtreatment. Raising awareness of this entity is the only modality to prevent misdiagnosis. We encourage reporting of further cases of syringomas, particularly those with atypical locations or patterns that may cause diagnostic problems.

To the Editor:

Sweet syndrome (SS), also known as acute febrile neutrophilic dermatosis, is an uncommon inflammatory skin disorder characterized by sudden onset of fever, leukocytosis, neutrophilia, and tender erythematous papules or plaques or both. Skin biopsy usually reveals extensive infiltration of neutrophils into the epidermis and dermis.^1-3 Although rare, cases of eosinophil-rich SS have been reported in patients with drug-induced and malignancy-associated SS.^4,5 We report a case of a patient with classical SS with dermal eosinophilic infiltration.

An 80-year-old Hispanic man presented with abrupt onset of a rash on the posterior scalp, left ear, back, and hands of 5 days’ duration. The lesions were painful and had progressed to the point of impairing hand grip. The patient’s medical history included a reported common cold the week prior, hyperlipidemia, and hypertension, for which he took metoprolol, simvastatin, aspirin, and clopidogrel. He denied oral lesions and medication changes. He was afebrile and did not experience dietary changes, weight loss, or fatigue. He recently returned from travel to the Dominican Republic.

Physical examination revealed tender, well demarcated, pink to violaceous, pseudovesicular papules and plaques on the palms and dorsal hands (Figure 1), the posterior scalp, left ear, proximal left arm, and back. Pink, juicy, targetoid papules were also found on the scalp, back, and left arm. There was no evidence of lymphadenopathy. Laboratory test results revealed an elevated white blood cell count (11,500/µL [reference range, 3800-10,800/µL]), absolute neutrophil count (8073/µL [reference range, 1500–7800/µL]), and eosinophil count (610/µL [reference range, 15–500/µL]). These results indicated leukocytosis with neutrophilia and mild eosinophilia. The patient also was anemic (hemoglobin, 11.5 g/dL [reference range, 13.2–17.1 g/dL]; hematocrit, 35.1% [reference range, 38.5%–50%]). Urine testing revealed altered renal function (serum creatinine, 2.42 mg/dL [reference range, 0.7–1.1 mg/dL]; blood urea nitrogen, 34 mg/dL [reference range, 7–25 mg/dL]; glomerular filtration rate, 4 mL/min/1.73 m² (reference range, ≥60 mL/min/1.73 m²]), suggesting stage 4 chronic kidney disease. Urinalysis showed mild hematuria and proteinuria.

In our patient, the observed clinical and histological findings were consistent with a diagnosis of SS,^2,10 specifically tender erythematous plaques of sudden onset, fast response to systemic corticosteroid therapy, a dermal neutrophilic infiltrate without evidence of leukocytoclastic vasculitis, and leukocytosis greater than 8000/µL with more than 70% neutrophils. He also exhibited targetoid lesions, which have been reported in 7% to 12% of SS patients.^10,11

The predominant cells involved in the dermis of SS lesions are mature neutrophils; however, eosinophils have been observed in small numbers within dermal infiltrates in skin lesions of patients with either classical SS or drug-induced dermatosis.² In 2 studies of cases of SS (N=73 and N=31), eosinophils were reported in 35% and 41% of skin biopsies, respectively.^4,5 Nevertheless, cases with dense eosinophilic infiltrates are rare. Furthermore, Masuda et al¹² reported a case of eosinophil-rich SS in a 29-year-old woman after treatment of an upper respiratory tract infection with an antibiotic, and Soon et al¹³ described an eosinophil-rich case of SS in the setting of new-onset enteropathy-associated T-cell lymphoma.

Our patient was considered to have classical SS because he had an episode of an upper respiratory tract infection 1 week prior to onset of clinical manifestations. The histologic finding of numerous eosinophils in our case was unusual for idiopathic SS. This finding might suggest a drug hypersensitivity reaction, but the lack of any change in the patient’s long-term medication list and the lack of any other episodes made a diagnosis of drug-induced SS less likely in our patient.

Eosinophilic dermatosis of hematologic malignancy is a rare cutaneous condition in which nodules, pruritic papules, and vesicles arise in patients with a hematologic malignancy, such as chronic lymphocytic leukemia and mantle cell lymphoma,¹³ in which a deep perivascular lymphocytic infiltrate and numerous eosinophils are observed. Malignancy was ruled out in our patient because of the lack of characteristic abnormalities in blood testing, the fast response to corticosteroid therapy, and the lack of recurrence posttreatment or additional systemic concerns.

The typical pathology findings of SS consist of mature neutrophils found in the dermis without evidence of leukocytoclastic vasculitis. Eosinophil-rich infiltration, however rare, has been reported in SS. This report highlights a case of classical SS with a particularly dense eosinophilic infiltrate, which could be mistaken for other eosinophilic dermatoses. Dermatologists should be aware of the possibility of marked eosinophilic infiltration in all subtypes of this disorder.

To the Editor:

Sweet syndrome (SS), also known as acute febrile neutrophilic dermatosis, is an uncommon inflammatory skin disorder characterized by sudden onset of fever, leukocytosis, neutrophilia, and tender erythematous papules or plaques or both. Skin biopsy usually reveals extensive infiltration of neutrophils into the epidermis and dermis.^1-3 Although rare, cases of eosinophil-rich SS have been reported in patients with drug-induced and malignancy-associated SS.^4,5 We report a case of a patient with classical SS with dermal eosinophilic infiltration.

An 80-year-old Hispanic man presented with abrupt onset of a rash on the posterior scalp, left ear, back, and hands of 5 days’ duration. The lesions were painful and had progressed to the point of impairing hand grip. The patient’s medical history included a reported common cold the week prior, hyperlipidemia, and hypertension, for which he took metoprolol, simvastatin, aspirin, and clopidogrel. He denied oral lesions and medication changes. He was afebrile and did not experience dietary changes, weight loss, or fatigue. He recently returned from travel to the Dominican Republic.

Physical examination revealed tender, well demarcated, pink to violaceous, pseudovesicular papules and plaques on the palms and dorsal hands (Figure 1), the posterior scalp, left ear, proximal left arm, and back. Pink, juicy, targetoid papules were also found on the scalp, back, and left arm. There was no evidence of lymphadenopathy. Laboratory test results revealed an elevated white blood cell count (11,500/µL [reference range, 3800-10,800/µL]), absolute neutrophil count (8073/µL [reference range, 1500–7800/µL]), and eosinophil count (610/µL [reference range, 15–500/µL]). These results indicated leukocytosis with neutrophilia and mild eosinophilia. The patient also was anemic (hemoglobin, 11.5 g/dL [reference range, 13.2–17.1 g/dL]; hematocrit, 35.1% [reference range, 38.5%–50%]). Urine testing revealed altered renal function (serum creatinine, 2.42 mg/dL [reference range, 0.7–1.1 mg/dL]; blood urea nitrogen, 34 mg/dL [reference range, 7–25 mg/dL]; glomerular filtration rate, 4 mL/min/1.73 m² (reference range, ≥60 mL/min/1.73 m²]), suggesting stage 4 chronic kidney disease. Urinalysis showed mild hematuria and proteinuria.

In our patient, the observed clinical and histological findings were consistent with a diagnosis of SS,^2,10 specifically tender erythematous plaques of sudden onset, fast response to systemic corticosteroid therapy, a dermal neutrophilic infiltrate without evidence of leukocytoclastic vasculitis, and leukocytosis greater than 8000/µL with more than 70% neutrophils. He also exhibited targetoid lesions, which have been reported in 7% to 12% of SS patients.^10,11

The predominant cells involved in the dermis of SS lesions are mature neutrophils; however, eosinophils have been observed in small numbers within dermal infiltrates in skin lesions of patients with either classical SS or drug-induced dermatosis.² In 2 studies of cases of SS (N=73 and N=31), eosinophils were reported in 35% and 41% of skin biopsies, respectively.^4,5 Nevertheless, cases with dense eosinophilic infiltrates are rare. Furthermore, Masuda et al¹² reported a case of eosinophil-rich SS in a 29-year-old woman after treatment of an upper respiratory tract infection with an antibiotic, and Soon et al¹³ described an eosinophil-rich case of SS in the setting of new-onset enteropathy-associated T-cell lymphoma.

Our patient was considered to have classical SS because he had an episode of an upper respiratory tract infection 1 week prior to onset of clinical manifestations. The histologic finding of numerous eosinophils in our case was unusual for idiopathic SS. This finding might suggest a drug hypersensitivity reaction, but the lack of any change in the patient’s long-term medication list and the lack of any other episodes made a diagnosis of drug-induced SS less likely in our patient.

Eosinophilic dermatosis of hematologic malignancy is a rare cutaneous condition in which nodules, pruritic papules, and vesicles arise in patients with a hematologic malignancy, such as chronic lymphocytic leukemia and mantle cell lymphoma,¹³ in which a deep perivascular lymphocytic infiltrate and numerous eosinophils are observed. Malignancy was ruled out in our patient because of the lack of characteristic abnormalities in blood testing, the fast response to corticosteroid therapy, and the lack of recurrence posttreatment or additional systemic concerns.

The typical pathology findings of SS consist of mature neutrophils found in the dermis without evidence of leukocytoclastic vasculitis. Eosinophil-rich infiltration, however rare, has been reported in SS. This report highlights a case of classical SS with a particularly dense eosinophilic infiltrate, which could be mistaken for other eosinophilic dermatoses. Dermatologists should be aware of the possibility of marked eosinophilic infiltration in all subtypes of this disorder.

To the Editor:

Sweet syndrome (SS), also known as acute febrile neutrophilic dermatosis, is an uncommon inflammatory skin disorder characterized by sudden onset of fever, leukocytosis, neutrophilia, and tender erythematous papules or plaques or both. Skin biopsy usually reveals extensive infiltration of neutrophils into the epidermis and dermis.^1-3 Although rare, cases of eosinophil-rich SS have been reported in patients with drug-induced and malignancy-associated SS.^4,5 We report a case of a patient with classical SS with dermal eosinophilic infiltration.

An 80-year-old Hispanic man presented with abrupt onset of a rash on the posterior scalp, left ear, back, and hands of 5 days’ duration. The lesions were painful and had progressed to the point of impairing hand grip. The patient’s medical history included a reported common cold the week prior, hyperlipidemia, and hypertension, for which he took metoprolol, simvastatin, aspirin, and clopidogrel. He denied oral lesions and medication changes. He was afebrile and did not experience dietary changes, weight loss, or fatigue. He recently returned from travel to the Dominican Republic.

Physical examination revealed tender, well demarcated, pink to violaceous, pseudovesicular papules and plaques on the palms and dorsal hands (Figure 1), the posterior scalp, left ear, proximal left arm, and back. Pink, juicy, targetoid papules were also found on the scalp, back, and left arm. There was no evidence of lymphadenopathy. Laboratory test results revealed an elevated white blood cell count (11,500/µL [reference range, 3800-10,800/µL]), absolute neutrophil count (8073/µL [reference range, 1500–7800/µL]), and eosinophil count (610/µL [reference range, 15–500/µL]). These results indicated leukocytosis with neutrophilia and mild eosinophilia. The patient also was anemic (hemoglobin, 11.5 g/dL [reference range, 13.2–17.1 g/dL]; hematocrit, 35.1% [reference range, 38.5%–50%]). Urine testing revealed altered renal function (serum creatinine, 2.42 mg/dL [reference range, 0.7–1.1 mg/dL]; blood urea nitrogen, 34 mg/dL [reference range, 7–25 mg/dL]; glomerular filtration rate, 4 mL/min/1.73 m² (reference range, ≥60 mL/min/1.73 m²]), suggesting stage 4 chronic kidney disease. Urinalysis showed mild hematuria and proteinuria.

In our patient, the observed clinical and histological findings were consistent with a diagnosis of SS,^2,10 specifically tender erythematous plaques of sudden onset, fast response to systemic corticosteroid therapy, a dermal neutrophilic infiltrate without evidence of leukocytoclastic vasculitis, and leukocytosis greater than 8000/µL with more than 70% neutrophils. He also exhibited targetoid lesions, which have been reported in 7% to 12% of SS patients.^10,11

The predominant cells involved in the dermis of SS lesions are mature neutrophils; however, eosinophils have been observed in small numbers within dermal infiltrates in skin lesions of patients with either classical SS or drug-induced dermatosis.² In 2 studies of cases of SS (N=73 and N=31), eosinophils were reported in 35% and 41% of skin biopsies, respectively.^4,5 Nevertheless, cases with dense eosinophilic infiltrates are rare. Furthermore, Masuda et al¹² reported a case of eosinophil-rich SS in a 29-year-old woman after treatment of an upper respiratory tract infection with an antibiotic, and Soon et al¹³ described an eosinophil-rich case of SS in the setting of new-onset enteropathy-associated T-cell lymphoma.

Our patient was considered to have classical SS because he had an episode of an upper respiratory tract infection 1 week prior to onset of clinical manifestations. The histologic finding of numerous eosinophils in our case was unusual for idiopathic SS. This finding might suggest a drug hypersensitivity reaction, but the lack of any change in the patient’s long-term medication list and the lack of any other episodes made a diagnosis of drug-induced SS less likely in our patient.

Eosinophilic dermatosis of hematologic malignancy is a rare cutaneous condition in which nodules, pruritic papules, and vesicles arise in patients with a hematologic malignancy, such as chronic lymphocytic leukemia and mantle cell lymphoma,¹³ in which a deep perivascular lymphocytic infiltrate and numerous eosinophils are observed. Malignancy was ruled out in our patient because of the lack of characteristic abnormalities in blood testing, the fast response to corticosteroid therapy, and the lack of recurrence posttreatment or additional systemic concerns.

The typical pathology findings of SS consist of mature neutrophils found in the dermis without evidence of leukocytoclastic vasculitis. Eosinophil-rich infiltration, however rare, has been reported in SS. This report highlights a case of classical SS with a particularly dense eosinophilic infiltrate, which could be mistaken for other eosinophilic dermatoses. Dermatologists should be aware of the possibility of marked eosinophilic infiltration in all subtypes of this disorder.

To the Editor:

Phytophotodermatitis is common in dermatology during the summer months, especially in individuals who spend time outdoors; however, identification of the offending plant can be challenging. We report a case of phytophotodermatitis in which the causative plant, common rue, was not identified until it was revealed that the patient was a butterfly enthusiast.

A 60-year-old woman presented to the outpatient dermatology clinic in late summer for a routine skin examination. An eruption was noted over the right thigh and knee that had first appeared approximately 2 weeks prior. The rash started as pruritic blisters but gradually progressed to erythema and then eventually to brown markings, which were observed at the current presentation. Physical examination revealed hyperpigmented, brown, streaky, linear patches and plaques over the right thigh, knee, and lower leg (Figure). When asked about her hobbies, the patient reported an affinity for butterflies and noted that she attracts them with specific species of plants in her garden. She recalled recently planting the herb of grace, or common rue, to attract the giant swallowtail butterfly (Papilio cresphontes). Upon further inquiry, she remembered working in the garden on her knees and digging up roots near the common rue plant while wearing shorts approximately 2 weeks prior to the current presentation. Given the streaky linear pattern of the eruption along with recent sun exposure and exposure to the common rue plant, a diagnosis of phytophotodermatitis was made. No further treatment was sought, as the eruption was not bothersome to her. She was intrigued that the common rue plant had caused the dermatitis and planned on taking proper precautions when working near the plant in the future.

This case illustrates a unique perspective of phytophotodermatitis, as butterfly enthusiasm is not commonly reported in association with the common rue plant with respect to phytophotodermatitis. This case underscores the importance of inquiring about patients’ professions and hobbies, both in dermatology and other specialties.

To the Editor:

Phytophotodermatitis is common in dermatology during the summer months, especially in individuals who spend time outdoors; however, identification of the offending plant can be challenging. We report a case of phytophotodermatitis in which the causative plant, common rue, was not identified until it was revealed that the patient was a butterfly enthusiast.

A 60-year-old woman presented to the outpatient dermatology clinic in late summer for a routine skin examination. An eruption was noted over the right thigh and knee that had first appeared approximately 2 weeks prior. The rash started as pruritic blisters but gradually progressed to erythema and then eventually to brown markings, which were observed at the current presentation. Physical examination revealed hyperpigmented, brown, streaky, linear patches and plaques over the right thigh, knee, and lower leg (Figure). When asked about her hobbies, the patient reported an affinity for butterflies and noted that she attracts them with specific species of plants in her garden. She recalled recently planting the herb of grace, or common rue, to attract the giant swallowtail butterfly (Papilio cresphontes). Upon further inquiry, she remembered working in the garden on her knees and digging up roots near the common rue plant while wearing shorts approximately 2 weeks prior to the current presentation. Given the streaky linear pattern of the eruption along with recent sun exposure and exposure to the common rue plant, a diagnosis of phytophotodermatitis was made. No further treatment was sought, as the eruption was not bothersome to her. She was intrigued that the common rue plant had caused the dermatitis and planned on taking proper precautions when working near the plant in the future.

This case illustrates a unique perspective of phytophotodermatitis, as butterfly enthusiasm is not commonly reported in association with the common rue plant with respect to phytophotodermatitis. This case underscores the importance of inquiring about patients’ professions and hobbies, both in dermatology and other specialties.

To the Editor:

Phytophotodermatitis is common in dermatology during the summer months, especially in individuals who spend time outdoors; however, identification of the offending plant can be challenging. We report a case of phytophotodermatitis in which the causative plant, common rue, was not identified until it was revealed that the patient was a butterfly enthusiast.

A 60-year-old woman presented to the outpatient dermatology clinic in late summer for a routine skin examination. An eruption was noted over the right thigh and knee that had first appeared approximately 2 weeks prior. The rash started as pruritic blisters but gradually progressed to erythema and then eventually to brown markings, which were observed at the current presentation. Physical examination revealed hyperpigmented, brown, streaky, linear patches and plaques over the right thigh, knee, and lower leg (Figure). When asked about her hobbies, the patient reported an affinity for butterflies and noted that she attracts them with specific species of plants in her garden. She recalled recently planting the herb of grace, or common rue, to attract the giant swallowtail butterfly (Papilio cresphontes). Upon further inquiry, she remembered working in the garden on her knees and digging up roots near the common rue plant while wearing shorts approximately 2 weeks prior to the current presentation. Given the streaky linear pattern of the eruption along with recent sun exposure and exposure to the common rue plant, a diagnosis of phytophotodermatitis was made. No further treatment was sought, as the eruption was not bothersome to her. She was intrigued that the common rue plant had caused the dermatitis and planned on taking proper precautions when working near the plant in the future.

This case illustrates a unique perspective of phytophotodermatitis, as butterfly enthusiasm is not commonly reported in association with the common rue plant with respect to phytophotodermatitis. This case underscores the importance of inquiring about patients’ professions and hobbies, both in dermatology and other specialties.

To the Editor:

The use of sunless tanners has become an alternative for individuals who wish to have tan skin without exposure to UV radiation.¹ We present a case of a patient who experienced persistent hyperpigmented patches on the hands months after the use of a sunless tanner containing dihydroxyacetone (DHA), a carbohydrate that reacts with amino acids in the stratum corneum to produce pigments called melanoidins. The hyperpigmentation caused by DHA is due to the Maillard reaction, which is the nonenzymatic glycation of amino groups of proteins by the carbonyl groups of sugar.² Many sunless tanners contain DHA at varying concentrations. Dermatologists should be aware of the benefits and potential side effects of these alternative products so that they can appropriately counsel patients.

A 20-year-old woman with no history of skin disease presented for evaluation of hyperpigmented patches on the dorsal hands of several months’ duration. Physical examination revealed ill-defined hyperpigmented patches on the dorsal fingers without associated scale or erythema (Figure 1). She had a remote history of Hodgkin lymphoma treated with chemotherapy and was in remission for 5 years prior to the current presentation. Her hematologists referred her to dermatology for evaluation, as they did not believe the patches could be related to her chemotherapy given that she had completed the treatment years before.

Products with DHA only confer a sun protection factor of approximately 3⁴; although patients may appear tan, they have no actual decreased risk for sunburn after use. Reports have shown that the use of sunless tanners containing DHA can alter the appearance of melanocytic lesions clinically and has caused pseudochromhidrosis on the palms.^3,5,6 A study performed on a human keratinocyte cell line, HaCaT, showed that DHA can induce DNA damage, cell-cycle block, and apoptosis.⁷ In addition, as described in our case, patients may experience prolonged hyperpigmentation after use.

This case demonstrates the potential for persistent hyperpigmentation months after the use of sunless tanners containing DHA. Asking patients specific questions regarding their history of tanning product use is essential in identifying the pathology. Although a skin biopsy may not be strictly indicated, it may aid diagnosis, especially when the history is unclear. As more dermatologists support the use of sunless tanner, we must be aware of this possible outcome, especially on more cosmetically sensitive areas such as the fingers in this patient. Clinicians should be aware that the US Food and Drug Administration recommends avoiding contact with mucous membranes when applying products containing DHA and also recommends use of a test spot prior to treating the entire body with the product.⁸ Patients must not only be educated on the benefits of using sunless tanners but on the potential side effects with use of these products as well.

To the Editor:

The use of sunless tanners has become an alternative for individuals who wish to have tan skin without exposure to UV radiation.¹ We present a case of a patient who experienced persistent hyperpigmented patches on the hands months after the use of a sunless tanner containing dihydroxyacetone (DHA), a carbohydrate that reacts with amino acids in the stratum corneum to produce pigments called melanoidins. The hyperpigmentation caused by DHA is due to the Maillard reaction, which is the nonenzymatic glycation of amino groups of proteins by the carbonyl groups of sugar.² Many sunless tanners contain DHA at varying concentrations. Dermatologists should be aware of the benefits and potential side effects of these alternative products so that they can appropriately counsel patients.

A 20-year-old woman with no history of skin disease presented for evaluation of hyperpigmented patches on the dorsal hands of several months’ duration. Physical examination revealed ill-defined hyperpigmented patches on the dorsal fingers without associated scale or erythema (Figure 1). She had a remote history of Hodgkin lymphoma treated with chemotherapy and was in remission for 5 years prior to the current presentation. Her hematologists referred her to dermatology for evaluation, as they did not believe the patches could be related to her chemotherapy given that she had completed the treatment years before.

Products with DHA only confer a sun protection factor of approximately 3⁴; although patients may appear tan, they have no actual decreased risk for sunburn after use. Reports have shown that the use of sunless tanners containing DHA can alter the appearance of melanocytic lesions clinically and has caused pseudochromhidrosis on the palms.^3,5,6 A study performed on a human keratinocyte cell line, HaCaT, showed that DHA can induce DNA damage, cell-cycle block, and apoptosis.⁷ In addition, as described in our case, patients may experience prolonged hyperpigmentation after use.

This case demonstrates the potential for persistent hyperpigmentation months after the use of sunless tanners containing DHA. Asking patients specific questions regarding their history of tanning product use is essential in identifying the pathology. Although a skin biopsy may not be strictly indicated, it may aid diagnosis, especially when the history is unclear. As more dermatologists support the use of sunless tanner, we must be aware of this possible outcome, especially on more cosmetically sensitive areas such as the fingers in this patient. Clinicians should be aware that the US Food and Drug Administration recommends avoiding contact with mucous membranes when applying products containing DHA and also recommends use of a test spot prior to treating the entire body with the product.⁸ Patients must not only be educated on the benefits of using sunless tanners but on the potential side effects with use of these products as well.

To the Editor:

The use of sunless tanners has become an alternative for individuals who wish to have tan skin without exposure to UV radiation.¹ We present a case of a patient who experienced persistent hyperpigmented patches on the hands months after the use of a sunless tanner containing dihydroxyacetone (DHA), a carbohydrate that reacts with amino acids in the stratum corneum to produce pigments called melanoidins. The hyperpigmentation caused by DHA is due to the Maillard reaction, which is the nonenzymatic glycation of amino groups of proteins by the carbonyl groups of sugar.² Many sunless tanners contain DHA at varying concentrations. Dermatologists should be aware of the benefits and potential side effects of these alternative products so that they can appropriately counsel patients.

A 20-year-old woman with no history of skin disease presented for evaluation of hyperpigmented patches on the dorsal hands of several months’ duration. Physical examination revealed ill-defined hyperpigmented patches on the dorsal fingers without associated scale or erythema (Figure 1). She had a remote history of Hodgkin lymphoma treated with chemotherapy and was in remission for 5 years prior to the current presentation. Her hematologists referred her to dermatology for evaluation, as they did not believe the patches could be related to her chemotherapy given that she had completed the treatment years before.

Products with DHA only confer a sun protection factor of approximately 3⁴; although patients may appear tan, they have no actual decreased risk for sunburn after use. Reports have shown that the use of sunless tanners containing DHA can alter the appearance of melanocytic lesions clinically and has caused pseudochromhidrosis on the palms.^3,5,6 A study performed on a human keratinocyte cell line, HaCaT, showed that DHA can induce DNA damage, cell-cycle block, and apoptosis.⁷ In addition, as described in our case, patients may experience prolonged hyperpigmentation after use.

This case demonstrates the potential for persistent hyperpigmentation months after the use of sunless tanners containing DHA. Asking patients specific questions regarding their history of tanning product use is essential in identifying the pathology. Although a skin biopsy may not be strictly indicated, it may aid diagnosis, especially when the history is unclear. As more dermatologists support the use of sunless tanner, we must be aware of this possible outcome, especially on more cosmetically sensitive areas such as the fingers in this patient. Clinicians should be aware that the US Food and Drug Administration recommends avoiding contact with mucous membranes when applying products containing DHA and also recommends use of a test spot prior to treating the entire body with the product.⁸ Patients must not only be educated on the benefits of using sunless tanners but on the potential side effects with use of these products as well.

To the Editor:

Pseudoepitheliomatous hyperplasia (PEH) is an uncommon type of reactive epidermal proliferation that can occur from a variety of causes, including an underlying infection, inflammation, neoplastic condition, or trauma induced from tattooing.¹ Diagnosis can be challenging and requires clinicopathologic correlation, as PEH can mimic malignancy on histopathology.^2-4 Histologically, PEH shows irregular hyperplasia of the epidermis and adnexal epithelium, elongation of the rete ridges, and extension of the reactive proliferation into the dermis. Absence of cytologic atypia is key to the diagnosis of PEH, helping to distinguish it from squamous cell carcinoma and keratoacanthoma. Clinically, patients typically present with well-demarcated, erythematous, scaly plaques or nodules in reactive areas, which can be symptomatically pruritic.

A 48-year-old woman presented with scaly and crusted verrucous plaques of 2 months’ duration that were isolated to the areas of purple pigment within a tattoo on the right lower leg. The patient reported pruritus in the affected areas that occurred immediately after obtaining the tattoo, which was her first and only tattoo. She denied any pertinent medical history, including an absence of immunosuppression and autoimmune or chronic inflammatory diseases.

Physical examination revealed scaly and crusted plaques isolated to areas of purple tattoo pigment (Figure 1). Areas of red, green, black, and blue pigmentation within the tattoo were uninvolved. With the initial suspicion of allergic contact dermatitis, two 6-mm punch biopsies were taken from adjacent linear plaques on the right leg for histology and tissue culture. Histopathologic evaluation revealed dermal tattoo pigment with overlying PEH and was negative for signs of infection (Figure 2). Infectious stains such as periodic acid–Schiff, Grocott-Gomori methenamine-silver, and Gram stains were performed and found to be negative. In addition, culture for mycobacteria came back negative. Prurigo was on the differential; however, histopathologic changes were more compatible with a PEH reaction to the tattoo.

An increase in the popularity of tattooing has led to more reports of various tattoo skin reactions.^4-6 The differential diagnosis is broad for tattoo reactions and includes granulomatous inflammation, sarcoidosis, psoriasis (Köbner phenomenon), allergic contact dermatitis, lichen planus, morphealike reactions, squamous cell carcinoma, and keratoacanthoma,⁵ which makes clinicopathologic correlation essential for accurate diagnosis. Our case demonstrated the characteristic epithelial hyperplasia in the absence of cytologic atypia. In addition, the presence of mixed dermal inflammation histologically was noted in our patient.

We report an unusual case of PEH occurring secondary to purple tattoo pigment. Our report also demonstrates the clinical and symptomatic improvement of PEH that can be achieved through the use of intralesional corticosteroid therapy. Our patient represents a case of PEH reactive to tattooing with purple ink. Further research to elucidate the precise pathogenesis of PEH tattoo reactions would be helpful in identifying high-risk patients and determining the most efficacious treatments.

To the Editor:

Pseudoepitheliomatous hyperplasia (PEH) is an uncommon type of reactive epidermal proliferation that can occur from a variety of causes, including an underlying infection, inflammation, neoplastic condition, or trauma induced from tattooing.¹ Diagnosis can be challenging and requires clinicopathologic correlation, as PEH can mimic malignancy on histopathology.^2-4 Histologically, PEH shows irregular hyperplasia of the epidermis and adnexal epithelium, elongation of the rete ridges, and extension of the reactive proliferation into the dermis. Absence of cytologic atypia is key to the diagnosis of PEH, helping to distinguish it from squamous cell carcinoma and keratoacanthoma. Clinically, patients typically present with well-demarcated, erythematous, scaly plaques or nodules in reactive areas, which can be symptomatically pruritic.

A 48-year-old woman presented with scaly and crusted verrucous plaques of 2 months’ duration that were isolated to the areas of purple pigment within a tattoo on the right lower leg. The patient reported pruritus in the affected areas that occurred immediately after obtaining the tattoo, which was her first and only tattoo. She denied any pertinent medical history, including an absence of immunosuppression and autoimmune or chronic inflammatory diseases.

Physical examination revealed scaly and crusted plaques isolated to areas of purple tattoo pigment (Figure 1). Areas of red, green, black, and blue pigmentation within the tattoo were uninvolved. With the initial suspicion of allergic contact dermatitis, two 6-mm punch biopsies were taken from adjacent linear plaques on the right leg for histology and tissue culture. Histopathologic evaluation revealed dermal tattoo pigment with overlying PEH and was negative for signs of infection (Figure 2). Infectious stains such as periodic acid–Schiff, Grocott-Gomori methenamine-silver, and Gram stains were performed and found to be negative. In addition, culture for mycobacteria came back negative. Prurigo was on the differential; however, histopathologic changes were more compatible with a PEH reaction to the tattoo.

An increase in the popularity of tattooing has led to more reports of various tattoo skin reactions.^4-6 The differential diagnosis is broad for tattoo reactions and includes granulomatous inflammation, sarcoidosis, psoriasis (Köbner phenomenon), allergic contact dermatitis, lichen planus, morphealike reactions, squamous cell carcinoma, and keratoacanthoma,⁵ which makes clinicopathologic correlation essential for accurate diagnosis. Our case demonstrated the characteristic epithelial hyperplasia in the absence of cytologic atypia. In addition, the presence of mixed dermal inflammation histologically was noted in our patient.

We report an unusual case of PEH occurring secondary to purple tattoo pigment. Our report also demonstrates the clinical and symptomatic improvement of PEH that can be achieved through the use of intralesional corticosteroid therapy. Our patient represents a case of PEH reactive to tattooing with purple ink. Further research to elucidate the precise pathogenesis of PEH tattoo reactions would be helpful in identifying high-risk patients and determining the most efficacious treatments.

To the Editor:

Pseudoepitheliomatous hyperplasia (PEH) is an uncommon type of reactive epidermal proliferation that can occur from a variety of causes, including an underlying infection, inflammation, neoplastic condition, or trauma induced from tattooing.¹ Diagnosis can be challenging and requires clinicopathologic correlation, as PEH can mimic malignancy on histopathology.^2-4 Histologically, PEH shows irregular hyperplasia of the epidermis and adnexal epithelium, elongation of the rete ridges, and extension of the reactive proliferation into the dermis. Absence of cytologic atypia is key to the diagnosis of PEH, helping to distinguish it from squamous cell carcinoma and keratoacanthoma. Clinically, patients typically present with well-demarcated, erythematous, scaly plaques or nodules in reactive areas, which can be symptomatically pruritic.

A 48-year-old woman presented with scaly and crusted verrucous plaques of 2 months’ duration that were isolated to the areas of purple pigment within a tattoo on the right lower leg. The patient reported pruritus in the affected areas that occurred immediately after obtaining the tattoo, which was her first and only tattoo. She denied any pertinent medical history, including an absence of immunosuppression and autoimmune or chronic inflammatory diseases.

Physical examination revealed scaly and crusted plaques isolated to areas of purple tattoo pigment (Figure 1). Areas of red, green, black, and blue pigmentation within the tattoo were uninvolved. With the initial suspicion of allergic contact dermatitis, two 6-mm punch biopsies were taken from adjacent linear plaques on the right leg for histology and tissue culture. Histopathologic evaluation revealed dermal tattoo pigment with overlying PEH and was negative for signs of infection (Figure 2). Infectious stains such as periodic acid–Schiff, Grocott-Gomori methenamine-silver, and Gram stains were performed and found to be negative. In addition, culture for mycobacteria came back negative. Prurigo was on the differential; however, histopathologic changes were more compatible with a PEH reaction to the tattoo.

An increase in the popularity of tattooing has led to more reports of various tattoo skin reactions.^4-6 The differential diagnosis is broad for tattoo reactions and includes granulomatous inflammation, sarcoidosis, psoriasis (Köbner phenomenon), allergic contact dermatitis, lichen planus, morphealike reactions, squamous cell carcinoma, and keratoacanthoma,⁵ which makes clinicopathologic correlation essential for accurate diagnosis. Our case demonstrated the characteristic epithelial hyperplasia in the absence of cytologic atypia. In addition, the presence of mixed dermal inflammation histologically was noted in our patient.

We report an unusual case of PEH occurring secondary to purple tattoo pigment. Our report also demonstrates the clinical and symptomatic improvement of PEH that can be achieved through the use of intralesional corticosteroid therapy. Our patient represents a case of PEH reactive to tattooing with purple ink. Further research to elucidate the precise pathogenesis of PEH tattoo reactions would be helpful in identifying high-risk patients and determining the most efficacious treatments.

To the Editor:

Lymphomatoid papulosis is a rare chronic skin disorder characterized by recurrent, self-healing crops of papulonodular eruptions, often resembling cutaneous T-cell lymphoma.¹ Oral bexarotene, a retinoid X receptor–selective retinoid, can be used to control the disease.^2,3 Lichen planopilaris (LPP) is a type of cicatricial alopecia characterized by irreversible hair loss, perifollicular inflammation, and follicular hyperkeratosis, commonly affecting the scalp vertex in adults.⁴ We report a case of a patient with lymphomatoid papulosis who was treated with bexarotene and subsequently developed LPP. We also discuss a proposed mechanism by which bexarotene may have influenced the onset of LPP.

A 35-year-old woman who was previously healthy initially presented with recurrent pruritic papular eruptions on the flank, axillae, and groin of several months’ duration. The lesions appeared as 2-mm, flat-topped, violaceous papules. The patient had no known drug allergies, no medical or family history of skin disease, and was only taking 3000 mg/d of omega-3 fatty acids (fish oil). Histopathologic examination of a biopsy specimen from the inner thigh showed enlarged, atypical, dermal lymphocytes that were CD30⁺ (Figure 1). These findings were consistent with lymphomatoid papulosis. As she had undergone tubal ligation several years prior, she was prescribed oral bexarotene 300 mg once daily in addition to triamcinolone cream 0.1% twice daily, as needed. Symptoms were well controlled on this regimen.

Although the exact mechanism of LPP is not fully understood, studies have suggested that cellular lipid metabolism may be responsible for the inflammation of the pilosebaceous unit.^4-11 Hyperlipidemia is the most common side effect of oral bexarotene, typically occurring within the first 2 to 4 weeks of treatment.^3,12 Considering the insights into the role of lipid regulation on LPP pathogenesis, it is reasonable to suspect that the dyslipidemia caused by bexarotene may have triggered the onset of LPP in our patient. The patient’s lipid values mostly remained within reference range throughout the course of treatment, though she did have elevation of triglycerides around the onset of LPP. Dyslipidemia has been reported in patients with lichen planus but not in patients with LPP. One case-control study showed no dyslipidemia in patients with LPP, but the triglyceride levels were not tracked over time and patients had varying durations since onset of disease at presentation.^9-11,13 In our case, we were fortunate to have this information, and it may suggest an interaction between lipid dysregulation and the development of LPP. It would be interesting to explore this further in a larger patient population and to evaluate if control of dyslipidemia reduces progression of disease as it appears to have done for our patient.

To the Editor:

Lymphomatoid papulosis is a rare chronic skin disorder characterized by recurrent, self-healing crops of papulonodular eruptions, often resembling cutaneous T-cell lymphoma.¹ Oral bexarotene, a retinoid X receptor–selective retinoid, can be used to control the disease.^2,3 Lichen planopilaris (LPP) is a type of cicatricial alopecia characterized by irreversible hair loss, perifollicular inflammation, and follicular hyperkeratosis, commonly affecting the scalp vertex in adults.⁴ We report a case of a patient with lymphomatoid papulosis who was treated with bexarotene and subsequently developed LPP. We also discuss a proposed mechanism by which bexarotene may have influenced the onset of LPP.

A 35-year-old woman who was previously healthy initially presented with recurrent pruritic papular eruptions on the flank, axillae, and groin of several months’ duration. The lesions appeared as 2-mm, flat-topped, violaceous papules. The patient had no known drug allergies, no medical or family history of skin disease, and was only taking 3000 mg/d of omega-3 fatty acids (fish oil). Histopathologic examination of a biopsy specimen from the inner thigh showed enlarged, atypical, dermal lymphocytes that were CD30⁺ (Figure 1). These findings were consistent with lymphomatoid papulosis. As she had undergone tubal ligation several years prior, she was prescribed oral bexarotene 300 mg once daily in addition to triamcinolone cream 0.1% twice daily, as needed. Symptoms were well controlled on this regimen.

Although the exact mechanism of LPP is not fully understood, studies have suggested that cellular lipid metabolism may be responsible for the inflammation of the pilosebaceous unit.^4-11 Hyperlipidemia is the most common side effect of oral bexarotene, typically occurring within the first 2 to 4 weeks of treatment.^3,12 Considering the insights into the role of lipid regulation on LPP pathogenesis, it is reasonable to suspect that the dyslipidemia caused by bexarotene may have triggered the onset of LPP in our patient. The patient’s lipid values mostly remained within reference range throughout the course of treatment, though she did have elevation of triglycerides around the onset of LPP. Dyslipidemia has been reported in patients with lichen planus but not in patients with LPP. One case-control study showed no dyslipidemia in patients with LPP, but the triglyceride levels were not tracked over time and patients had varying durations since onset of disease at presentation.^9-11,13 In our case, we were fortunate to have this information, and it may suggest an interaction between lipid dysregulation and the development of LPP. It would be interesting to explore this further in a larger patient population and to evaluate if control of dyslipidemia reduces progression of disease as it appears to have done for our patient.

To the Editor:

Lymphomatoid papulosis is a rare chronic skin disorder characterized by recurrent, self-healing crops of papulonodular eruptions, often resembling cutaneous T-cell lymphoma.¹ Oral bexarotene, a retinoid X receptor–selective retinoid, can be used to control the disease.^2,3 Lichen planopilaris (LPP) is a type of cicatricial alopecia characterized by irreversible hair loss, perifollicular inflammation, and follicular hyperkeratosis, commonly affecting the scalp vertex in adults.⁴ We report a case of a patient with lymphomatoid papulosis who was treated with bexarotene and subsequently developed LPP. We also discuss a proposed mechanism by which bexarotene may have influenced the onset of LPP.

A 35-year-old woman who was previously healthy initially presented with recurrent pruritic papular eruptions on the flank, axillae, and groin of several months’ duration. The lesions appeared as 2-mm, flat-topped, violaceous papules. The patient had no known drug allergies, no medical or family history of skin disease, and was only taking 3000 mg/d of omega-3 fatty acids (fish oil). Histopathologic examination of a biopsy specimen from the inner thigh showed enlarged, atypical, dermal lymphocytes that were CD30⁺ (Figure 1). These findings were consistent with lymphomatoid papulosis. As she had undergone tubal ligation several years prior, she was prescribed oral bexarotene 300 mg once daily in addition to triamcinolone cream 0.1% twice daily, as needed. Symptoms were well controlled on this regimen.

Although the exact mechanism of LPP is not fully understood, studies have suggested that cellular lipid metabolism may be responsible for the inflammation of the pilosebaceous unit.^4-11 Hyperlipidemia is the most common side effect of oral bexarotene, typically occurring within the first 2 to 4 weeks of treatment.^3,12 Considering the insights into the role of lipid regulation on LPP pathogenesis, it is reasonable to suspect that the dyslipidemia caused by bexarotene may have triggered the onset of LPP in our patient. The patient’s lipid values mostly remained within reference range throughout the course of treatment, though she did have elevation of triglycerides around the onset of LPP. Dyslipidemia has been reported in patients with lichen planus but not in patients with LPP. One case-control study showed no dyslipidemia in patients with LPP, but the triglyceride levels were not tracked over time and patients had varying durations since onset of disease at presentation.^9-11,13 In our case, we were fortunate to have this information, and it may suggest an interaction between lipid dysregulation and the development of LPP. It would be interesting to explore this further in a larger patient population and to evaluate if control of dyslipidemia reduces progression of disease as it appears to have done for our patient.