Cutis

The cutaneous manifestations of Crohn disease (CD) are varied, including pyoderma gangrenosum, erythema nodosum, and metastatic CD (MCD). First described by Parks et al,¹ MCD is defined as the occurrence of granulomatous lesions at a skin site distant from the gastrointestinal tract.^1-20 Metastatic CD presents a diagnostic challenge because it is a rare component in the spectrum of inflammatory bowel disease complications, and many physicians are unaware of its existence. It may precede, coincide with, or develop after the diagnosis of intestinal disease.^2-5 Vulvoperineal involvement is particularly problematic because a multitude of other, more likely disease processes are considered first. Typically it is initially diagnosed as a presumed infection prompting reflexive treatment with antivirals, antifungals, and antibiotics. Patients may experience symptoms for years prior to correct diagnosis and institution of proper therapy. A variety of clinical presentations have been described, including nonspecific pain and swelling, erythematous papules and plaques, and nonhealing ulcers. Skin biopsy characteristically confirms the diagnosis and reveals dermal noncaseating granulomas. Multiple oral and parenteral therapies are available, with surgical intervention reserved for resistant cases. We present a case of vulvovaginal MCD in the setting of well-controlled intestinal disease. We also provide a review of the literature regarding genital CD and emphasize the need to keep MCD in the differential of vulvoperineal pathology.

Case Report

A 29-year-old woman was referred to the dermatology clinic with vulvar pain, swelling, and pruritus of 14 months’ duration. Her medical history was remarkable for CD following a colectomy with colostomy. Prior therapies included methotrexate with infliximab for 5 years followed by a 2-year regimen with adalimumab, which induced remission of the intestinal disease.

The patient previously had taken a variety of topical and oral antimicrobials based on treatment from a primary care physician because fungal, bacterial, and viral infections initially were suspected; however, the vulvar disease persisted, and drug-induced immunosuppression was considered to be an underlying factor. Thus, adalimumab was discontinued. Despite elimination of the biologic, the vulvar disease progressed, which prompted referral to the dermatology clinic.

Physical examination revealed diffuse vulvar edema with overlying erythema and scale (Figure 1A). Upon closer inspection, scattered violaceous papules atop a backdrop of lichenification were evident, imparting a cobblestone appearance (Figure 1B). Additionally, a fissure was present on the gluteal cleft. Biopsy from the left labia majora demonstrated well-formed granulomas within a fibrotic reticular dermis (Figures 2A and 2B). The granulomas consisted of both mononucleated and multinucleated histiocytes, rimmed peripherally by lymphocytes and plasma cells (Figure 2C). Periodic acid–Schiff–diastase and acid-fast bacilli stains as well as polarizing microscopy were negative.

Comment

Crohn disease is an idiopathic chronic inflammatory condition that primarily affects the gastrointestinal tract, anywhere from the mouth to the anus. It is characterized by transmural inflammation and fissures that can extend beyond the muscularis propria.^4,6 Extraintestinal manifestations are common.³

Cutaneous CD often presents as perianal, perifistular, or peristomal inflammation or ulceration.⁷ Other skin manifestations include pyoderma gangrenosum, erythema nodosum, erythema multiforme, epidermolysis bullosa acquisita, and palmar erythema.⁷ Metastatic CD involves skin noncontiguous with the gastrointestinal tract^1-20 and may involve any portion of the cutis. Although rare, MCD is the typical etiology underlying vulvar CD.⁸

Approximately 20% of MCD patients have cutaneous lesions without a history of gastrointestinal disease. More than half of cases in adults and approximately two-thirds in children involve the genitalia. Although more common in adults, vulvar involvement has been reported in children as young as 6 years of age.² Diagnosis is especially challenging when bowel symptoms are absent; those patients should be evaluated and followed for subsequent intestinal involvement.⁶

Clinically, symptoms may include general discomfort, pain, pruritus, and dyspareunia. Psychosocial and sexual dysfunction are prevalent and also should be addressed.⁹ Depending on the stage of the disease, physical examination may reveal erythema, edema, papules, pustules, nodules, condylomatous lesions, abscesses, fissures, fistulas, ulceration, acrochordons, and scarring.^2-6,10,11

A host of infections (ie, mycobacterial, actinomycosis, deep fungal, sexually transmitted, schistosomiasis), inflammatory conditions (ie, sarcoid, hidradenitis suppurativa), foreign body reactions, Melkersson-Rosenthal syndrome, and sexual abuse should be included in the differential diagnosis.^2,6,10-12 Once infection, sarcoid, and foreign body reaction have been ruled out, noncaseating granulomas in skin are highly suggestive of CD.⁷

Histopathologic findings of MCD reveal myriad morphological reaction patterns,^5,13 including high-grade dysplasia and carcinoma of the vulva; therefore, it may be imprudent to withhold diagnosis based on the absence of the historically pathognomonic noncaseating granulomas.⁵

The etiopathogenesis of MCD remains an enigma. Dermatopathologic examinations consistently reveal a vascular injury syndrome,¹³ implicating a possible circulatory system contribution via deposition of immune complexes or antigens in skin.⁷ Bacterial infection has been implicated in the intestinal manifestations of CD; however, failure to detect microbial ribosomal RNA in MCD biopsies refutes theories of hematogenous spread of microbes.¹³ Another plausible explanation is that antibodies are formed to conserved microbial epitopes following loss of tolerance to gut flora, which results in an excessive immunologic response at distinct sites in susceptible individuals.¹³ A T-lymphocyte–mediated type IV hypersensitivity reaction also has been proposed via cross-reactivity of lymphocytes, with skin antigens precipitating extraintestinal granuloma formation and vascular injury.³ Clearly, further investigation is needed.

Magnetic resonanance imaging can identify the extent and anatomy of intestinal and pelvic disease and can assist in the diagnosis of vulvar CD.^10,11,14 For these reasons, some experts propose that imaging should be instituted prior to therapy,^12,15,16 especially when direct extension is suspected.¹⁷

Treatment is challenging and often involves collaboration among several specialties.¹² Many treatment options exist because therapeutic responses vary and genital MCD is frequently recalcitrant to therapy.⁴ Medical therapy includes antibiotics such as metronidazole, corticosteroids (ie, topical, intralesional, systemic), and immune modulators (eg, azathioprine, 6-mercaptopurine, cyclosporine, methotrexate, mycophenolate mofetil, tumor necrosis factor α inhibitors).^{2,3,6,10,16,18} Thalidomide has been used for refractory cases.¹⁹ These treatments can be used alone or in combination. Patients should be monitored for side effects and informed that many treatment regimens may be required before a sustained response is achieved.^4,16,18 Surgery is reserved for the most resistant cases. Extensive radical excision of the involved area is the best approach, as limited local excision often is followed by recurrence.²⁰

Conclusion

Our case highlights that vulvar CD can develop in the setting of well-controlled intestinal disease. Vulvoperineal CD should be considered in the differential diagnosis of chronic vulvar pain, swelling, and pruritus, especially in cases resistant to standard therapies and regardless of whether or not gastrointestinal tract symptoms are present. Physicians must be cognizant that vulvar signs and symptoms may precede, coincide with, or follow the diagnosis of intestinal CD. Increased awareness of this entity may facilitate its early recognition and prompt more timely treatment among women with vulvar disease caused by MCD.

The cutaneous manifestations of Crohn disease (CD) are varied, including pyoderma gangrenosum, erythema nodosum, and metastatic CD (MCD). First described by Parks et al,¹ MCD is defined as the occurrence of granulomatous lesions at a skin site distant from the gastrointestinal tract.^1-20 Metastatic CD presents a diagnostic challenge because it is a rare component in the spectrum of inflammatory bowel disease complications, and many physicians are unaware of its existence. It may precede, coincide with, or develop after the diagnosis of intestinal disease.^2-5 Vulvoperineal involvement is particularly problematic because a multitude of other, more likely disease processes are considered first. Typically it is initially diagnosed as a presumed infection prompting reflexive treatment with antivirals, antifungals, and antibiotics. Patients may experience symptoms for years prior to correct diagnosis and institution of proper therapy. A variety of clinical presentations have been described, including nonspecific pain and swelling, erythematous papules and plaques, and nonhealing ulcers. Skin biopsy characteristically confirms the diagnosis and reveals dermal noncaseating granulomas. Multiple oral and parenteral therapies are available, with surgical intervention reserved for resistant cases. We present a case of vulvovaginal MCD in the setting of well-controlled intestinal disease. We also provide a review of the literature regarding genital CD and emphasize the need to keep MCD in the differential of vulvoperineal pathology.

Case Report

A 29-year-old woman was referred to the dermatology clinic with vulvar pain, swelling, and pruritus of 14 months’ duration. Her medical history was remarkable for CD following a colectomy with colostomy. Prior therapies included methotrexate with infliximab for 5 years followed by a 2-year regimen with adalimumab, which induced remission of the intestinal disease.

The patient previously had taken a variety of topical and oral antimicrobials based on treatment from a primary care physician because fungal, bacterial, and viral infections initially were suspected; however, the vulvar disease persisted, and drug-induced immunosuppression was considered to be an underlying factor. Thus, adalimumab was discontinued. Despite elimination of the biologic, the vulvar disease progressed, which prompted referral to the dermatology clinic.

Physical examination revealed diffuse vulvar edema with overlying erythema and scale (Figure 1A). Upon closer inspection, scattered violaceous papules atop a backdrop of lichenification were evident, imparting a cobblestone appearance (Figure 1B). Additionally, a fissure was present on the gluteal cleft. Biopsy from the left labia majora demonstrated well-formed granulomas within a fibrotic reticular dermis (Figures 2A and 2B). The granulomas consisted of both mononucleated and multinucleated histiocytes, rimmed peripherally by lymphocytes and plasma cells (Figure 2C). Periodic acid–Schiff–diastase and acid-fast bacilli stains as well as polarizing microscopy were negative.

Comment

Crohn disease is an idiopathic chronic inflammatory condition that primarily affects the gastrointestinal tract, anywhere from the mouth to the anus. It is characterized by transmural inflammation and fissures that can extend beyond the muscularis propria.^4,6 Extraintestinal manifestations are common.³

Cutaneous CD often presents as perianal, perifistular, or peristomal inflammation or ulceration.⁷ Other skin manifestations include pyoderma gangrenosum, erythema nodosum, erythema multiforme, epidermolysis bullosa acquisita, and palmar erythema.⁷ Metastatic CD involves skin noncontiguous with the gastrointestinal tract^1-20 and may involve any portion of the cutis. Although rare, MCD is the typical etiology underlying vulvar CD.⁸

Approximately 20% of MCD patients have cutaneous lesions without a history of gastrointestinal disease. More than half of cases in adults and approximately two-thirds in children involve the genitalia. Although more common in adults, vulvar involvement has been reported in children as young as 6 years of age.² Diagnosis is especially challenging when bowel symptoms are absent; those patients should be evaluated and followed for subsequent intestinal involvement.⁶

Clinically, symptoms may include general discomfort, pain, pruritus, and dyspareunia. Psychosocial and sexual dysfunction are prevalent and also should be addressed.⁹ Depending on the stage of the disease, physical examination may reveal erythema, edema, papules, pustules, nodules, condylomatous lesions, abscesses, fissures, fistulas, ulceration, acrochordons, and scarring.^2-6,10,11

A host of infections (ie, mycobacterial, actinomycosis, deep fungal, sexually transmitted, schistosomiasis), inflammatory conditions (ie, sarcoid, hidradenitis suppurativa), foreign body reactions, Melkersson-Rosenthal syndrome, and sexual abuse should be included in the differential diagnosis.^2,6,10-12 Once infection, sarcoid, and foreign body reaction have been ruled out, noncaseating granulomas in skin are highly suggestive of CD.⁷

Histopathologic findings of MCD reveal myriad morphological reaction patterns,^5,13 including high-grade dysplasia and carcinoma of the vulva; therefore, it may be imprudent to withhold diagnosis based on the absence of the historically pathognomonic noncaseating granulomas.⁵

The etiopathogenesis of MCD remains an enigma. Dermatopathologic examinations consistently reveal a vascular injury syndrome,¹³ implicating a possible circulatory system contribution via deposition of immune complexes or antigens in skin.⁷ Bacterial infection has been implicated in the intestinal manifestations of CD; however, failure to detect microbial ribosomal RNA in MCD biopsies refutes theories of hematogenous spread of microbes.¹³ Another plausible explanation is that antibodies are formed to conserved microbial epitopes following loss of tolerance to gut flora, which results in an excessive immunologic response at distinct sites in susceptible individuals.¹³ A T-lymphocyte–mediated type IV hypersensitivity reaction also has been proposed via cross-reactivity of lymphocytes, with skin antigens precipitating extraintestinal granuloma formation and vascular injury.³ Clearly, further investigation is needed.

Magnetic resonanance imaging can identify the extent and anatomy of intestinal and pelvic disease and can assist in the diagnosis of vulvar CD.^10,11,14 For these reasons, some experts propose that imaging should be instituted prior to therapy,^12,15,16 especially when direct extension is suspected.¹⁷

Treatment is challenging and often involves collaboration among several specialties.¹² Many treatment options exist because therapeutic responses vary and genital MCD is frequently recalcitrant to therapy.⁴ Medical therapy includes antibiotics such as metronidazole, corticosteroids (ie, topical, intralesional, systemic), and immune modulators (eg, azathioprine, 6-mercaptopurine, cyclosporine, methotrexate, mycophenolate mofetil, tumor necrosis factor α inhibitors).^{2,3,6,10,16,18} Thalidomide has been used for refractory cases.¹⁹ These treatments can be used alone or in combination. Patients should be monitored for side effects and informed that many treatment regimens may be required before a sustained response is achieved.^4,16,18 Surgery is reserved for the most resistant cases. Extensive radical excision of the involved area is the best approach, as limited local excision often is followed by recurrence.²⁰

Conclusion

Our case highlights that vulvar CD can develop in the setting of well-controlled intestinal disease. Vulvoperineal CD should be considered in the differential diagnosis of chronic vulvar pain, swelling, and pruritus, especially in cases resistant to standard therapies and regardless of whether or not gastrointestinal tract symptoms are present. Physicians must be cognizant that vulvar signs and symptoms may precede, coincide with, or follow the diagnosis of intestinal CD. Increased awareness of this entity may facilitate its early recognition and prompt more timely treatment among women with vulvar disease caused by MCD.

The cutaneous manifestations of Crohn disease (CD) are varied, including pyoderma gangrenosum, erythema nodosum, and metastatic CD (MCD). First described by Parks et al,¹ MCD is defined as the occurrence of granulomatous lesions at a skin site distant from the gastrointestinal tract.^1-20 Metastatic CD presents a diagnostic challenge because it is a rare component in the spectrum of inflammatory bowel disease complications, and many physicians are unaware of its existence. It may precede, coincide with, or develop after the diagnosis of intestinal disease.^2-5 Vulvoperineal involvement is particularly problematic because a multitude of other, more likely disease processes are considered first. Typically it is initially diagnosed as a presumed infection prompting reflexive treatment with antivirals, antifungals, and antibiotics. Patients may experience symptoms for years prior to correct diagnosis and institution of proper therapy. A variety of clinical presentations have been described, including nonspecific pain and swelling, erythematous papules and plaques, and nonhealing ulcers. Skin biopsy characteristically confirms the diagnosis and reveals dermal noncaseating granulomas. Multiple oral and parenteral therapies are available, with surgical intervention reserved for resistant cases. We present a case of vulvovaginal MCD in the setting of well-controlled intestinal disease. We also provide a review of the literature regarding genital CD and emphasize the need to keep MCD in the differential of vulvoperineal pathology.

Case Report

A 29-year-old woman was referred to the dermatology clinic with vulvar pain, swelling, and pruritus of 14 months’ duration. Her medical history was remarkable for CD following a colectomy with colostomy. Prior therapies included methotrexate with infliximab for 5 years followed by a 2-year regimen with adalimumab, which induced remission of the intestinal disease.

The patient previously had taken a variety of topical and oral antimicrobials based on treatment from a primary care physician because fungal, bacterial, and viral infections initially were suspected; however, the vulvar disease persisted, and drug-induced immunosuppression was considered to be an underlying factor. Thus, adalimumab was discontinued. Despite elimination of the biologic, the vulvar disease progressed, which prompted referral to the dermatology clinic.

Physical examination revealed diffuse vulvar edema with overlying erythema and scale (Figure 1A). Upon closer inspection, scattered violaceous papules atop a backdrop of lichenification were evident, imparting a cobblestone appearance (Figure 1B). Additionally, a fissure was present on the gluteal cleft. Biopsy from the left labia majora demonstrated well-formed granulomas within a fibrotic reticular dermis (Figures 2A and 2B). The granulomas consisted of both mononucleated and multinucleated histiocytes, rimmed peripherally by lymphocytes and plasma cells (Figure 2C). Periodic acid–Schiff–diastase and acid-fast bacilli stains as well as polarizing microscopy were negative.

Comment

Crohn disease is an idiopathic chronic inflammatory condition that primarily affects the gastrointestinal tract, anywhere from the mouth to the anus. It is characterized by transmural inflammation and fissures that can extend beyond the muscularis propria.^4,6 Extraintestinal manifestations are common.³

Cutaneous CD often presents as perianal, perifistular, or peristomal inflammation or ulceration.⁷ Other skin manifestations include pyoderma gangrenosum, erythema nodosum, erythema multiforme, epidermolysis bullosa acquisita, and palmar erythema.⁷ Metastatic CD involves skin noncontiguous with the gastrointestinal tract^1-20 and may involve any portion of the cutis. Although rare, MCD is the typical etiology underlying vulvar CD.⁸

Approximately 20% of MCD patients have cutaneous lesions without a history of gastrointestinal disease. More than half of cases in adults and approximately two-thirds in children involve the genitalia. Although more common in adults, vulvar involvement has been reported in children as young as 6 years of age.² Diagnosis is especially challenging when bowel symptoms are absent; those patients should be evaluated and followed for subsequent intestinal involvement.⁶

Clinically, symptoms may include general discomfort, pain, pruritus, and dyspareunia. Psychosocial and sexual dysfunction are prevalent and also should be addressed.⁹ Depending on the stage of the disease, physical examination may reveal erythema, edema, papules, pustules, nodules, condylomatous lesions, abscesses, fissures, fistulas, ulceration, acrochordons, and scarring.^2-6,10,11

A host of infections (ie, mycobacterial, actinomycosis, deep fungal, sexually transmitted, schistosomiasis), inflammatory conditions (ie, sarcoid, hidradenitis suppurativa), foreign body reactions, Melkersson-Rosenthal syndrome, and sexual abuse should be included in the differential diagnosis.^2,6,10-12 Once infection, sarcoid, and foreign body reaction have been ruled out, noncaseating granulomas in skin are highly suggestive of CD.⁷

Histopathologic findings of MCD reveal myriad morphological reaction patterns,^5,13 including high-grade dysplasia and carcinoma of the vulva; therefore, it may be imprudent to withhold diagnosis based on the absence of the historically pathognomonic noncaseating granulomas.⁵

The etiopathogenesis of MCD remains an enigma. Dermatopathologic examinations consistently reveal a vascular injury syndrome,¹³ implicating a possible circulatory system contribution via deposition of immune complexes or antigens in skin.⁷ Bacterial infection has been implicated in the intestinal manifestations of CD; however, failure to detect microbial ribosomal RNA in MCD biopsies refutes theories of hematogenous spread of microbes.¹³ Another plausible explanation is that antibodies are formed to conserved microbial epitopes following loss of tolerance to gut flora, which results in an excessive immunologic response at distinct sites in susceptible individuals.¹³ A T-lymphocyte–mediated type IV hypersensitivity reaction also has been proposed via cross-reactivity of lymphocytes, with skin antigens precipitating extraintestinal granuloma formation and vascular injury.³ Clearly, further investigation is needed.

Magnetic resonanance imaging can identify the extent and anatomy of intestinal and pelvic disease and can assist in the diagnosis of vulvar CD.^10,11,14 For these reasons, some experts propose that imaging should be instituted prior to therapy,^12,15,16 especially when direct extension is suspected.¹⁷

Treatment is challenging and often involves collaboration among several specialties.¹² Many treatment options exist because therapeutic responses vary and genital MCD is frequently recalcitrant to therapy.⁴ Medical therapy includes antibiotics such as metronidazole, corticosteroids (ie, topical, intralesional, systemic), and immune modulators (eg, azathioprine, 6-mercaptopurine, cyclosporine, methotrexate, mycophenolate mofetil, tumor necrosis factor α inhibitors).^{2,3,6,10,16,18} Thalidomide has been used for refractory cases.¹⁹ These treatments can be used alone or in combination. Patients should be monitored for side effects and informed that many treatment regimens may be required before a sustained response is achieved.^4,16,18 Surgery is reserved for the most resistant cases. Extensive radical excision of the involved area is the best approach, as limited local excision often is followed by recurrence.²⁰

Conclusion

Our case highlights that vulvar CD can develop in the setting of well-controlled intestinal disease. Vulvoperineal CD should be considered in the differential diagnosis of chronic vulvar pain, swelling, and pruritus, especially in cases resistant to standard therapies and regardless of whether or not gastrointestinal tract symptoms are present. Physicians must be cognizant that vulvar signs and symptoms may precede, coincide with, or follow the diagnosis of intestinal CD. Increased awareness of this entity may facilitate its early recognition and prompt more timely treatment among women with vulvar disease caused by MCD.

To the Editor:

Microcystic adnexal carcinoma (MAC), described by Goldstein et al¹ in 1982, is a relatively uncommon cutaneous neoplasm. This locally aggressive malignant adnexal tumor has high potential for local recurrence. The skin of the head, particularly in the nasolabial and periorbital regions, most often is involved.² Involvement of the external auditory canal (EAC) is relatively rare. We report a case of MAC of the EAC.

A 52-year-old man presented with 1 palpable nodule on the right EAC of approximately 1 year’s duration. The lesion was asymptomatic, and the patient had no history of radiation exposure. The patient was an airport employee required to wear an earplug in the right ear. Endoscopic examination identified a 1×1 cm² erythematous nodule on the anterior inferior quadrant of the right external ear canal orifice (Figure 1). Axial and coronal computed tomography demonstrated a soft tissue mass in the right EAC without any bony erosion. No clinical signs of regional lymphadenopathy or distant metastasis were present. Excision was performed under microscopic visualization.

Histopathology of the nodule showed marked proliferation of multiple keratin-containing cysts, irregular ductal structures, and solid epithelial nests in the deep dermis (Figure 2). Irregular ductal structures with 2 cell layer walls and several epithelial strands or small nests of tumor cells within desmoplastic stroma were noted (Figure 3). No perineural infiltration or tumor infiltration existed at the margin. Based on the clinical and histopathologic findings, the final diagnosis was MAC. Complete resolution was noted after the excision. The patient returned for regular follow-up and no signs of recurrence were noted for 7 years postoperatively.

Microcystic adnexal carcinoma, also known as sclerosing sweat duct (syringomatous) carcinoma, malignant syringoma, and syringoid eccrine carcinoma, is characterized by slow and locally aggressive growth with high likelihood of perineural invasion and frequent recurrence.² Regional lymph node metastasis is uncommon, and systemic metastasis is rare.^2-4

Although the head most often is involved, a PubMed search of articles indexed for MEDLINE using the terms microcystic adnexal carcinoma and external auditory canal revealed 4 cases (Table).^5-8 Our report adds another case of MAC arising solely in the EAC. Although the etiology of MAC is unknown, prior studies indicated that radiotherapy is a risk factor for MAC. Other possible risk factors include UV light exposure and immunodeficiency.² Our patient had no history of these factors and experienced chronic friction caused by use of an occupational unilateral earplug, which may be a notable factor. Locations of MAC arising outside the head region include the axilla, vulva, breast, palm, toe, perianal skin, buttock, chest, and an ovarian cystic teratoma.^3,9 Friction commonly occurs in many of these areas. Therefore, we propose that friction may be a risk factor for MAC.

Microcystic adnexal carcinoma should be included in the differential diagnosis of any slowly growing cutaneous tumor, even in the EAC. Once diagnosed, the tumor should be surgically excised. Because local recurrence is common and may occur several decades after excision, lifetime follow-up for recurrence signs is essential.

To the Editor:

Microcystic adnexal carcinoma (MAC), described by Goldstein et al¹ in 1982, is a relatively uncommon cutaneous neoplasm. This locally aggressive malignant adnexal tumor has high potential for local recurrence. The skin of the head, particularly in the nasolabial and periorbital regions, most often is involved.² Involvement of the external auditory canal (EAC) is relatively rare. We report a case of MAC of the EAC.

A 52-year-old man presented with 1 palpable nodule on the right EAC of approximately 1 year’s duration. The lesion was asymptomatic, and the patient had no history of radiation exposure. The patient was an airport employee required to wear an earplug in the right ear. Endoscopic examination identified a 1×1 cm² erythematous nodule on the anterior inferior quadrant of the right external ear canal orifice (Figure 1). Axial and coronal computed tomography demonstrated a soft tissue mass in the right EAC without any bony erosion. No clinical signs of regional lymphadenopathy or distant metastasis were present. Excision was performed under microscopic visualization.

Histopathology of the nodule showed marked proliferation of multiple keratin-containing cysts, irregular ductal structures, and solid epithelial nests in the deep dermis (Figure 2). Irregular ductal structures with 2 cell layer walls and several epithelial strands or small nests of tumor cells within desmoplastic stroma were noted (Figure 3). No perineural infiltration or tumor infiltration existed at the margin. Based on the clinical and histopathologic findings, the final diagnosis was MAC. Complete resolution was noted after the excision. The patient returned for regular follow-up and no signs of recurrence were noted for 7 years postoperatively.

Microcystic adnexal carcinoma, also known as sclerosing sweat duct (syringomatous) carcinoma, malignant syringoma, and syringoid eccrine carcinoma, is characterized by slow and locally aggressive growth with high likelihood of perineural invasion and frequent recurrence.² Regional lymph node metastasis is uncommon, and systemic metastasis is rare.^2-4

Although the head most often is involved, a PubMed search of articles indexed for MEDLINE using the terms microcystic adnexal carcinoma and external auditory canal revealed 4 cases (Table).^5-8 Our report adds another case of MAC arising solely in the EAC. Although the etiology of MAC is unknown, prior studies indicated that radiotherapy is a risk factor for MAC. Other possible risk factors include UV light exposure and immunodeficiency.² Our patient had no history of these factors and experienced chronic friction caused by use of an occupational unilateral earplug, which may be a notable factor. Locations of MAC arising outside the head region include the axilla, vulva, breast, palm, toe, perianal skin, buttock, chest, and an ovarian cystic teratoma.^3,9 Friction commonly occurs in many of these areas. Therefore, we propose that friction may be a risk factor for MAC.

Microcystic adnexal carcinoma should be included in the differential diagnosis of any slowly growing cutaneous tumor, even in the EAC. Once diagnosed, the tumor should be surgically excised. Because local recurrence is common and may occur several decades after excision, lifetime follow-up for recurrence signs is essential.

To the Editor:

Microcystic adnexal carcinoma (MAC), described by Goldstein et al¹ in 1982, is a relatively uncommon cutaneous neoplasm. This locally aggressive malignant adnexal tumor has high potential for local recurrence. The skin of the head, particularly in the nasolabial and periorbital regions, most often is involved.² Involvement of the external auditory canal (EAC) is relatively rare. We report a case of MAC of the EAC.

A 52-year-old man presented with 1 palpable nodule on the right EAC of approximately 1 year’s duration. The lesion was asymptomatic, and the patient had no history of radiation exposure. The patient was an airport employee required to wear an earplug in the right ear. Endoscopic examination identified a 1×1 cm² erythematous nodule on the anterior inferior quadrant of the right external ear canal orifice (Figure 1). Axial and coronal computed tomography demonstrated a soft tissue mass in the right EAC without any bony erosion. No clinical signs of regional lymphadenopathy or distant metastasis were present. Excision was performed under microscopic visualization.

Histopathology of the nodule showed marked proliferation of multiple keratin-containing cysts, irregular ductal structures, and solid epithelial nests in the deep dermis (Figure 2). Irregular ductal structures with 2 cell layer walls and several epithelial strands or small nests of tumor cells within desmoplastic stroma were noted (Figure 3). No perineural infiltration or tumor infiltration existed at the margin. Based on the clinical and histopathologic findings, the final diagnosis was MAC. Complete resolution was noted after the excision. The patient returned for regular follow-up and no signs of recurrence were noted for 7 years postoperatively.

Microcystic adnexal carcinoma, also known as sclerosing sweat duct (syringomatous) carcinoma, malignant syringoma, and syringoid eccrine carcinoma, is characterized by slow and locally aggressive growth with high likelihood of perineural invasion and frequent recurrence.² Regional lymph node metastasis is uncommon, and systemic metastasis is rare.^2-4

Although the head most often is involved, a PubMed search of articles indexed for MEDLINE using the terms microcystic adnexal carcinoma and external auditory canal revealed 4 cases (Table).^5-8 Our report adds another case of MAC arising solely in the EAC. Although the etiology of MAC is unknown, prior studies indicated that radiotherapy is a risk factor for MAC. Other possible risk factors include UV light exposure and immunodeficiency.² Our patient had no history of these factors and experienced chronic friction caused by use of an occupational unilateral earplug, which may be a notable factor. Locations of MAC arising outside the head region include the axilla, vulva, breast, palm, toe, perianal skin, buttock, chest, and an ovarian cystic teratoma.^3,9 Friction commonly occurs in many of these areas. Therefore, we propose that friction may be a risk factor for MAC.

Microcystic adnexal carcinoma should be included in the differential diagnosis of any slowly growing cutaneous tumor, even in the EAC. Once diagnosed, the tumor should be surgically excised. Because local recurrence is common and may occur several decades after excision, lifetime follow-up for recurrence signs is essential.

The Diagnosis: Unilateral Nevoid Telangiectasia

Unilateral nevoid telangiectasia (UNT) is an uncommon, or perhaps underreported, cutaneous condition involving telangiectatic patches in a unilateral dermatomal or blaschkoid pattern.¹ The condition has been described as either congenital or acquired. Congenital UNT is thought to be a result of somatic mosaicism, whereby a mutation during embryogenesis leads to a distinct population of cells expressing the vascular malformation.¹ Congenital UNT has been associated with Becker nevus, which also is thought to be a result of somatic mosaicism, further providing evidence for this theory, though it is unclear whether this finding is incidental.² The acquired form often is associated with fluctuation of hormones, such as in pregnancy or with oral contraceptive initiation, as well as with hepatic disease as seen in our patient. However, there are many cases of acquired UNT with no implicated underlying disease, alcohol abuse, or hormonal changes, which calls into question if UNT is definitively an estrogen-related condition.³ One study demonstrated an increased level of estrogen and progesterone receptors in affected skin, which may have led to expression of the cutaneous changes at that site.⁴ More research is needed to elucidate this point, as other studies have not reproduced similar findings.

Congenital UNT occurs more commonly in males, whereas the acquired variant is seen more frequently in females. The third and fourth cervical dermatomes most often are involved.⁵ Most lesions persist without spontaneous resolution. Treatment options are limited and include pulsed dye laser treatment and makeup application to cover the telangiectatic patches. The main side effect seen with pulsed dye laser treatment is reversible pigmentary changes, with 1 report of textural skin change.⁶

A biopsy was deemed unnecessary for the clinical diagnosis in our patient because there was a clear explanation for the physical examination findings due to long-standing underlying liver disease. When biopsied, UNT characteristically demonstrates dilated dermal capillaries.⁵ Our patient elected not to pursue laser therapy but expressed interest in using makeup to camouflage the lesion.

The differential diagnosis includes acquired nevus flammeus, which typically is present on the face and often appears following mechanical or thermal trauma. Angioma serpiginosum most often occurs on the buttocks and legs as small red papules or puncta coalescing into a serpiginous linear arrangement. It often appears in childhood. Angiosarcoma is an aggressive malignancy that often occurs on the head and neck in elderly patients. It is associated with areas of long-standing lymphedema and often appears as a bruiselike lesion. Rosacea typically is not fixed in its clinical appearance and presents as transitory flushing of the head and neck with or without a history of acneform eruptions on the face. It typically is not unilateral.

The Diagnosis: Unilateral Nevoid Telangiectasia

Unilateral nevoid telangiectasia (UNT) is an uncommon, or perhaps underreported, cutaneous condition involving telangiectatic patches in a unilateral dermatomal or blaschkoid pattern.¹ The condition has been described as either congenital or acquired. Congenital UNT is thought to be a result of somatic mosaicism, whereby a mutation during embryogenesis leads to a distinct population of cells expressing the vascular malformation.¹ Congenital UNT has been associated with Becker nevus, which also is thought to be a result of somatic mosaicism, further providing evidence for this theory, though it is unclear whether this finding is incidental.² The acquired form often is associated with fluctuation of hormones, such as in pregnancy or with oral contraceptive initiation, as well as with hepatic disease as seen in our patient. However, there are many cases of acquired UNT with no implicated underlying disease, alcohol abuse, or hormonal changes, which calls into question if UNT is definitively an estrogen-related condition.³ One study demonstrated an increased level of estrogen and progesterone receptors in affected skin, which may have led to expression of the cutaneous changes at that site.⁴ More research is needed to elucidate this point, as other studies have not reproduced similar findings.

Congenital UNT occurs more commonly in males, whereas the acquired variant is seen more frequently in females. The third and fourth cervical dermatomes most often are involved.⁵ Most lesions persist without spontaneous resolution. Treatment options are limited and include pulsed dye laser treatment and makeup application to cover the telangiectatic patches. The main side effect seen with pulsed dye laser treatment is reversible pigmentary changes, with 1 report of textural skin change.⁶

A biopsy was deemed unnecessary for the clinical diagnosis in our patient because there was a clear explanation for the physical examination findings due to long-standing underlying liver disease. When biopsied, UNT characteristically demonstrates dilated dermal capillaries.⁵ Our patient elected not to pursue laser therapy but expressed interest in using makeup to camouflage the lesion.

The differential diagnosis includes acquired nevus flammeus, which typically is present on the face and often appears following mechanical or thermal trauma. Angioma serpiginosum most often occurs on the buttocks and legs as small red papules or puncta coalescing into a serpiginous linear arrangement. It often appears in childhood. Angiosarcoma is an aggressive malignancy that often occurs on the head and neck in elderly patients. It is associated with areas of long-standing lymphedema and often appears as a bruiselike lesion. Rosacea typically is not fixed in its clinical appearance and presents as transitory flushing of the head and neck with or without a history of acneform eruptions on the face. It typically is not unilateral.

The Diagnosis: Unilateral Nevoid Telangiectasia

Unilateral nevoid telangiectasia (UNT) is an uncommon, or perhaps underreported, cutaneous condition involving telangiectatic patches in a unilateral dermatomal or blaschkoid pattern.¹ The condition has been described as either congenital or acquired. Congenital UNT is thought to be a result of somatic mosaicism, whereby a mutation during embryogenesis leads to a distinct population of cells expressing the vascular malformation.¹ Congenital UNT has been associated with Becker nevus, which also is thought to be a result of somatic mosaicism, further providing evidence for this theory, though it is unclear whether this finding is incidental.² The acquired form often is associated with fluctuation of hormones, such as in pregnancy or with oral contraceptive initiation, as well as with hepatic disease as seen in our patient. However, there are many cases of acquired UNT with no implicated underlying disease, alcohol abuse, or hormonal changes, which calls into question if UNT is definitively an estrogen-related condition.³ One study demonstrated an increased level of estrogen and progesterone receptors in affected skin, which may have led to expression of the cutaneous changes at that site.⁴ More research is needed to elucidate this point, as other studies have not reproduced similar findings.

Congenital UNT occurs more commonly in males, whereas the acquired variant is seen more frequently in females. The third and fourth cervical dermatomes most often are involved.⁵ Most lesions persist without spontaneous resolution. Treatment options are limited and include pulsed dye laser treatment and makeup application to cover the telangiectatic patches. The main side effect seen with pulsed dye laser treatment is reversible pigmentary changes, with 1 report of textural skin change.⁶

A biopsy was deemed unnecessary for the clinical diagnosis in our patient because there was a clear explanation for the physical examination findings due to long-standing underlying liver disease. When biopsied, UNT characteristically demonstrates dilated dermal capillaries.⁵ Our patient elected not to pursue laser therapy but expressed interest in using makeup to camouflage the lesion.

The differential diagnosis includes acquired nevus flammeus, which typically is present on the face and often appears following mechanical or thermal trauma. Angioma serpiginosum most often occurs on the buttocks and legs as small red papules or puncta coalescing into a serpiginous linear arrangement. It often appears in childhood. Angiosarcoma is an aggressive malignancy that often occurs on the head and neck in elderly patients. It is associated with areas of long-standing lymphedema and often appears as a bruiselike lesion. Rosacea typically is not fixed in its clinical appearance and presents as transitory flushing of the head and neck with or without a history of acneform eruptions on the face. It typically is not unilateral.

The Diagnosis: Radiation Mucositis

The patient was undergoing active radiation therapy for squamous cell carcinoma of the tongue, and according to the oncology team, the findings were in the precise location of radiation exposure. Radiation mucositis is a major and limiting side effect of radiation therapy for head and neck mucosal cancers, and symptom management is critical to ensure completion of the full radiation dose. Although infectious etiologies must be considered, the patient was already on prophylactic antiviral and antibacterial therapies. Moreover, the focal involvement with sparing of more mucosal tissue is atypical for most infections. Fixed drug reactions can present with localized mucosal and nonmucosal inflammation leading to erosion or ulceration. In this case, the only potential culprit was levofloxacin; however, it was initiated 2 days prior, and the patient never had reactions to this medication in the past.

Acute radiation mucositis is a transient but major limiting side effect of radiation therapy. The associated odynophagia, secondary infection, and reduced oral intake often can lead to diminished disease control secondary to treatment interruption and subsequent development of resistant tumor burden. Concurrent chemotherapy and alternated fractionation radiation therapy increase the incidence of mucositis. Trotti et al¹ (n=6181) reported that severe mucositis (grades 3 to 4) was found in 56% of patients receiving altered fractionation radiation therapy compared to 34% of patients who received conventional radiation therapy. Other risk factors related to the development of acute radiation mucositis include associated chemotherapy, age (>65 years), poor oral hygiene, diabetes mellitus, and prior periodontal disease.² 

Radiation causes direct cellular damage to keratinocytes, leading to ulceration and erythema, as well as keratinocyte stem cells, which interferes with the healing process. Typical symptoms of mucosal radiation injury may include erythema (asymptomatic or causing intolerance of warm foods) that develops at the end of the second week of radiation therapy, focal areas of desquamation that develops in week 3, and confluent mucositis that can further progress to ulceration and necrosis in weeks 4 to 5.² The development of dysgeusia, which is estimated to occur in 67% of patients receiving radiotherapy and 76% of patients receiving combination therapy, also can contribute to nutritional difficulties and weight loss.³

Avoiding overtreatment by constraining radiation volume and limiting concurrent chemotherapy are important preventative measures. The mainstay for managing mucositis includes symptomatic relief with oral hygiene, topical agents, topical plus systemic analgesia, dietary changes, and treatment of associated infections. Benzydamine, a nonsteroidal anti-inflammatory drug, is not available in the United States but has been shown to effectively improve symptoms.⁴ Various formulations of topical anesthetics consisting of diphenhydramine with or without corticosteroids, antibiotics, and antifungals help alleviate symptoms of mucositis; however, no single formulation has been studied. Low-level laser therapy also has shown efficacy in managing symptoms of mucositis.^5,6 For persistent odynophagia, systemic opioid therapy should be attempted to achieve uninterrupted radiation therapy. Severe mucositis requires balancing risks and benefits of interrupting treatment, as additional damage may cause permanent mucosal injury.

Our patient had adequate symptom control with benzocaine lozenges and a combination mouthwash containing diphenhydramine, nystatin, lidocaine, hydrocortisone, and tetracycline. He required only occasional doses of systemic oxycodone. After a 1-week hospital admission for treatment of the pneumonia, he resumed radiation therapy and completed a full 8-week radiation course.

The Diagnosis: Radiation Mucositis

The patient was undergoing active radiation therapy for squamous cell carcinoma of the tongue, and according to the oncology team, the findings were in the precise location of radiation exposure. Radiation mucositis is a major and limiting side effect of radiation therapy for head and neck mucosal cancers, and symptom management is critical to ensure completion of the full radiation dose. Although infectious etiologies must be considered, the patient was already on prophylactic antiviral and antibacterial therapies. Moreover, the focal involvement with sparing of more mucosal tissue is atypical for most infections. Fixed drug reactions can present with localized mucosal and nonmucosal inflammation leading to erosion or ulceration. In this case, the only potential culprit was levofloxacin; however, it was initiated 2 days prior, and the patient never had reactions to this medication in the past.

Acute radiation mucositis is a transient but major limiting side effect of radiation therapy. The associated odynophagia, secondary infection, and reduced oral intake often can lead to diminished disease control secondary to treatment interruption and subsequent development of resistant tumor burden. Concurrent chemotherapy and alternated fractionation radiation therapy increase the incidence of mucositis. Trotti et al¹ (n=6181) reported that severe mucositis (grades 3 to 4) was found in 56% of patients receiving altered fractionation radiation therapy compared to 34% of patients who received conventional radiation therapy. Other risk factors related to the development of acute radiation mucositis include associated chemotherapy, age (>65 years), poor oral hygiene, diabetes mellitus, and prior periodontal disease.² 

Radiation causes direct cellular damage to keratinocytes, leading to ulceration and erythema, as well as keratinocyte stem cells, which interferes with the healing process. Typical symptoms of mucosal radiation injury may include erythema (asymptomatic or causing intolerance of warm foods) that develops at the end of the second week of radiation therapy, focal areas of desquamation that develops in week 3, and confluent mucositis that can further progress to ulceration and necrosis in weeks 4 to 5.² The development of dysgeusia, which is estimated to occur in 67% of patients receiving radiotherapy and 76% of patients receiving combination therapy, also can contribute to nutritional difficulties and weight loss.³

Avoiding overtreatment by constraining radiation volume and limiting concurrent chemotherapy are important preventative measures. The mainstay for managing mucositis includes symptomatic relief with oral hygiene, topical agents, topical plus systemic analgesia, dietary changes, and treatment of associated infections. Benzydamine, a nonsteroidal anti-inflammatory drug, is not available in the United States but has been shown to effectively improve symptoms.⁴ Various formulations of topical anesthetics consisting of diphenhydramine with or without corticosteroids, antibiotics, and antifungals help alleviate symptoms of mucositis; however, no single formulation has been studied. Low-level laser therapy also has shown efficacy in managing symptoms of mucositis.^5,6 For persistent odynophagia, systemic opioid therapy should be attempted to achieve uninterrupted radiation therapy. Severe mucositis requires balancing risks and benefits of interrupting treatment, as additional damage may cause permanent mucosal injury.

Our patient had adequate symptom control with benzocaine lozenges and a combination mouthwash containing diphenhydramine, nystatin, lidocaine, hydrocortisone, and tetracycline. He required only occasional doses of systemic oxycodone. After a 1-week hospital admission for treatment of the pneumonia, he resumed radiation therapy and completed a full 8-week radiation course.

The Diagnosis: Radiation Mucositis

The patient was undergoing active radiation therapy for squamous cell carcinoma of the tongue, and according to the oncology team, the findings were in the precise location of radiation exposure. Radiation mucositis is a major and limiting side effect of radiation therapy for head and neck mucosal cancers, and symptom management is critical to ensure completion of the full radiation dose. Although infectious etiologies must be considered, the patient was already on prophylactic antiviral and antibacterial therapies. Moreover, the focal involvement with sparing of more mucosal tissue is atypical for most infections. Fixed drug reactions can present with localized mucosal and nonmucosal inflammation leading to erosion or ulceration. In this case, the only potential culprit was levofloxacin; however, it was initiated 2 days prior, and the patient never had reactions to this medication in the past.

Acute radiation mucositis is a transient but major limiting side effect of radiation therapy. The associated odynophagia, secondary infection, and reduced oral intake often can lead to diminished disease control secondary to treatment interruption and subsequent development of resistant tumor burden. Concurrent chemotherapy and alternated fractionation radiation therapy increase the incidence of mucositis. Trotti et al¹ (n=6181) reported that severe mucositis (grades 3 to 4) was found in 56% of patients receiving altered fractionation radiation therapy compared to 34% of patients who received conventional radiation therapy. Other risk factors related to the development of acute radiation mucositis include associated chemotherapy, age (>65 years), poor oral hygiene, diabetes mellitus, and prior periodontal disease.² 

Radiation causes direct cellular damage to keratinocytes, leading to ulceration and erythema, as well as keratinocyte stem cells, which interferes with the healing process. Typical symptoms of mucosal radiation injury may include erythema (asymptomatic or causing intolerance of warm foods) that develops at the end of the second week of radiation therapy, focal areas of desquamation that develops in week 3, and confluent mucositis that can further progress to ulceration and necrosis in weeks 4 to 5.² The development of dysgeusia, which is estimated to occur in 67% of patients receiving radiotherapy and 76% of patients receiving combination therapy, also can contribute to nutritional difficulties and weight loss.³

Avoiding overtreatment by constraining radiation volume and limiting concurrent chemotherapy are important preventative measures. The mainstay for managing mucositis includes symptomatic relief with oral hygiene, topical agents, topical plus systemic analgesia, dietary changes, and treatment of associated infections. Benzydamine, a nonsteroidal anti-inflammatory drug, is not available in the United States but has been shown to effectively improve symptoms.⁴ Various formulations of topical anesthetics consisting of diphenhydramine with or without corticosteroids, antibiotics, and antifungals help alleviate symptoms of mucositis; however, no single formulation has been studied. Low-level laser therapy also has shown efficacy in managing symptoms of mucositis.^5,6 For persistent odynophagia, systemic opioid therapy should be attempted to achieve uninterrupted radiation therapy. Severe mucositis requires balancing risks and benefits of interrupting treatment, as additional damage may cause permanent mucosal injury.

Our patient had adequate symptom control with benzocaine lozenges and a combination mouthwash containing diphenhydramine, nystatin, lidocaine, hydrocortisone, and tetracycline. He required only occasional doses of systemic oxycodone. After a 1-week hospital admission for treatment of the pneumonia, he resumed radiation therapy and completed a full 8-week radiation course.

To the Editor:

Morphea, atrophoderma, guttate lichen sclerosus et atrophicus (LS&A), anetoderma, and their subtypes are inflammatory processes ultimately leading to dermal remodeling. We report a case of a scaly, hypopigmented, macular rash that clinically appeared as an entity along the morphea-atrophoderma spectrum and demonstrated unique histopathologic changes in both collagen and elastin confined to the upper reticular and papillary dermis. This case is a potentially rare variant representing a combination of clinical and microscopic findings.

A 29-year-old woman presented for an increasing number of white spots distributed on the trunk, arms, and legs. She denied local and systemic symptoms. The patient reported that she was stung by 100 wasps 23 years prior. Following the assault, her grandmother placed chewed tobacco leaves atop the painful erythematous wheals and flares. Upon resolution, hypopigmented macules and patches remained in their place. The patient denied associated symptoms or new lesions; she did not seek care at that time.

In her early 20s, the patient noted new, similarly distributed hypopigmented macules and patches without associated arthropod assault. She was treated by an outside dermatologist without result for presumed tinea versicolor. A follow-up superficial shave biopsy cited subtle psoriasiform dermatitis. Topical steroids did not improve the lesions. Her medical history also was remarkable for a reportedly unprovoked complete rotator cuff tear.

Physical examination revealed 0.5- to 2.0-cm, ill-defined, perifollicular and nonfollicular, slightly scaly macules and patches on the trunk, arms, and legs. There was no follicular plugging (Figure 1A). The hands, feet, face, and mucosal surfaces were spared. She had no family history of similar lesions. Although atrophic in appearance, a single lesion on the left thigh was palpably depressed (Figure 1B). Serology demonstrated a normal complete blood cell count and comprehensive metabolic panel, and negative Lyme titers. Light therapy and topical steroids failed to improve the lesions; calcipotriene cream 0.005% made the lesions erythematous and pruritic.

A biopsy from a flank lesion demonstrated a normal epithelium without thinning, a normal basal melanocyte population, and minimally effaced rete ridges. Thin collagen bundles were noted in the upper reticular and papillary dermis with associated fibroplasia (Figure 2). Verhoeff-van Gieson stain revealed decreased and fragmented elastin filaments in the same dermal distribution as the changed collagen (Figure 3). There was no evidence of primary inflammatory disease. The dermis was thinned. Periodic acid–Schiff stain confirmed the absence of hyphae and spores.

The relevant findings in our patient including the following: (1) onset of hypopigmented macules and patches following resolution of a toxic insult; (2) initially stable number of lesions that progressed in number but not size; (3) thinned collagen associated with fibroplasia in the upper reticular and papillary dermis; (4) decreased number and fragmentation of elastin filaments confined to the same region; (5) no congenital lesions or similar lesions in family members; and (6) a complete rotator cuff tear with no findings of a systemic connective-tissue disorder such as Ehlers-Danlos syndrome.

We performed a literature search of PubMed articles indexed for MEDLINE using combinations of the terms atrophic, hypopigmented, white, spot disease, confetti-like, guttate, macules, atrophoderma, morphea, anetoderma, elastin, and collagen to identify potentially similar reports of guttate hypopigmented macules demonstrating changes of the collagen and elastin in the papillary and upper reticular dermis. Some variants, namely atrophoderma of Pasini and Pierini (APP), guttate morphea, and superficial morphea, demonstrate similar clinical and histopathologic findings.

Findings similar to our case were documented in case reports of 2 women (aged 34 and 42 years)¹ presenting with asymptomatic, atrophic, well-demarcated, shiny, hypopigmented macules over the trunk and upper extremities, which demonstrated a thinned epidermis with coarse hyalinized collagen bundles in the mid and lower dermis. There was upper and diffuse dermal elastolysis (patient 1 and patient 2, respectively).¹ Our patient’s lesions were hypopigmented and atrophic in appearance but were slightly scaly and also involved the extremities. Distinct from these patient reports, histopathology from our case demonstrated thin packed collagen bundles and decreased fragmented elastin filaments confined to the upper reticular and papillary dermis.

Plaque morphea is the most common type of localized scleroderma.² The subtype APP demonstrates round to ovoid, gray-brown depressions with cliff-drop borders. They may appear flesh colored or hypopigmented.^3,4 These sclerodermoid lesions lack the violaceous border classic to morphea. Sclerosis and induration also are typically absent.⁵ Clinically, our patient’s macules resembled this entity. Histopathologically, APP shows normal epithelium with an increased basal layer pigmentation; preserved adnexal structures; and mid to lower dermal collagen edema, clumping, and homogenization.^3,4 Elastic fibers classically are unchanged, with exceptions.^6-11 Changes in the collagen and elastin of our patient were unlike those reported in APP, which occur in the mid to lower dermis.

Guttate morphea demonstrates small, pale, minimally indurated, coin-shaped lesions on the trunk. Histopathology reveals less sclerosis and more edema, resembling LS&A.¹² The earliest descriptions of this entity describe 3 stages: ivory/chalk white, scaly, and atrophic. Follicular plugging (absent in this patient) and fine scale can exist at any stage.^13,14 Flattened rete ridges mark an otherwise preserved epidermis; hyalinized collagen typically is superficial and demonstrates less sclerosis yet increased edema.^12-14 Fewer elastic fibers typically are present compared to normal skin. Changes seen in this entity are more superficial, as with our patient, than classic scleroderma. However, classic edema was not found in our patient’s biopsy specimen.

Superficial morphea, occurring predominantly in females, presents with hyperpigmented or hypopigmented patches having minimal to no induration. The lesions typically are asymptomatic. Histopathologically, collagen deposition and inflammation are confined to the superficial dermis without homogenization associated with LS&A, findings that were consistent with this patient’s biopsy.^15,16 However, similar to other morpheaform variants, elastic fibers are unchanged.¹⁵ Verhoeff-van Gieson stain of the biopsy (Figure 3) showed the decreased and fragmented elastin network in the upper reticular and papillary dermis, making this entity less compatible.

Guttate LS&A may present with interfollicular, bluish white macules or papules coalescing into patches or plaques. Lesions evolve to reveal atrophic thin skin with follicular plugging. Histology demonstrates a thinned epidermis with orthohypokeratosis marked by flattened rete ridges. The dermis reveals short hyalinized collagen fibrils with a loss of elastic fibers in the papillary and upper reticular dermis, giving a homogenized appearance. Early disease is marked by an inflammatory infiltrate.¹⁷ Most of these findings are consistent with our patient’s pathology, which was confined to the upper dermis. Lacking, however, were characteristic findings of LS&A, including upper dermal homogenization, near-total effacement of rete ridges, orthokeratosis, and vacuolar degeneration at the dermoepidermal junction. As such, this entity is less compatible.

Atrophoderma elastolyticum discretum has clinical features of atrophoderma with elastolytic histopathologic findings.¹ Anetoderma presents with outpouchings of atrophic skin with a surrounding ring of normal tissue. Histopathologically, this entity shows normal collagen with elastolysis; there also is a decrease in desmosine, an elastin cross-linker.^1,3 Neither the clinical nor histopathologic findings in this patient matched these 2 entities.

The reported chronologic association of these lesions with an arthropod assault raised suspicion to their association with toxic insult or postinflammatory changes. One study reported mechanical trauma, including insect bites, as a possible inciting factor of morphea.¹¹ These data, gathered from patient surveys, reported trauma associated to lesion development.^1,17 A review of the literature regarding atrophoderma, morphea, and LS&A failed to identify pathogenic changes seen in this patient following initial trauma. Moreover, although it is difficult to prove causality in the formation of the original hypopigmented spots, the development of identical spots in a similar distribution without further trauma suggests against these etiologies to fully explain her lesions. Nonetheless, circumstance makes it difficult to prove whether the original arthropod insult spurred a smoldering reactive process that caused the newer lesions.

Hereditary connective-tissue disorders also were considered in the differential diagnosis. Because of the patient’s history of an unprovoked complete rotator cuff tear, Ehlers-Danlos syndrome was considered; however, the remainder of her examination was normal, making a syndromic systemic disorder a less likely etiology.Because of the distinct clinical and histopathologic findings, this case may represent a rare and previously unreported variant of morphea. Clinically, these hypopigmented macules and patches exist somewhere along the morphea-atrophoderma spectrum. Histopathologic findings do not conform to prior reports. The name atrophodermalike guttate morphea may be an appropriate appellation. It is possible this presentation represents a variant of what dermatologists have referred to as white spot disease.¹⁸ We hope that this case may bring others to discussion, allowing for the identification of a more precise entity and etiology so that patients may receive more directed therapy.

To the Editor:

Morphea, atrophoderma, guttate lichen sclerosus et atrophicus (LS&A), anetoderma, and their subtypes are inflammatory processes ultimately leading to dermal remodeling. We report a case of a scaly, hypopigmented, macular rash that clinically appeared as an entity along the morphea-atrophoderma spectrum and demonstrated unique histopathologic changes in both collagen and elastin confined to the upper reticular and papillary dermis. This case is a potentially rare variant representing a combination of clinical and microscopic findings.

A 29-year-old woman presented for an increasing number of white spots distributed on the trunk, arms, and legs. She denied local and systemic symptoms. The patient reported that she was stung by 100 wasps 23 years prior. Following the assault, her grandmother placed chewed tobacco leaves atop the painful erythematous wheals and flares. Upon resolution, hypopigmented macules and patches remained in their place. The patient denied associated symptoms or new lesions; she did not seek care at that time.

In her early 20s, the patient noted new, similarly distributed hypopigmented macules and patches without associated arthropod assault. She was treated by an outside dermatologist without result for presumed tinea versicolor. A follow-up superficial shave biopsy cited subtle psoriasiform dermatitis. Topical steroids did not improve the lesions. Her medical history also was remarkable for a reportedly unprovoked complete rotator cuff tear.

Physical examination revealed 0.5- to 2.0-cm, ill-defined, perifollicular and nonfollicular, slightly scaly macules and patches on the trunk, arms, and legs. There was no follicular plugging (Figure 1A). The hands, feet, face, and mucosal surfaces were spared. She had no family history of similar lesions. Although atrophic in appearance, a single lesion on the left thigh was palpably depressed (Figure 1B). Serology demonstrated a normal complete blood cell count and comprehensive metabolic panel, and negative Lyme titers. Light therapy and topical steroids failed to improve the lesions; calcipotriene cream 0.005% made the lesions erythematous and pruritic.

A biopsy from a flank lesion demonstrated a normal epithelium without thinning, a normal basal melanocyte population, and minimally effaced rete ridges. Thin collagen bundles were noted in the upper reticular and papillary dermis with associated fibroplasia (Figure 2). Verhoeff-van Gieson stain revealed decreased and fragmented elastin filaments in the same dermal distribution as the changed collagen (Figure 3). There was no evidence of primary inflammatory disease. The dermis was thinned. Periodic acid–Schiff stain confirmed the absence of hyphae and spores.

The relevant findings in our patient including the following: (1) onset of hypopigmented macules and patches following resolution of a toxic insult; (2) initially stable number of lesions that progressed in number but not size; (3) thinned collagen associated with fibroplasia in the upper reticular and papillary dermis; (4) decreased number and fragmentation of elastin filaments confined to the same region; (5) no congenital lesions or similar lesions in family members; and (6) a complete rotator cuff tear with no findings of a systemic connective-tissue disorder such as Ehlers-Danlos syndrome.

We performed a literature search of PubMed articles indexed for MEDLINE using combinations of the terms atrophic, hypopigmented, white, spot disease, confetti-like, guttate, macules, atrophoderma, morphea, anetoderma, elastin, and collagen to identify potentially similar reports of guttate hypopigmented macules demonstrating changes of the collagen and elastin in the papillary and upper reticular dermis. Some variants, namely atrophoderma of Pasini and Pierini (APP), guttate morphea, and superficial morphea, demonstrate similar clinical and histopathologic findings.

Findings similar to our case were documented in case reports of 2 women (aged 34 and 42 years)¹ presenting with asymptomatic, atrophic, well-demarcated, shiny, hypopigmented macules over the trunk and upper extremities, which demonstrated a thinned epidermis with coarse hyalinized collagen bundles in the mid and lower dermis. There was upper and diffuse dermal elastolysis (patient 1 and patient 2, respectively).¹ Our patient’s lesions were hypopigmented and atrophic in appearance but were slightly scaly and also involved the extremities. Distinct from these patient reports, histopathology from our case demonstrated thin packed collagen bundles and decreased fragmented elastin filaments confined to the upper reticular and papillary dermis.

Plaque morphea is the most common type of localized scleroderma.² The subtype APP demonstrates round to ovoid, gray-brown depressions with cliff-drop borders. They may appear flesh colored or hypopigmented.^3,4 These sclerodermoid lesions lack the violaceous border classic to morphea. Sclerosis and induration also are typically absent.⁵ Clinically, our patient’s macules resembled this entity. Histopathologically, APP shows normal epithelium with an increased basal layer pigmentation; preserved adnexal structures; and mid to lower dermal collagen edema, clumping, and homogenization.^3,4 Elastic fibers classically are unchanged, with exceptions.^6-11 Changes in the collagen and elastin of our patient were unlike those reported in APP, which occur in the mid to lower dermis.

Guttate morphea demonstrates small, pale, minimally indurated, coin-shaped lesions on the trunk. Histopathology reveals less sclerosis and more edema, resembling LS&A.¹² The earliest descriptions of this entity describe 3 stages: ivory/chalk white, scaly, and atrophic. Follicular plugging (absent in this patient) and fine scale can exist at any stage.^13,14 Flattened rete ridges mark an otherwise preserved epidermis; hyalinized collagen typically is superficial and demonstrates less sclerosis yet increased edema.^12-14 Fewer elastic fibers typically are present compared to normal skin. Changes seen in this entity are more superficial, as with our patient, than classic scleroderma. However, classic edema was not found in our patient’s biopsy specimen.

Superficial morphea, occurring predominantly in females, presents with hyperpigmented or hypopigmented patches having minimal to no induration. The lesions typically are asymptomatic. Histopathologically, collagen deposition and inflammation are confined to the superficial dermis without homogenization associated with LS&A, findings that were consistent with this patient’s biopsy.^15,16 However, similar to other morpheaform variants, elastic fibers are unchanged.¹⁵ Verhoeff-van Gieson stain of the biopsy (Figure 3) showed the decreased and fragmented elastin network in the upper reticular and papillary dermis, making this entity less compatible.

Guttate LS&A may present with interfollicular, bluish white macules or papules coalescing into patches or plaques. Lesions evolve to reveal atrophic thin skin with follicular plugging. Histology demonstrates a thinned epidermis with orthohypokeratosis marked by flattened rete ridges. The dermis reveals short hyalinized collagen fibrils with a loss of elastic fibers in the papillary and upper reticular dermis, giving a homogenized appearance. Early disease is marked by an inflammatory infiltrate.¹⁷ Most of these findings are consistent with our patient’s pathology, which was confined to the upper dermis. Lacking, however, were characteristic findings of LS&A, including upper dermal homogenization, near-total effacement of rete ridges, orthokeratosis, and vacuolar degeneration at the dermoepidermal junction. As such, this entity is less compatible.

Atrophoderma elastolyticum discretum has clinical features of atrophoderma with elastolytic histopathologic findings.¹ Anetoderma presents with outpouchings of atrophic skin with a surrounding ring of normal tissue. Histopathologically, this entity shows normal collagen with elastolysis; there also is a decrease in desmosine, an elastin cross-linker.^1,3 Neither the clinical nor histopathologic findings in this patient matched these 2 entities.

The reported chronologic association of these lesions with an arthropod assault raised suspicion to their association with toxic insult or postinflammatory changes. One study reported mechanical trauma, including insect bites, as a possible inciting factor of morphea.¹¹ These data, gathered from patient surveys, reported trauma associated to lesion development.^1,17 A review of the literature regarding atrophoderma, morphea, and LS&A failed to identify pathogenic changes seen in this patient following initial trauma. Moreover, although it is difficult to prove causality in the formation of the original hypopigmented spots, the development of identical spots in a similar distribution without further trauma suggests against these etiologies to fully explain her lesions. Nonetheless, circumstance makes it difficult to prove whether the original arthropod insult spurred a smoldering reactive process that caused the newer lesions.

Hereditary connective-tissue disorders also were considered in the differential diagnosis. Because of the patient’s history of an unprovoked complete rotator cuff tear, Ehlers-Danlos syndrome was considered; however, the remainder of her examination was normal, making a syndromic systemic disorder a less likely etiology.Because of the distinct clinical and histopathologic findings, this case may represent a rare and previously unreported variant of morphea. Clinically, these hypopigmented macules and patches exist somewhere along the morphea-atrophoderma spectrum. Histopathologic findings do not conform to prior reports. The name atrophodermalike guttate morphea may be an appropriate appellation. It is possible this presentation represents a variant of what dermatologists have referred to as white spot disease.¹⁸ We hope that this case may bring others to discussion, allowing for the identification of a more precise entity and etiology so that patients may receive more directed therapy.

To the Editor:

Morphea, atrophoderma, guttate lichen sclerosus et atrophicus (LS&A), anetoderma, and their subtypes are inflammatory processes ultimately leading to dermal remodeling. We report a case of a scaly, hypopigmented, macular rash that clinically appeared as an entity along the morphea-atrophoderma spectrum and demonstrated unique histopathologic changes in both collagen and elastin confined to the upper reticular and papillary dermis. This case is a potentially rare variant representing a combination of clinical and microscopic findings.

A 29-year-old woman presented for an increasing number of white spots distributed on the trunk, arms, and legs. She denied local and systemic symptoms. The patient reported that she was stung by 100 wasps 23 years prior. Following the assault, her grandmother placed chewed tobacco leaves atop the painful erythematous wheals and flares. Upon resolution, hypopigmented macules and patches remained in their place. The patient denied associated symptoms or new lesions; she did not seek care at that time.

In her early 20s, the patient noted new, similarly distributed hypopigmented macules and patches without associated arthropod assault. She was treated by an outside dermatologist without result for presumed tinea versicolor. A follow-up superficial shave biopsy cited subtle psoriasiform dermatitis. Topical steroids did not improve the lesions. Her medical history also was remarkable for a reportedly unprovoked complete rotator cuff tear.

Physical examination revealed 0.5- to 2.0-cm, ill-defined, perifollicular and nonfollicular, slightly scaly macules and patches on the trunk, arms, and legs. There was no follicular plugging (Figure 1A). The hands, feet, face, and mucosal surfaces were spared. She had no family history of similar lesions. Although atrophic in appearance, a single lesion on the left thigh was palpably depressed (Figure 1B). Serology demonstrated a normal complete blood cell count and comprehensive metabolic panel, and negative Lyme titers. Light therapy and topical steroids failed to improve the lesions; calcipotriene cream 0.005% made the lesions erythematous and pruritic.

A biopsy from a flank lesion demonstrated a normal epithelium without thinning, a normal basal melanocyte population, and minimally effaced rete ridges. Thin collagen bundles were noted in the upper reticular and papillary dermis with associated fibroplasia (Figure 2). Verhoeff-van Gieson stain revealed decreased and fragmented elastin filaments in the same dermal distribution as the changed collagen (Figure 3). There was no evidence of primary inflammatory disease. The dermis was thinned. Periodic acid–Schiff stain confirmed the absence of hyphae and spores.

The relevant findings in our patient including the following: (1) onset of hypopigmented macules and patches following resolution of a toxic insult; (2) initially stable number of lesions that progressed in number but not size; (3) thinned collagen associated with fibroplasia in the upper reticular and papillary dermis; (4) decreased number and fragmentation of elastin filaments confined to the same region; (5) no congenital lesions or similar lesions in family members; and (6) a complete rotator cuff tear with no findings of a systemic connective-tissue disorder such as Ehlers-Danlos syndrome.

We performed a literature search of PubMed articles indexed for MEDLINE using combinations of the terms atrophic, hypopigmented, white, spot disease, confetti-like, guttate, macules, atrophoderma, morphea, anetoderma, elastin, and collagen to identify potentially similar reports of guttate hypopigmented macules demonstrating changes of the collagen and elastin in the papillary and upper reticular dermis. Some variants, namely atrophoderma of Pasini and Pierini (APP), guttate morphea, and superficial morphea, demonstrate similar clinical and histopathologic findings.

Findings similar to our case were documented in case reports of 2 women (aged 34 and 42 years)¹ presenting with asymptomatic, atrophic, well-demarcated, shiny, hypopigmented macules over the trunk and upper extremities, which demonstrated a thinned epidermis with coarse hyalinized collagen bundles in the mid and lower dermis. There was upper and diffuse dermal elastolysis (patient 1 and patient 2, respectively).¹ Our patient’s lesions were hypopigmented and atrophic in appearance but were slightly scaly and also involved the extremities. Distinct from these patient reports, histopathology from our case demonstrated thin packed collagen bundles and decreased fragmented elastin filaments confined to the upper reticular and papillary dermis.

Plaque morphea is the most common type of localized scleroderma.² The subtype APP demonstrates round to ovoid, gray-brown depressions with cliff-drop borders. They may appear flesh colored or hypopigmented.^3,4 These sclerodermoid lesions lack the violaceous border classic to morphea. Sclerosis and induration also are typically absent.⁵ Clinically, our patient’s macules resembled this entity. Histopathologically, APP shows normal epithelium with an increased basal layer pigmentation; preserved adnexal structures; and mid to lower dermal collagen edema, clumping, and homogenization.^3,4 Elastic fibers classically are unchanged, with exceptions.^6-11 Changes in the collagen and elastin of our patient were unlike those reported in APP, which occur in the mid to lower dermis.

Guttate morphea demonstrates small, pale, minimally indurated, coin-shaped lesions on the trunk. Histopathology reveals less sclerosis and more edema, resembling LS&A.¹² The earliest descriptions of this entity describe 3 stages: ivory/chalk white, scaly, and atrophic. Follicular plugging (absent in this patient) and fine scale can exist at any stage.^13,14 Flattened rete ridges mark an otherwise preserved epidermis; hyalinized collagen typically is superficial and demonstrates less sclerosis yet increased edema.^12-14 Fewer elastic fibers typically are present compared to normal skin. Changes seen in this entity are more superficial, as with our patient, than classic scleroderma. However, classic edema was not found in our patient’s biopsy specimen.

Superficial morphea, occurring predominantly in females, presents with hyperpigmented or hypopigmented patches having minimal to no induration. The lesions typically are asymptomatic. Histopathologically, collagen deposition and inflammation are confined to the superficial dermis without homogenization associated with LS&A, findings that were consistent with this patient’s biopsy.^15,16 However, similar to other morpheaform variants, elastic fibers are unchanged.¹⁵ Verhoeff-van Gieson stain of the biopsy (Figure 3) showed the decreased and fragmented elastin network in the upper reticular and papillary dermis, making this entity less compatible.

Guttate LS&A may present with interfollicular, bluish white macules or papules coalescing into patches or plaques. Lesions evolve to reveal atrophic thin skin with follicular plugging. Histology demonstrates a thinned epidermis with orthohypokeratosis marked by flattened rete ridges. The dermis reveals short hyalinized collagen fibrils with a loss of elastic fibers in the papillary and upper reticular dermis, giving a homogenized appearance. Early disease is marked by an inflammatory infiltrate.¹⁷ Most of these findings are consistent with our patient’s pathology, which was confined to the upper dermis. Lacking, however, were characteristic findings of LS&A, including upper dermal homogenization, near-total effacement of rete ridges, orthokeratosis, and vacuolar degeneration at the dermoepidermal junction. As such, this entity is less compatible.

Atrophoderma elastolyticum discretum has clinical features of atrophoderma with elastolytic histopathologic findings.¹ Anetoderma presents with outpouchings of atrophic skin with a surrounding ring of normal tissue. Histopathologically, this entity shows normal collagen with elastolysis; there also is a decrease in desmosine, an elastin cross-linker.^1,3 Neither the clinical nor histopathologic findings in this patient matched these 2 entities.

The reported chronologic association of these lesions with an arthropod assault raised suspicion to their association with toxic insult or postinflammatory changes. One study reported mechanical trauma, including insect bites, as a possible inciting factor of morphea.¹¹ These data, gathered from patient surveys, reported trauma associated to lesion development.^1,17 A review of the literature regarding atrophoderma, morphea, and LS&A failed to identify pathogenic changes seen in this patient following initial trauma. Moreover, although it is difficult to prove causality in the formation of the original hypopigmented spots, the development of identical spots in a similar distribution without further trauma suggests against these etiologies to fully explain her lesions. Nonetheless, circumstance makes it difficult to prove whether the original arthropod insult spurred a smoldering reactive process that caused the newer lesions.

Hereditary connective-tissue disorders also were considered in the differential diagnosis. Because of the patient’s history of an unprovoked complete rotator cuff tear, Ehlers-Danlos syndrome was considered; however, the remainder of her examination was normal, making a syndromic systemic disorder a less likely etiology.Because of the distinct clinical and histopathologic findings, this case may represent a rare and previously unreported variant of morphea. Clinically, these hypopigmented macules and patches exist somewhere along the morphea-atrophoderma spectrum. Histopathologic findings do not conform to prior reports. The name atrophodermalike guttate morphea may be an appropriate appellation. It is possible this presentation represents a variant of what dermatologists have referred to as white spot disease.¹⁸ We hope that this case may bring others to discussion, allowing for the identification of a more precise entity and etiology so that patients may receive more directed therapy.