Make the Diagnosis

Dermatofibrosarcoma protuberans (DFSP) is an uncommon malignant tumor. Young and middle-aged adults tend to be affected most often, though childhood DFSP has been reported.

Chromosomal translocation abnormalities in the tumor cells involving chromosomes 17 and 22 resulting in the fusion gene COL1A1-PDGFB have been reported in DFSP. This translocation causes an overproduction of the protein platelet-derived growth factor, resulting in tumor growth.

Lesions are most common in the trunk and proximal extremities. Less commonly, the head and neck may be involved. Lesions present as painless slow-growing red-brown nodules that may become painful as they enlarge. The differential diagnosis for early DFSP includes large dermatofibroma, keloid, dermatomyofibroma, and morphea. DFSP in childhood tends to appear more atrophic. It may be difficult to diagnose DFSP if the initial biopsy is superficial. If clinical suspicion is high, rebiopsy, ideally into the fat, is recommended.

Histologically, there is a cellular proliferation of thin spindled fibroblasts and collagen in the dermis that extend into the fat, often in a multilayered pattern. Adnexal structures can be obliterated. Fibroblasts may form a cartwheel or storiform pattern. There is mild cytologic atypia. Fibrosarcomatous change may signal increased risk of metastasis. CD34 is often positive and factor XIIIa is negative, unlike in dermatofibroma, which is opposite. Forms of DFSP that can be seen histologically include atrophic DFSP (flat rather than nodular), myxoid DFSP, and pigmented DFSP (also known as Bednar tumor).

DFSP can have irregular shapes with extensions into the fat. Subsequently, DFSP has a high recurrence rate with traditional surgical removal. Mohs surgery is now the treatment of choice. Recurrent tumors should be resected. As metastasis is rare, further work-up is not routinely indicated unless history and physical examination warrant it. Imatinib mesylate (Gleevec), which targets the platelet-derived growth factor receptor, has been tried with patients with inoperable or metastatic DFSP with some success. Radiation may also be used as an adjuvant after surgery. Regular follow-up exams with examination of the surgical site for possible recurrence should be performed every 6-12 months.

This case and photo were submitted by Dr. Bilu Martin.

Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at mdedge.com/dermatology. To submit a case for possible publication, send an email to [email protected].

Dermatofibrosarcoma protuberans (DFSP) is an uncommon malignant tumor. Young and middle-aged adults tend to be affected most often, though childhood DFSP has been reported.

Chromosomal translocation abnormalities in the tumor cells involving chromosomes 17 and 22 resulting in the fusion gene COL1A1-PDGFB have been reported in DFSP. This translocation causes an overproduction of the protein platelet-derived growth factor, resulting in tumor growth.

Lesions are most common in the trunk and proximal extremities. Less commonly, the head and neck may be involved. Lesions present as painless slow-growing red-brown nodules that may become painful as they enlarge. The differential diagnosis for early DFSP includes large dermatofibroma, keloid, dermatomyofibroma, and morphea. DFSP in childhood tends to appear more atrophic. It may be difficult to diagnose DFSP if the initial biopsy is superficial. If clinical suspicion is high, rebiopsy, ideally into the fat, is recommended.

Histologically, there is a cellular proliferation of thin spindled fibroblasts and collagen in the dermis that extend into the fat, often in a multilayered pattern. Adnexal structures can be obliterated. Fibroblasts may form a cartwheel or storiform pattern. There is mild cytologic atypia. Fibrosarcomatous change may signal increased risk of metastasis. CD34 is often positive and factor XIIIa is negative, unlike in dermatofibroma, which is opposite. Forms of DFSP that can be seen histologically include atrophic DFSP (flat rather than nodular), myxoid DFSP, and pigmented DFSP (also known as Bednar tumor).

DFSP can have irregular shapes with extensions into the fat. Subsequently, DFSP has a high recurrence rate with traditional surgical removal. Mohs surgery is now the treatment of choice. Recurrent tumors should be resected. As metastasis is rare, further work-up is not routinely indicated unless history and physical examination warrant it. Imatinib mesylate (Gleevec), which targets the platelet-derived growth factor receptor, has been tried with patients with inoperable or metastatic DFSP with some success. Radiation may also be used as an adjuvant after surgery. Regular follow-up exams with examination of the surgical site for possible recurrence should be performed every 6-12 months.

This case and photo were submitted by Dr. Bilu Martin.

Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at mdedge.com/dermatology. To submit a case for possible publication, send an email to [email protected].

Dermatofibrosarcoma protuberans (DFSP) is an uncommon malignant tumor. Young and middle-aged adults tend to be affected most often, though childhood DFSP has been reported.

Chromosomal translocation abnormalities in the tumor cells involving chromosomes 17 and 22 resulting in the fusion gene COL1A1-PDGFB have been reported in DFSP. This translocation causes an overproduction of the protein platelet-derived growth factor, resulting in tumor growth.

Lesions are most common in the trunk and proximal extremities. Less commonly, the head and neck may be involved. Lesions present as painless slow-growing red-brown nodules that may become painful as they enlarge. The differential diagnosis for early DFSP includes large dermatofibroma, keloid, dermatomyofibroma, and morphea. DFSP in childhood tends to appear more atrophic. It may be difficult to diagnose DFSP if the initial biopsy is superficial. If clinical suspicion is high, rebiopsy, ideally into the fat, is recommended.

Histologically, there is a cellular proliferation of thin spindled fibroblasts and collagen in the dermis that extend into the fat, often in a multilayered pattern. Adnexal structures can be obliterated. Fibroblasts may form a cartwheel or storiform pattern. There is mild cytologic atypia. Fibrosarcomatous change may signal increased risk of metastasis. CD34 is often positive and factor XIIIa is negative, unlike in dermatofibroma, which is opposite. Forms of DFSP that can be seen histologically include atrophic DFSP (flat rather than nodular), myxoid DFSP, and pigmented DFSP (also known as Bednar tumor).

DFSP can have irregular shapes with extensions into the fat. Subsequently, DFSP has a high recurrence rate with traditional surgical removal. Mohs surgery is now the treatment of choice. Recurrent tumors should be resected. As metastasis is rare, further work-up is not routinely indicated unless history and physical examination warrant it. Imatinib mesylate (Gleevec), which targets the platelet-derived growth factor receptor, has been tried with patients with inoperable or metastatic DFSP with some success. Radiation may also be used as an adjuvant after surgery. Regular follow-up exams with examination of the surgical site for possible recurrence should be performed every 6-12 months.

This case and photo were submitted by Dr. Bilu Martin.

Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at mdedge.com/dermatology. To submit a case for possible publication, send an email to [email protected].

Nummular dermatitis, or nummular eczema, is an inflammatory skin condition that is considered to be a distinctive form of idiopathic eczema, while the term also is used to describe lesional morphology associated with other conditions.

The term nummular derives from the Latin word for “coin,” as lesions are commonly annular plaques. Lesions of nummular dermatitis can be single or multiple. The typical distribution involves the extremities and, although less common, it can affect the trunk as well. The morphology of lesions is consistent with a localized eczematous reaction, with induration, oozing, crusting, and/or scaling.

Nummular dermatitis may be associated with atopic dermatitis, or it can be an isolated condition.¹ While the pathogenesis is uncertain, instigating factors include xerotic skin, insect bites, or scratches or scrapes.¹Staphylococcus infection or colonization, contact allergies to metals such as nickel and less commonly mercury, sensitivity to formaldehyde or medicines such as neomycin, and sensitization to an environmental aeroallergen (such as Candida albicans, dust mites) are considered risk factors.²

The diagnosis of nummular dermatitis is clinical. Laboratory testing and/or biopsy generally are not necessary, although a bacterial culture can be considered in patients with exudative and/or crusted lesions to rule out impetigo as a primary process of secondary infection. In some cases, patch testing for allergic contact dermatitis may be useful.

The differential diagnosis of nummular dermatitis includes tinea corporis (ringworm), atopic dermatitis, allergic contact dermatitis, impetigo, and psoriasis. Tinea corporis usually presents as annular lesions with a distinct peripheral scaling, rather than the diffuse induration of nummular dermatitis. Potassium hydroxide preparation or fungal culture can identify tinea species. Nummular dermatitis may be seen in patients with atopic dermatitis, who should have typical history, morphology, and course consistent with standard diagnostic criteria. Allergic contact dermatitis can present with regional, localized eczematous plaques in areas exposed to contact allergens. Patterns of lesions in areas of contact and worsening with repeat exposures can be clues to this diagnosis. Impetigo can present with honey-colored crusted lesions and/or superficial erosions, or purulent pyoderma. Lesions can be single or multiple and generally appear less inflammatory than nummular dermatitis. Psoriasis lesions may be annular, are more common on extensor surfaces, and usually have more prominent overlying pinkish, silvery white or micaceous scale.

Management of nummular dermatitis requires strong anti-inflammatory medications, usually mid-potency or higher topical corticosteroids, along with moisturizers and limiting exposure to skin irritants. “Wet wraps,” with application of topical corticosteroids to wet skin with occlusive wet dressings can enhance response. Transition from higher strength topical corticosteroids to lower strength agents used intermittently can help achieve remission or cure. Management practices include less frequent bathing with lukewarm water, using hypoallergenic cleansers and detergents, and applying moisturizers frequently. If plaques do recur, they tend to do so in the same location and in some patients resolution may result in hyper or hypopigmentation. Refractory disease may be managed with intralesional steroid injections, or systemic medications such as methotrexate.³

Dr. Tracy is a research fellow in pediatric dermatology at Rady Children’s Hospital–San Diego and the University of California, San Diego. Dr. Eichenfield is chief of pediatric and adolescent dermatology at Rady Children’s Hospital–San Diego. He is vice chair of the department of dermatology and professor of dermatology and pediatrics at the University of California, San Diego. Neither Dr. Tracy nor Dr. Eichenfield have any relevant financial disclosures. Email them at [email protected].

References

1. Pediatr Dermatol. 2012 Sep-Oct;29(5):580-3.

2. American Academy of Dermatology. Nummular Dermatitis Overview

3. Pediatr Dermatol. 2018 Sep;35(5):611-5.

Nummular dermatitis, or nummular eczema, is an inflammatory skin condition that is considered to be a distinctive form of idiopathic eczema, while the term also is used to describe lesional morphology associated with other conditions.

The term nummular derives from the Latin word for “coin,” as lesions are commonly annular plaques. Lesions of nummular dermatitis can be single or multiple. The typical distribution involves the extremities and, although less common, it can affect the trunk as well. The morphology of lesions is consistent with a localized eczematous reaction, with induration, oozing, crusting, and/or scaling.

Nummular dermatitis may be associated with atopic dermatitis, or it can be an isolated condition.¹ While the pathogenesis is uncertain, instigating factors include xerotic skin, insect bites, or scratches or scrapes.¹Staphylococcus infection or colonization, contact allergies to metals such as nickel and less commonly mercury, sensitivity to formaldehyde or medicines such as neomycin, and sensitization to an environmental aeroallergen (such as Candida albicans, dust mites) are considered risk factors.²

The diagnosis of nummular dermatitis is clinical. Laboratory testing and/or biopsy generally are not necessary, although a bacterial culture can be considered in patients with exudative and/or crusted lesions to rule out impetigo as a primary process of secondary infection. In some cases, patch testing for allergic contact dermatitis may be useful.

The differential diagnosis of nummular dermatitis includes tinea corporis (ringworm), atopic dermatitis, allergic contact dermatitis, impetigo, and psoriasis. Tinea corporis usually presents as annular lesions with a distinct peripheral scaling, rather than the diffuse induration of nummular dermatitis. Potassium hydroxide preparation or fungal culture can identify tinea species. Nummular dermatitis may be seen in patients with atopic dermatitis, who should have typical history, morphology, and course consistent with standard diagnostic criteria. Allergic contact dermatitis can present with regional, localized eczematous plaques in areas exposed to contact allergens. Patterns of lesions in areas of contact and worsening with repeat exposures can be clues to this diagnosis. Impetigo can present with honey-colored crusted lesions and/or superficial erosions, or purulent pyoderma. Lesions can be single or multiple and generally appear less inflammatory than nummular dermatitis. Psoriasis lesions may be annular, are more common on extensor surfaces, and usually have more prominent overlying pinkish, silvery white or micaceous scale.

Management of nummular dermatitis requires strong anti-inflammatory medications, usually mid-potency or higher topical corticosteroids, along with moisturizers and limiting exposure to skin irritants. “Wet wraps,” with application of topical corticosteroids to wet skin with occlusive wet dressings can enhance response. Transition from higher strength topical corticosteroids to lower strength agents used intermittently can help achieve remission or cure. Management practices include less frequent bathing with lukewarm water, using hypoallergenic cleansers and detergents, and applying moisturizers frequently. If plaques do recur, they tend to do so in the same location and in some patients resolution may result in hyper or hypopigmentation. Refractory disease may be managed with intralesional steroid injections, or systemic medications such as methotrexate.³

Dr. Tracy is a research fellow in pediatric dermatology at Rady Children’s Hospital–San Diego and the University of California, San Diego. Dr. Eichenfield is chief of pediatric and adolescent dermatology at Rady Children’s Hospital–San Diego. He is vice chair of the department of dermatology and professor of dermatology and pediatrics at the University of California, San Diego. Neither Dr. Tracy nor Dr. Eichenfield have any relevant financial disclosures. Email them at [email protected].

References

1. Pediatr Dermatol. 2012 Sep-Oct;29(5):580-3.

2. American Academy of Dermatology. Nummular Dermatitis Overview

3. Pediatr Dermatol. 2018 Sep;35(5):611-5.

Nummular dermatitis, or nummular eczema, is an inflammatory skin condition that is considered to be a distinctive form of idiopathic eczema, while the term also is used to describe lesional morphology associated with other conditions.

The term nummular derives from the Latin word for “coin,” as lesions are commonly annular plaques. Lesions of nummular dermatitis can be single or multiple. The typical distribution involves the extremities and, although less common, it can affect the trunk as well. The morphology of lesions is consistent with a localized eczematous reaction, with induration, oozing, crusting, and/or scaling.

Nummular dermatitis may be associated with atopic dermatitis, or it can be an isolated condition.¹ While the pathogenesis is uncertain, instigating factors include xerotic skin, insect bites, or scratches or scrapes.¹Staphylococcus infection or colonization, contact allergies to metals such as nickel and less commonly mercury, sensitivity to formaldehyde or medicines such as neomycin, and sensitization to an environmental aeroallergen (such as Candida albicans, dust mites) are considered risk factors.²

The diagnosis of nummular dermatitis is clinical. Laboratory testing and/or biopsy generally are not necessary, although a bacterial culture can be considered in patients with exudative and/or crusted lesions to rule out impetigo as a primary process of secondary infection. In some cases, patch testing for allergic contact dermatitis may be useful.

The differential diagnosis of nummular dermatitis includes tinea corporis (ringworm), atopic dermatitis, allergic contact dermatitis, impetigo, and psoriasis. Tinea corporis usually presents as annular lesions with a distinct peripheral scaling, rather than the diffuse induration of nummular dermatitis. Potassium hydroxide preparation or fungal culture can identify tinea species. Nummular dermatitis may be seen in patients with atopic dermatitis, who should have typical history, morphology, and course consistent with standard diagnostic criteria. Allergic contact dermatitis can present with regional, localized eczematous plaques in areas exposed to contact allergens. Patterns of lesions in areas of contact and worsening with repeat exposures can be clues to this diagnosis. Impetigo can present with honey-colored crusted lesions and/or superficial erosions, or purulent pyoderma. Lesions can be single or multiple and generally appear less inflammatory than nummular dermatitis. Psoriasis lesions may be annular, are more common on extensor surfaces, and usually have more prominent overlying pinkish, silvery white or micaceous scale.

Management of nummular dermatitis requires strong anti-inflammatory medications, usually mid-potency or higher topical corticosteroids, along with moisturizers and limiting exposure to skin irritants. “Wet wraps,” with application of topical corticosteroids to wet skin with occlusive wet dressings can enhance response. Transition from higher strength topical corticosteroids to lower strength agents used intermittently can help achieve remission or cure. Management practices include less frequent bathing with lukewarm water, using hypoallergenic cleansers and detergents, and applying moisturizers frequently. If plaques do recur, they tend to do so in the same location and in some patients resolution may result in hyper or hypopigmentation. Refractory disease may be managed with intralesional steroid injections, or systemic medications such as methotrexate.³

Dr. Tracy is a research fellow in pediatric dermatology at Rady Children’s Hospital–San Diego and the University of California, San Diego. Dr. Eichenfield is chief of pediatric and adolescent dermatology at Rady Children’s Hospital–San Diego. He is vice chair of the department of dermatology and professor of dermatology and pediatrics at the University of California, San Diego. Neither Dr. Tracy nor Dr. Eichenfield have any relevant financial disclosures. Email them at [email protected].

References

1. Pediatr Dermatol. 2012 Sep-Oct;29(5):580-3.

2. American Academy of Dermatology. Nummular Dermatitis Overview

3. Pediatr Dermatol. 2018 Sep;35(5):611-5.

Progressive macular hypomelanosis (PMH) presents as asymptomatic, ill-defined hypopigmented macules and patches on the trunk and back. They often coalesce near the midline, and occasionally extend beyond the trunk to the arms, legs, head, or neck. PMH is more frequently diagnosed among black individuals, although it affects all races and ethnicities. The natural history of PMH can vary from stable and progressive disease, and may resolve spontaneously after a few years. The pathogenesis of PMH remains unknown. It has been proposed that the hypopigmentation is caused by decreased melanin production and altered melanosome dispersal in reaction to Propionibacterium acnes.

PMH must be distinguished from some of its clinical mimickers, including vitiligo, hypopigmented mycosis fungoides, tinea versicolor, and pityriasis alba. Potassium hydroxide preparations can be performed in the office to evaluate for tinea versicolor. An additional tool to aid in diagnosis is the use of a Wood’s light. The lesions of PMH characteristically show punctiform orange-red follicular fluorescence when exposed to a Wood’s light, indicating the presence of a porphyrin-producing organism, presumably P. acnes. A skin biopsy is necessary to rule out hypopigmented mycosis fungoides.

Skin biopsy of PMH typically reveals decreased melanin with a normal number of melanocytes. In our patient, a punch biopsy of the right lateral arm demonstrated minimally decreased density of epidermal melanocytes with dermal pigment incontinence. SOX10 immunohistochemical staining demonstrated scattered melanocytes in the epidermis. Preserved melanin within keratinocytes was noted.

In our patient, there was significant spread to the face, which is highly unusual and has only been documented in a few case series. There are no standard recommendations for definitive treatment of PMH. Topical antimicrobial therapies, such as clindamycin solution and benzoyl peroxide gel, have been beneficial in some studies. Tetracyclines, narrow-band ultraviolet B phototherapy, and even isotretinoin have had some reported success.

This case and photo were submitted by Mr. Franzetti, Dr. Rush, and Dr. Shalin of the University of Arkansas for Medical Sciences, Little Rock.

Donna Bilu Martin, MD, is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at mdedge.com/dermatology. To submit a case for possible publication, send an email to [email protected].

Progressive macular hypomelanosis (PMH) presents as asymptomatic, ill-defined hypopigmented macules and patches on the trunk and back. They often coalesce near the midline, and occasionally extend beyond the trunk to the arms, legs, head, or neck. PMH is more frequently diagnosed among black individuals, although it affects all races and ethnicities. The natural history of PMH can vary from stable and progressive disease, and may resolve spontaneously after a few years. The pathogenesis of PMH remains unknown. It has been proposed that the hypopigmentation is caused by decreased melanin production and altered melanosome dispersal in reaction to Propionibacterium acnes.

PMH must be distinguished from some of its clinical mimickers, including vitiligo, hypopigmented mycosis fungoides, tinea versicolor, and pityriasis alba. Potassium hydroxide preparations can be performed in the office to evaluate for tinea versicolor. An additional tool to aid in diagnosis is the use of a Wood’s light. The lesions of PMH characteristically show punctiform orange-red follicular fluorescence when exposed to a Wood’s light, indicating the presence of a porphyrin-producing organism, presumably P. acnes. A skin biopsy is necessary to rule out hypopigmented mycosis fungoides.

Skin biopsy of PMH typically reveals decreased melanin with a normal number of melanocytes. In our patient, a punch biopsy of the right lateral arm demonstrated minimally decreased density of epidermal melanocytes with dermal pigment incontinence. SOX10 immunohistochemical staining demonstrated scattered melanocytes in the epidermis. Preserved melanin within keratinocytes was noted.

In our patient, there was significant spread to the face, which is highly unusual and has only been documented in a few case series. There are no standard recommendations for definitive treatment of PMH. Topical antimicrobial therapies, such as clindamycin solution and benzoyl peroxide gel, have been beneficial in some studies. Tetracyclines, narrow-band ultraviolet B phototherapy, and even isotretinoin have had some reported success.

This case and photo were submitted by Mr. Franzetti, Dr. Rush, and Dr. Shalin of the University of Arkansas for Medical Sciences, Little Rock.

Donna Bilu Martin, MD, is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at mdedge.com/dermatology. To submit a case for possible publication, send an email to [email protected].

Progressive macular hypomelanosis (PMH) presents as asymptomatic, ill-defined hypopigmented macules and patches on the trunk and back. They often coalesce near the midline, and occasionally extend beyond the trunk to the arms, legs, head, or neck. PMH is more frequently diagnosed among black individuals, although it affects all races and ethnicities. The natural history of PMH can vary from stable and progressive disease, and may resolve spontaneously after a few years. The pathogenesis of PMH remains unknown. It has been proposed that the hypopigmentation is caused by decreased melanin production and altered melanosome dispersal in reaction to Propionibacterium acnes.

PMH must be distinguished from some of its clinical mimickers, including vitiligo, hypopigmented mycosis fungoides, tinea versicolor, and pityriasis alba. Potassium hydroxide preparations can be performed in the office to evaluate for tinea versicolor. An additional tool to aid in diagnosis is the use of a Wood’s light. The lesions of PMH characteristically show punctiform orange-red follicular fluorescence when exposed to a Wood’s light, indicating the presence of a porphyrin-producing organism, presumably P. acnes. A skin biopsy is necessary to rule out hypopigmented mycosis fungoides.

Skin biopsy of PMH typically reveals decreased melanin with a normal number of melanocytes. In our patient, a punch biopsy of the right lateral arm demonstrated minimally decreased density of epidermal melanocytes with dermal pigment incontinence. SOX10 immunohistochemical staining demonstrated scattered melanocytes in the epidermis. Preserved melanin within keratinocytes was noted.

In our patient, there was significant spread to the face, which is highly unusual and has only been documented in a few case series. There are no standard recommendations for definitive treatment of PMH. Topical antimicrobial therapies, such as clindamycin solution and benzoyl peroxide gel, have been beneficial in some studies. Tetracyclines, narrow-band ultraviolet B phototherapy, and even isotretinoin have had some reported success.

This case and photo were submitted by Mr. Franzetti, Dr. Rush, and Dr. Shalin of the University of Arkansas for Medical Sciences, Little Rock.

Donna Bilu Martin, MD, is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at mdedge.com/dermatology. To submit a case for possible publication, send an email to [email protected].

A shave biopsy of one of the lesions was performed that showed a proliferation of nests of basaloid cells on the dermis with palisading and rare vacuolated clear cell change. A rare ductal structure with luminal proteinaceous contents was noted. The findings were consistent with a trichoepithelioma.

Trichoepitheliomas are rare, benign, adnexal skin tumors that can start in early childhood or during puberty. The lesions are most commonly seen in girls as skin color papules on the face, and sometimes on the trunk and the neck. Trichoepitheliomas can appear as a benign single lesion nonfamilial form or as a familial form with multiple lesions.¹ Brooke-Spiegler syndrome (BSS) is a rare autosomal dominant condition where affected individuals have multiple trichoepitheliomas, cylindromas, and spiradenomas. Depending on the predominant type of lesion, phenotypic variants include multiple familial trichoepithelioma type 1 and familial cylindromatosis.² BSS is caused by mutations within CYLD, a tumor-suppressor gene located on chromosome 16q12-q13.³ Our patient presented only with trichoepitheliomas with no other lesions on the scalp, neck, or torso.

Multiple trichoepitheliomas also can be seen in other syndromes including Rombo syndrome, which is characterized by basal cell carcinomas, milia, hypotrichosis, distal vasodilation, and atrophoderma vermiculata; none seen in our patient. Bazex-Dupré-Christol syndrome is an X-linked dominant condition in which affected individuals can present with multiple trichoepitheliomas, as well as milia, hypotrichosis, follicular atrophoderma, and basal cell carcinomas.

The differential diagnosis of skin color papules on the central face on a child should include acne, flat warts, and angiofibromas seen in tuberous sclerosis. Our patient’s lesions were monomorphous, and there were no comedones, pustules, or inflammatory papules characteristic of acne.

He had warts on his hands which could make it suspicious for the face lesions to be verrucous in nature. Flat warts also present as skin color papules, but characteristically are flat, not round and shiny as our patient’s lesions were. Angiofibromas, as seen in individuals with tuberous sclerosis, also can start at an early age in the same location as trichoepitheliomas in BSS, but clinically the lesions are pinker and redder rather than the skin-color, round shape papules characteristic of trichoepitheliomas. Patients may have other findings suggestive of tuberous sclerosis including confetti hypopigmentation, ash leaf spots, shagreen patch, and a history of seizures or developmental delay – none of which were present in our patient. Children with basal cell nevus syndrome can present with skin color to shiny telangiectatic papules (basal cell carcinomas) that can be single or multiple on the face, chest, and back. The lesions usually are not seen in clusters around the nose and central face as seen in patients with BSS. Patients with basal cell nevus syndrome can develop jaw bone cysts, brain tumors (medulloblastoma), and fibromas on the heart or ovaries, palmar pits and be macrocephalic.⁴

Trichoepitheliomas usually are treated surgically but other nonsurgical removing techniques include laser resurfacing, curettage, and electrocautery.⁵ Malignant transformation can occur in 5%-10% of the individuals and should be managed by a multidisciplinary team. Topical treatment with sirolimus previously has been reported to be effective in young patients.⁶

Dr. Matiz is a pediatric dermatologist at Southern California Permanente Medical Group, San Diego. She said she had no relevant financial disclosures. Email Dr. Matiz at [email protected].

References

1. Acta Dermatovenerol Croat. 2018 Jun;26(2):162-5.

2. Eur J Med Genet. 2015;58(5):271-8.

3. Am J Dermatopathol. 2014;36(11):868-74.

4. Int J Dermatol. 2016 Apr;55(4):367-75.

5. Int J Dermatol. 2007;46(6):583-6.

6. Dermatol Ther. 2017 Mar. doi: 10.1111/dth.12458.

A shave biopsy of one of the lesions was performed that showed a proliferation of nests of basaloid cells on the dermis with palisading and rare vacuolated clear cell change. A rare ductal structure with luminal proteinaceous contents was noted. The findings were consistent with a trichoepithelioma.

Trichoepitheliomas are rare, benign, adnexal skin tumors that can start in early childhood or during puberty. The lesions are most commonly seen in girls as skin color papules on the face, and sometimes on the trunk and the neck. Trichoepitheliomas can appear as a benign single lesion nonfamilial form or as a familial form with multiple lesions.¹ Brooke-Spiegler syndrome (BSS) is a rare autosomal dominant condition where affected individuals have multiple trichoepitheliomas, cylindromas, and spiradenomas. Depending on the predominant type of lesion, phenotypic variants include multiple familial trichoepithelioma type 1 and familial cylindromatosis.² BSS is caused by mutations within CYLD, a tumor-suppressor gene located on chromosome 16q12-q13.³ Our patient presented only with trichoepitheliomas with no other lesions on the scalp, neck, or torso.

Multiple trichoepitheliomas also can be seen in other syndromes including Rombo syndrome, which is characterized by basal cell carcinomas, milia, hypotrichosis, distal vasodilation, and atrophoderma vermiculata; none seen in our patient. Bazex-Dupré-Christol syndrome is an X-linked dominant condition in which affected individuals can present with multiple trichoepitheliomas, as well as milia, hypotrichosis, follicular atrophoderma, and basal cell carcinomas.

The differential diagnosis of skin color papules on the central face on a child should include acne, flat warts, and angiofibromas seen in tuberous sclerosis. Our patient’s lesions were monomorphous, and there were no comedones, pustules, or inflammatory papules characteristic of acne.

He had warts on his hands which could make it suspicious for the face lesions to be verrucous in nature. Flat warts also present as skin color papules, but characteristically are flat, not round and shiny as our patient’s lesions were. Angiofibromas, as seen in individuals with tuberous sclerosis, also can start at an early age in the same location as trichoepitheliomas in BSS, but clinically the lesions are pinker and redder rather than the skin-color, round shape papules characteristic of trichoepitheliomas. Patients may have other findings suggestive of tuberous sclerosis including confetti hypopigmentation, ash leaf spots, shagreen patch, and a history of seizures or developmental delay – none of which were present in our patient. Children with basal cell nevus syndrome can present with skin color to shiny telangiectatic papules (basal cell carcinomas) that can be single or multiple on the face, chest, and back. The lesions usually are not seen in clusters around the nose and central face as seen in patients with BSS. Patients with basal cell nevus syndrome can develop jaw bone cysts, brain tumors (medulloblastoma), and fibromas on the heart or ovaries, palmar pits and be macrocephalic.⁴

Trichoepitheliomas usually are treated surgically but other nonsurgical removing techniques include laser resurfacing, curettage, and electrocautery.⁵ Malignant transformation can occur in 5%-10% of the individuals and should be managed by a multidisciplinary team. Topical treatment with sirolimus previously has been reported to be effective in young patients.⁶

Dr. Matiz is a pediatric dermatologist at Southern California Permanente Medical Group, San Diego. She said she had no relevant financial disclosures. Email Dr. Matiz at [email protected].

References

1. Acta Dermatovenerol Croat. 2018 Jun;26(2):162-5.

2. Eur J Med Genet. 2015;58(5):271-8.

3. Am J Dermatopathol. 2014;36(11):868-74.

4. Int J Dermatol. 2016 Apr;55(4):367-75.

5. Int J Dermatol. 2007;46(6):583-6.

6. Dermatol Ther. 2017 Mar. doi: 10.1111/dth.12458.

A shave biopsy of one of the lesions was performed that showed a proliferation of nests of basaloid cells on the dermis with palisading and rare vacuolated clear cell change. A rare ductal structure with luminal proteinaceous contents was noted. The findings were consistent with a trichoepithelioma.

Trichoepitheliomas are rare, benign, adnexal skin tumors that can start in early childhood or during puberty. The lesions are most commonly seen in girls as skin color papules on the face, and sometimes on the trunk and the neck. Trichoepitheliomas can appear as a benign single lesion nonfamilial form or as a familial form with multiple lesions.¹ Brooke-Spiegler syndrome (BSS) is a rare autosomal dominant condition where affected individuals have multiple trichoepitheliomas, cylindromas, and spiradenomas. Depending on the predominant type of lesion, phenotypic variants include multiple familial trichoepithelioma type 1 and familial cylindromatosis.² BSS is caused by mutations within CYLD, a tumor-suppressor gene located on chromosome 16q12-q13.³ Our patient presented only with trichoepitheliomas with no other lesions on the scalp, neck, or torso.

Multiple trichoepitheliomas also can be seen in other syndromes including Rombo syndrome, which is characterized by basal cell carcinomas, milia, hypotrichosis, distal vasodilation, and atrophoderma vermiculata; none seen in our patient. Bazex-Dupré-Christol syndrome is an X-linked dominant condition in which affected individuals can present with multiple trichoepitheliomas, as well as milia, hypotrichosis, follicular atrophoderma, and basal cell carcinomas.

The differential diagnosis of skin color papules on the central face on a child should include acne, flat warts, and angiofibromas seen in tuberous sclerosis. Our patient’s lesions were monomorphous, and there were no comedones, pustules, or inflammatory papules characteristic of acne.

He had warts on his hands which could make it suspicious for the face lesions to be verrucous in nature. Flat warts also present as skin color papules, but characteristically are flat, not round and shiny as our patient’s lesions were. Angiofibromas, as seen in individuals with tuberous sclerosis, also can start at an early age in the same location as trichoepitheliomas in BSS, but clinically the lesions are pinker and redder rather than the skin-color, round shape papules characteristic of trichoepitheliomas. Patients may have other findings suggestive of tuberous sclerosis including confetti hypopigmentation, ash leaf spots, shagreen patch, and a history of seizures or developmental delay – none of which were present in our patient. Children with basal cell nevus syndrome can present with skin color to shiny telangiectatic papules (basal cell carcinomas) that can be single or multiple on the face, chest, and back. The lesions usually are not seen in clusters around the nose and central face as seen in patients with BSS. Patients with basal cell nevus syndrome can develop jaw bone cysts, brain tumors (medulloblastoma), and fibromas on the heart or ovaries, palmar pits and be macrocephalic.⁴

Trichoepitheliomas usually are treated surgically but other nonsurgical removing techniques include laser resurfacing, curettage, and electrocautery.⁵ Malignant transformation can occur in 5%-10% of the individuals and should be managed by a multidisciplinary team. Topical treatment with sirolimus previously has been reported to be effective in young patients.⁶

Dr. Matiz is a pediatric dermatologist at Southern California Permanente Medical Group, San Diego. She said she had no relevant financial disclosures. Email Dr. Matiz at [email protected].

References

1. Acta Dermatovenerol Croat. 2018 Jun;26(2):162-5.

2. Eur J Med Genet. 2015;58(5):271-8.

3. Am J Dermatopathol. 2014;36(11):868-74.

4. Int J Dermatol. 2016 Apr;55(4):367-75.

5. Int J Dermatol. 2007;46(6):583-6.

6. Dermatol Ther. 2017 Mar. doi: 10.1111/dth.12458.

Cardiac implantable electronic devices (CIEDs) – cardiac pacemakers and implantable cardioverter defibrillators –are an established treatment for the management of cardiac dysrhythmias in millions of patients. Complications occur in up to 15%, some of which may present first to the dermatologist.

The differential diagnosis of dermatoses overlying pacemakers includes infection, irritant or allergic contact dermatitis, reticular telangiectatic erythema (caused by local venous obstruction and pressure dermatitis), and impending skin erosion/device extrusion.

Erosion and extrusion is a major complication with significant morbidity and mortality. The two main causes are pressure necrosis and infection. Pressure necrosis is influenced by the size of the device, complexity of the connections, and technical skill with which the pacemaker chest wall pocket is created.

After extrusion, the pacemaker should be considered contaminated and removed, and the necrotic tissue debrided. If infected, a prolonged course of appropriate antibiotic therapy is indicated. A bacterial culture in the patient presented here was negative.

Pocket infection of CIEDs is rare and may manifest as erythema, tenderness, drainage, erosion, or pruritus above the site of the pacemaker, along with systemic symptoms and signs, including fever, chills, or malaise. Some may have just the systemic symptoms. Fewer than half of patients with CIED infection present within 1 year of their last procedure.

Ruptured epidermal cysts usually manifest as acute swelling, inflammation, and tenderness of previously long-standing asymptomatic epidermal cysts. There may be drainage of malodorous keratinous and purulent debris. They are typically not infected. Treatment includes incision and drainage for fluctuant lesions or intralesional corticosteroid injection for early, nonfluctuant cases.

Allergic contact dermatitis to metal may be seen with implantable devices. Patch testing to various metal allergens can be helpful in determining if any allergy is present.

This case and photo were submitted by Michael Stierstorfer, MD, East Penn Dermatology, North Wales, Pa.

Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, in Aventura, Fla. More diagnostic cases are available at mdedge.com/dermatology. To submit a case for possible publication, send an email to [email protected].

Cardiac implantable electronic devices (CIEDs) – cardiac pacemakers and implantable cardioverter defibrillators –are an established treatment for the management of cardiac dysrhythmias in millions of patients. Complications occur in up to 15%, some of which may present first to the dermatologist.

The differential diagnosis of dermatoses overlying pacemakers includes infection, irritant or allergic contact dermatitis, reticular telangiectatic erythema (caused by local venous obstruction and pressure dermatitis), and impending skin erosion/device extrusion.

Erosion and extrusion is a major complication with significant morbidity and mortality. The two main causes are pressure necrosis and infection. Pressure necrosis is influenced by the size of the device, complexity of the connections, and technical skill with which the pacemaker chest wall pocket is created.

After extrusion, the pacemaker should be considered contaminated and removed, and the necrotic tissue debrided. If infected, a prolonged course of appropriate antibiotic therapy is indicated. A bacterial culture in the patient presented here was negative.

Pocket infection of CIEDs is rare and may manifest as erythema, tenderness, drainage, erosion, or pruritus above the site of the pacemaker, along with systemic symptoms and signs, including fever, chills, or malaise. Some may have just the systemic symptoms. Fewer than half of patients with CIED infection present within 1 year of their last procedure.

Ruptured epidermal cysts usually manifest as acute swelling, inflammation, and tenderness of previously long-standing asymptomatic epidermal cysts. There may be drainage of malodorous keratinous and purulent debris. They are typically not infected. Treatment includes incision and drainage for fluctuant lesions or intralesional corticosteroid injection for early, nonfluctuant cases.

Allergic contact dermatitis to metal may be seen with implantable devices. Patch testing to various metal allergens can be helpful in determining if any allergy is present.

This case and photo were submitted by Michael Stierstorfer, MD, East Penn Dermatology, North Wales, Pa.

Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, in Aventura, Fla. More diagnostic cases are available at mdedge.com/dermatology. To submit a case for possible publication, send an email to [email protected].

Cardiac implantable electronic devices (CIEDs) – cardiac pacemakers and implantable cardioverter defibrillators –are an established treatment for the management of cardiac dysrhythmias in millions of patients. Complications occur in up to 15%, some of which may present first to the dermatologist.

The differential diagnosis of dermatoses overlying pacemakers includes infection, irritant or allergic contact dermatitis, reticular telangiectatic erythema (caused by local venous obstruction and pressure dermatitis), and impending skin erosion/device extrusion.

Erosion and extrusion is a major complication with significant morbidity and mortality. The two main causes are pressure necrosis and infection. Pressure necrosis is influenced by the size of the device, complexity of the connections, and technical skill with which the pacemaker chest wall pocket is created.

After extrusion, the pacemaker should be considered contaminated and removed, and the necrotic tissue debrided. If infected, a prolonged course of appropriate antibiotic therapy is indicated. A bacterial culture in the patient presented here was negative.

Pocket infection of CIEDs is rare and may manifest as erythema, tenderness, drainage, erosion, or pruritus above the site of the pacemaker, along with systemic symptoms and signs, including fever, chills, or malaise. Some may have just the systemic symptoms. Fewer than half of patients with CIED infection present within 1 year of their last procedure.

Ruptured epidermal cysts usually manifest as acute swelling, inflammation, and tenderness of previously long-standing asymptomatic epidermal cysts. There may be drainage of malodorous keratinous and purulent debris. They are typically not infected. Treatment includes incision and drainage for fluctuant lesions or intralesional corticosteroid injection for early, nonfluctuant cases.

Allergic contact dermatitis to metal may be seen with implantable devices. Patch testing to various metal allergens can be helpful in determining if any allergy is present.

This case and photo were submitted by Michael Stierstorfer, MD, East Penn Dermatology, North Wales, Pa.

Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, in Aventura, Fla. More diagnostic cases are available at mdedge.com/dermatology. To submit a case for possible publication, send an email to [email protected].

Lichen striatus (LS) is a common benign skin condition that presents in children between the ages of 5 and 15 years.¹ The rash is typically unilateral and most frequently on the extremities, although it may appear on the face, trunk, or buttocks. The lesions start as pink or skin-colored asymptomatic papules in a linear orientation following the lines of Blaschko. Lesions usually evolve over several weeks, and typically resolve in 6-12 months. There may be residual postinflammatory hypo- or hyperpigmentation which often improves within a few years.

Of note, there are subsets of lichen striatus: Hypopigmented lichen striatus with minimal papules has been termed “lichen striatus albus.” Nail lichen striatus may present as onycholysis or fissuring of nails, present as an isolated finding, or more commonly in association with concurrent affected skin. Nail lichen striatus typically resolves on its own, however there are case reports of improvement with intralesional steroids.²

There is no established etiology for LS. Autoimmune disease, viruses, immunizations, medications, and hypersensitivity reactions have been associated with triggering LS in various case reports, although strength of the associations is low. Children have been reported to have LS following scarlet fever and Candida vulvitis.³ Diagnosis usually is clinical, although biopsy may be helpful for histopathologic confirmation. No work-up for associated infections or conditions is warranted.

The differential for linear papular lesions includes inflammatory linear verrucous epidermal nevus (ILVEN), blaschkitis, or linear morphea. ILVEN is a hamartoma that usually is congenital or presents in early childhood; presents with linear or whorled, hyperkeratotic papules and plaque in similar linear “line of Blaschko” patterns; and represents cutaneous mosaicism. It is often difficult to differentiate between lichen striatus and ILVEN, however lichen striatus is not congenital, and is a self-limited condition. Under dermoscopy (polarized light systems) findings of LS more frequently demonstrate gray granular pigmentation. ILVEN is more frequently associated with cerebriform pattern.⁴ Blaschkitis is a term for a blaschkoid inflammation of the skin that presents with more eczematous findings and histology of spongiosis, unlike the lichenoid findings of LS. It is typically accompanied by noticeable pruritus and broader bands of involved area, and has older age of onset than LS. Linear morphea is a deeper inflammatory process of the dermis or subcutaneous fat, presenting with sclerotic skin, and typically has associated atrophy.

Treatment need not be pursued for lichen striatus because it is a benign condition. The lesions typically self-resolve without any residual scarring. If patients have associated pruritus then low- to midpotency topical steroids can be used for symptomatic relief.
 

Dr. Kaushik is with the division of pediatric and adolescent dermatology at Rady Children’s Hospital-San Diego, and Dr. Eichenfield is chief of pediatric and adolescent dermatology at Rady Children’s Hospital-San Diego. He is vice chair of the department of dermatology and professor of dermatology and pediatrics at the University of California, San Diego. There are no conflicts of interest or financial disclosures for Dr. Kaushik or Dr. Eichenfield. Email them at [email protected].

References

1. Gupta D, Mathes E. Lichen Striatus. (Levy ML ed.) 2019: UpToDate.

2. Dermatol Ther. 2018 Nov;31(6):e12713.

3. Int J Dermatol. 2018 Sep;57(9):1118-9.

4. J Dermatol. 2017 Dec;44(12):e355-6.

Lichen striatus (LS) is a common benign skin condition that presents in children between the ages of 5 and 15 years.¹ The rash is typically unilateral and most frequently on the extremities, although it may appear on the face, trunk, or buttocks. The lesions start as pink or skin-colored asymptomatic papules in a linear orientation following the lines of Blaschko. Lesions usually evolve over several weeks, and typically resolve in 6-12 months. There may be residual postinflammatory hypo- or hyperpigmentation which often improves within a few years.

Of note, there are subsets of lichen striatus: Hypopigmented lichen striatus with minimal papules has been termed “lichen striatus albus.” Nail lichen striatus may present as onycholysis or fissuring of nails, present as an isolated finding, or more commonly in association with concurrent affected skin. Nail lichen striatus typically resolves on its own, however there are case reports of improvement with intralesional steroids.²

There is no established etiology for LS. Autoimmune disease, viruses, immunizations, medications, and hypersensitivity reactions have been associated with triggering LS in various case reports, although strength of the associations is low. Children have been reported to have LS following scarlet fever and Candida vulvitis.³ Diagnosis usually is clinical, although biopsy may be helpful for histopathologic confirmation. No work-up for associated infections or conditions is warranted.

The differential for linear papular lesions includes inflammatory linear verrucous epidermal nevus (ILVEN), blaschkitis, or linear morphea. ILVEN is a hamartoma that usually is congenital or presents in early childhood; presents with linear or whorled, hyperkeratotic papules and plaque in similar linear “line of Blaschko” patterns; and represents cutaneous mosaicism. It is often difficult to differentiate between lichen striatus and ILVEN, however lichen striatus is not congenital, and is a self-limited condition. Under dermoscopy (polarized light systems) findings of LS more frequently demonstrate gray granular pigmentation. ILVEN is more frequently associated with cerebriform pattern.⁴ Blaschkitis is a term for a blaschkoid inflammation of the skin that presents with more eczematous findings and histology of spongiosis, unlike the lichenoid findings of LS. It is typically accompanied by noticeable pruritus and broader bands of involved area, and has older age of onset than LS. Linear morphea is a deeper inflammatory process of the dermis or subcutaneous fat, presenting with sclerotic skin, and typically has associated atrophy.

Treatment need not be pursued for lichen striatus because it is a benign condition. The lesions typically self-resolve without any residual scarring. If patients have associated pruritus then low- to midpotency topical steroids can be used for symptomatic relief.
 

Dr. Kaushik is with the division of pediatric and adolescent dermatology at Rady Children’s Hospital-San Diego, and Dr. Eichenfield is chief of pediatric and adolescent dermatology at Rady Children’s Hospital-San Diego. He is vice chair of the department of dermatology and professor of dermatology and pediatrics at the University of California, San Diego. There are no conflicts of interest or financial disclosures for Dr. Kaushik or Dr. Eichenfield. Email them at [email protected].

References

1. Gupta D, Mathes E. Lichen Striatus. (Levy ML ed.) 2019: UpToDate.

2. Dermatol Ther. 2018 Nov;31(6):e12713.

3. Int J Dermatol. 2018 Sep;57(9):1118-9.

4. J Dermatol. 2017 Dec;44(12):e355-6.

Lichen striatus (LS) is a common benign skin condition that presents in children between the ages of 5 and 15 years.¹ The rash is typically unilateral and most frequently on the extremities, although it may appear on the face, trunk, or buttocks. The lesions start as pink or skin-colored asymptomatic papules in a linear orientation following the lines of Blaschko. Lesions usually evolve over several weeks, and typically resolve in 6-12 months. There may be residual postinflammatory hypo- or hyperpigmentation which often improves within a few years.

Of note, there are subsets of lichen striatus: Hypopigmented lichen striatus with minimal papules has been termed “lichen striatus albus.” Nail lichen striatus may present as onycholysis or fissuring of nails, present as an isolated finding, or more commonly in association with concurrent affected skin. Nail lichen striatus typically resolves on its own, however there are case reports of improvement with intralesional steroids.²

There is no established etiology for LS. Autoimmune disease, viruses, immunizations, medications, and hypersensitivity reactions have been associated with triggering LS in various case reports, although strength of the associations is low. Children have been reported to have LS following scarlet fever and Candida vulvitis.³ Diagnosis usually is clinical, although biopsy may be helpful for histopathologic confirmation. No work-up for associated infections or conditions is warranted.

The differential for linear papular lesions includes inflammatory linear verrucous epidermal nevus (ILVEN), blaschkitis, or linear morphea. ILVEN is a hamartoma that usually is congenital or presents in early childhood; presents with linear or whorled, hyperkeratotic papules and plaque in similar linear “line of Blaschko” patterns; and represents cutaneous mosaicism. It is often difficult to differentiate between lichen striatus and ILVEN, however lichen striatus is not congenital, and is a self-limited condition. Under dermoscopy (polarized light systems) findings of LS more frequently demonstrate gray granular pigmentation. ILVEN is more frequently associated with cerebriform pattern.⁴ Blaschkitis is a term for a blaschkoid inflammation of the skin that presents with more eczematous findings and histology of spongiosis, unlike the lichenoid findings of LS. It is typically accompanied by noticeable pruritus and broader bands of involved area, and has older age of onset than LS. Linear morphea is a deeper inflammatory process of the dermis or subcutaneous fat, presenting with sclerotic skin, and typically has associated atrophy.

Treatment need not be pursued for lichen striatus because it is a benign condition. The lesions typically self-resolve without any residual scarring. If patients have associated pruritus then low- to midpotency topical steroids can be used for symptomatic relief.
 

Dr. Kaushik is with the division of pediatric and adolescent dermatology at Rady Children’s Hospital-San Diego, and Dr. Eichenfield is chief of pediatric and adolescent dermatology at Rady Children’s Hospital-San Diego. He is vice chair of the department of dermatology and professor of dermatology and pediatrics at the University of California, San Diego. There are no conflicts of interest or financial disclosures for Dr. Kaushik or Dr. Eichenfield. Email them at [email protected].

References

1. Gupta D, Mathes E. Lichen Striatus. (Levy ML ed.) 2019: UpToDate.

2. Dermatol Ther. 2018 Nov;31(6):e12713.

3. Int J Dermatol. 2018 Sep;57(9):1118-9.

4. J Dermatol. 2017 Dec;44(12):e355-6.

Scleromyxedema
 

Scleromyxedema is an uncommon disease affecting middle-aged adults and typically presents with numerous waxy, monomorphic papules on the head and neck area, characteristically involving the glabella and ears. In some patients, the skin may be intensely pruritic, but this is not a universal finding and varies considerably among patients. In addition to affecting the skin, scleromyxedema has variable multisystem effects on the gastrointestinal tract, and musculoskeletal, pulmonary, cardiovascular, renal, and central nervous systems. The most common symptoms are proximal muscle weakness, dysphagia, and dyspnea on exertion. Scleromyxedema can also be associated with a paraproteinemia, mainly immunoglobulin G-lambda type.

Courtesy Jennifer Maldonado

Scleromyxedema shares some features with other cutaneous diseases, and the main differential diagnosis includes localized scleromyxedema, also known as lichen myxedematosus. Lichen myxedematosus presents with waxy, firm papules and plaques. Systemic involvement and monoclonal gammopathy are characteristically absent. Scleroderma differs given the increase in fibrosis of cutaneous lesions, a higher percentage of Raynaud’s phenomenon, prominent lung disease, and autoantibodies.

On histopathologic review, scleromyxedema is associated with papular and mucin deposition, and increased fibroblast proliferation. The punch biopsy of the exhibited patient demonstrated a dome-shaped dermal nodule composed of fibroblasts in an edematous stroma. An Alcian blue stain highlighted increased mucin in the dermis and S-100 staining highlighted rare cells in the dermis.

The treatment of choice for scleromyxedema varies but includes intravenous immunoglobulins, systemic glucocorticosteroids, thalidomide, or immunosuppressant medications. In this patient, an IgG paraproteinemia was found on serum protein electrophoresis. The patient was evaluated by hematology-oncology, and no underlying myeloproliferative or dysplastic disease was found. The patient was started on intravenous immunoglobulin infusions with near complete resolution of his eruption and arthritic symptoms.

This case and photo were submitted by Jennifer Maldonado, a medical student at Nova Southeastern University, Ft. Lauderdale, Fla., and Kate Oberlin, MD, and Brian Morrison, MD, of the department of dermatology and cutaneous surgery, University of Miami; and Michelle Demory Beckler, PhD, of the College of Osteopathic Medicine and the department of microbiology, College of Medical Sciences, Nova Southeastern University.

Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at mdedge.com/dermatology. To submit a case for possible publication, send an email to [email protected].

Scleromyxedema
 

Scleromyxedema is an uncommon disease affecting middle-aged adults and typically presents with numerous waxy, monomorphic papules on the head and neck area, characteristically involving the glabella and ears. In some patients, the skin may be intensely pruritic, but this is not a universal finding and varies considerably among patients. In addition to affecting the skin, scleromyxedema has variable multisystem effects on the gastrointestinal tract, and musculoskeletal, pulmonary, cardiovascular, renal, and central nervous systems. The most common symptoms are proximal muscle weakness, dysphagia, and dyspnea on exertion. Scleromyxedema can also be associated with a paraproteinemia, mainly immunoglobulin G-lambda type.

Courtesy Jennifer Maldonado

Scleromyxedema shares some features with other cutaneous diseases, and the main differential diagnosis includes localized scleromyxedema, also known as lichen myxedematosus. Lichen myxedematosus presents with waxy, firm papules and plaques. Systemic involvement and monoclonal gammopathy are characteristically absent. Scleroderma differs given the increase in fibrosis of cutaneous lesions, a higher percentage of Raynaud’s phenomenon, prominent lung disease, and autoantibodies.

On histopathologic review, scleromyxedema is associated with papular and mucin deposition, and increased fibroblast proliferation. The punch biopsy of the exhibited patient demonstrated a dome-shaped dermal nodule composed of fibroblasts in an edematous stroma. An Alcian blue stain highlighted increased mucin in the dermis and S-100 staining highlighted rare cells in the dermis.

The treatment of choice for scleromyxedema varies but includes intravenous immunoglobulins, systemic glucocorticosteroids, thalidomide, or immunosuppressant medications. In this patient, an IgG paraproteinemia was found on serum protein electrophoresis. The patient was evaluated by hematology-oncology, and no underlying myeloproliferative or dysplastic disease was found. The patient was started on intravenous immunoglobulin infusions with near complete resolution of his eruption and arthritic symptoms.

This case and photo were submitted by Jennifer Maldonado, a medical student at Nova Southeastern University, Ft. Lauderdale, Fla., and Kate Oberlin, MD, and Brian Morrison, MD, of the department of dermatology and cutaneous surgery, University of Miami; and Michelle Demory Beckler, PhD, of the College of Osteopathic Medicine and the department of microbiology, College of Medical Sciences, Nova Southeastern University.

Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at mdedge.com/dermatology. To submit a case for possible publication, send an email to [email protected].

Scleromyxedema
 

Scleromyxedema is an uncommon disease affecting middle-aged adults and typically presents with numerous waxy, monomorphic papules on the head and neck area, characteristically involving the glabella and ears. In some patients, the skin may be intensely pruritic, but this is not a universal finding and varies considerably among patients. In addition to affecting the skin, scleromyxedema has variable multisystem effects on the gastrointestinal tract, and musculoskeletal, pulmonary, cardiovascular, renal, and central nervous systems. The most common symptoms are proximal muscle weakness, dysphagia, and dyspnea on exertion. Scleromyxedema can also be associated with a paraproteinemia, mainly immunoglobulin G-lambda type.

Courtesy Jennifer Maldonado

Scleromyxedema shares some features with other cutaneous diseases, and the main differential diagnosis includes localized scleromyxedema, also known as lichen myxedematosus. Lichen myxedematosus presents with waxy, firm papules and plaques. Systemic involvement and monoclonal gammopathy are characteristically absent. Scleroderma differs given the increase in fibrosis of cutaneous lesions, a higher percentage of Raynaud’s phenomenon, prominent lung disease, and autoantibodies.

On histopathologic review, scleromyxedema is associated with papular and mucin deposition, and increased fibroblast proliferation. The punch biopsy of the exhibited patient demonstrated a dome-shaped dermal nodule composed of fibroblasts in an edematous stroma. An Alcian blue stain highlighted increased mucin in the dermis and S-100 staining highlighted rare cells in the dermis.

The treatment of choice for scleromyxedema varies but includes intravenous immunoglobulins, systemic glucocorticosteroids, thalidomide, or immunosuppressant medications. In this patient, an IgG paraproteinemia was found on serum protein electrophoresis. The patient was evaluated by hematology-oncology, and no underlying myeloproliferative or dysplastic disease was found. The patient was started on intravenous immunoglobulin infusions with near complete resolution of his eruption and arthritic symptoms.

This case and photo were submitted by Jennifer Maldonado, a medical student at Nova Southeastern University, Ft. Lauderdale, Fla., and Kate Oberlin, MD, and Brian Morrison, MD, of the department of dermatology and cutaneous surgery, University of Miami; and Michelle Demory Beckler, PhD, of the College of Osteopathic Medicine and the department of microbiology, College of Medical Sciences, Nova Southeastern University.

Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at mdedge.com/dermatology. To submit a case for possible publication, send an email to [email protected].

Benign tumors consisting of glomus cells may be subdivided into two types: glomus tumors and glomuvenous malformations or glomangiomas. Glomus cells are modified smooth muscle cells that normally line the Sucquet-Hoyer canal, an arteriovenous fistula that is involved in temperature regulation in the digits.

In adults, solitary glomus tumors are more common and are sporadically inherited. Upper extremities are often affected. In women, lesions more frequently occur on the fingers (especially nail beds). Glomus tumors are firm subcutaneous nodules, often skin colored or bluish in color. Subungual tumors tend to appear bluish under the nail plate. Lesions are extremely tender or painful, with worse pain upon palpation. Occasionally, nontender lesions can be seen.

In children, multiple nontender lesions are called glomangiomas or glomuvenous malformations. They may be sporadic or can be inherited in an autosomal dominant fashion due to a mutation in glomulin on chromosome 1p21-p22. Multiple lesions may be scattered or grouped, often in a segmental distribution. Congenital lesions tend to be large, blue-purple in color with a cobblestone appearance. They are more superficial than venous malformations.

Histologically, a proliferation of blood vessels surrounded by glomus cells is seen. Glomus cells appear as monotonous cells with a dense, round nucleus and abundant pink cytoplasm. Glomus cells can also be appreciated single-filing through pale stroma, resembling strings of black pearls. Glomus cells stain positive for smooth muscle actin and vimentin.

The painful tumor differential diagnosis has been described in the literature by the mnemonic “LEND AN EGG:” leiomyoma, eccrine spiradenoma, neuroma, dermatofibroma, angiolipoma, neurilemmoma, endometrioma, glomus tumor, and granular cell tumor.

The malignant counterpart is glomangiosarcoma, which is a rare tumor. These lesions are often large and deeply located on the extremities. Histologically, sarcomatous areas are mixed with areas of benign glomus tumor.

Surgical excision is the treatment of choice for solitary glomus tumors to provide pain relief. Subungual tumors are more challenging due to their small size, but may be excised as well. Glomuvenous malformations may require different treatment modalities, such as surgery and laser, due to their larger size.

This case and photo were submitted by Dr. Bilu Martin.

Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at www.mdedge.com/dermatology. To submit a case for possible publication, send an email to [email protected].

Benign tumors consisting of glomus cells may be subdivided into two types: glomus tumors and glomuvenous malformations or glomangiomas. Glomus cells are modified smooth muscle cells that normally line the Sucquet-Hoyer canal, an arteriovenous fistula that is involved in temperature regulation in the digits.

In adults, solitary glomus tumors are more common and are sporadically inherited. Upper extremities are often affected. In women, lesions more frequently occur on the fingers (especially nail beds). Glomus tumors are firm subcutaneous nodules, often skin colored or bluish in color. Subungual tumors tend to appear bluish under the nail plate. Lesions are extremely tender or painful, with worse pain upon palpation. Occasionally, nontender lesions can be seen.

In children, multiple nontender lesions are called glomangiomas or glomuvenous malformations. They may be sporadic or can be inherited in an autosomal dominant fashion due to a mutation in glomulin on chromosome 1p21-p22. Multiple lesions may be scattered or grouped, often in a segmental distribution. Congenital lesions tend to be large, blue-purple in color with a cobblestone appearance. They are more superficial than venous malformations.

Histologically, a proliferation of blood vessels surrounded by glomus cells is seen. Glomus cells appear as monotonous cells with a dense, round nucleus and abundant pink cytoplasm. Glomus cells can also be appreciated single-filing through pale stroma, resembling strings of black pearls. Glomus cells stain positive for smooth muscle actin and vimentin.

The painful tumor differential diagnosis has been described in the literature by the mnemonic “LEND AN EGG:” leiomyoma, eccrine spiradenoma, neuroma, dermatofibroma, angiolipoma, neurilemmoma, endometrioma, glomus tumor, and granular cell tumor.

The malignant counterpart is glomangiosarcoma, which is a rare tumor. These lesions are often large and deeply located on the extremities. Histologically, sarcomatous areas are mixed with areas of benign glomus tumor.

Surgical excision is the treatment of choice for solitary glomus tumors to provide pain relief. Subungual tumors are more challenging due to their small size, but may be excised as well. Glomuvenous malformations may require different treatment modalities, such as surgery and laser, due to their larger size.

This case and photo were submitted by Dr. Bilu Martin.

Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at www.mdedge.com/dermatology. To submit a case for possible publication, send an email to [email protected].

Benign tumors consisting of glomus cells may be subdivided into two types: glomus tumors and glomuvenous malformations or glomangiomas. Glomus cells are modified smooth muscle cells that normally line the Sucquet-Hoyer canal, an arteriovenous fistula that is involved in temperature regulation in the digits.

In adults, solitary glomus tumors are more common and are sporadically inherited. Upper extremities are often affected. In women, lesions more frequently occur on the fingers (especially nail beds). Glomus tumors are firm subcutaneous nodules, often skin colored or bluish in color. Subungual tumors tend to appear bluish under the nail plate. Lesions are extremely tender or painful, with worse pain upon palpation. Occasionally, nontender lesions can be seen.

In children, multiple nontender lesions are called glomangiomas or glomuvenous malformations. They may be sporadic or can be inherited in an autosomal dominant fashion due to a mutation in glomulin on chromosome 1p21-p22. Multiple lesions may be scattered or grouped, often in a segmental distribution. Congenital lesions tend to be large, blue-purple in color with a cobblestone appearance. They are more superficial than venous malformations.

Histologically, a proliferation of blood vessels surrounded by glomus cells is seen. Glomus cells appear as monotonous cells with a dense, round nucleus and abundant pink cytoplasm. Glomus cells can also be appreciated single-filing through pale stroma, resembling strings of black pearls. Glomus cells stain positive for smooth muscle actin and vimentin.

The painful tumor differential diagnosis has been described in the literature by the mnemonic “LEND AN EGG:” leiomyoma, eccrine spiradenoma, neuroma, dermatofibroma, angiolipoma, neurilemmoma, endometrioma, glomus tumor, and granular cell tumor.

The malignant counterpart is glomangiosarcoma, which is a rare tumor. These lesions are often large and deeply located on the extremities. Histologically, sarcomatous areas are mixed with areas of benign glomus tumor.

Surgical excision is the treatment of choice for solitary glomus tumors to provide pain relief. Subungual tumors are more challenging due to their small size, but may be excised as well. Glomuvenous malformations may require different treatment modalities, such as surgery and laser, due to their larger size.

This case and photo were submitted by Dr. Bilu Martin.

Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at www.mdedge.com/dermatology. To submit a case for possible publication, send an email to [email protected].

Ichthyosis vulgaris
 

Ichthyoses describe a group of disorders of cornification in which the epidermis differentiates abnormally, leading to generalized scaling of the skin. Ichthyosis is derived from the Greek word for fish, “ichthys.” Ichthyosis vulgaris is the most common of these conditions and often presents in early childhood during the first year of life. It is inherited in an autosomal-dominant pattern. Skin is dry and scaly over the entire body, although the antecubital and popliteal fossa may be uninvolved. The scalp may be involved as well. Atopy and keratosis pilaris may be associated. By adulthood, symptoms tend to abate.

Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at mdedge.com/edermatologynews.com. To submit a case for possible publication, send an email to [email protected].

Ichthyosis vulgaris
 

Ichthyoses describe a group of disorders of cornification in which the epidermis differentiates abnormally, leading to generalized scaling of the skin. Ichthyosis is derived from the Greek word for fish, “ichthys.” Ichthyosis vulgaris is the most common of these conditions and often presents in early childhood during the first year of life. It is inherited in an autosomal-dominant pattern. Skin is dry and scaly over the entire body, although the antecubital and popliteal fossa may be uninvolved. The scalp may be involved as well. Atopy and keratosis pilaris may be associated. By adulthood, symptoms tend to abate.

Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at mdedge.com/edermatologynews.com. To submit a case for possible publication, send an email to [email protected].

Ichthyosis vulgaris
 

Ichthyoses describe a group of disorders of cornification in which the epidermis differentiates abnormally, leading to generalized scaling of the skin. Ichthyosis is derived from the Greek word for fish, “ichthys.” Ichthyosis vulgaris is the most common of these conditions and often presents in early childhood during the first year of life. It is inherited in an autosomal-dominant pattern. Skin is dry and scaly over the entire body, although the antecubital and popliteal fossa may be uninvolved. The scalp may be involved as well. Atopy and keratosis pilaris may be associated. By adulthood, symptoms tend to abate.

Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at mdedge.com/edermatologynews.com. To submit a case for possible publication, send an email to [email protected].

Livedoid vasculopathy, also known as atrophie blanche and PURPLE (painful purpuric ulcers with reticular pattern of the lower extremities) is a rare, chronic vascular condition. It is a vasculopathy rather than a vasculitis as the former is caused by occlusion of blood vessels and the latter results from inflammation of the vessels. Middle-aged women tend to be affected more frequently. Although the exact cause is unclear, systemic diseases, such as hypercoagulable states, may predispose vessels to develop occlusion. Associated disorders include antiphospholipid syndrome, protein C deficiency, factor V mutation, arteriosclerosis, hyperhomocysteinemia, and hepatitis C.

Typically, lesions begin as painful purpura or reticulated macules on the lower extremities that ulcerate and heal very slowly. Ankles, particularly malleoli, are more frequently affected. When they heal, they form painless white stellate scars typical of atrophie blanche. Surrounding erythema, telangiectasias, and sclerosis may be present; livedo reticularis may be seen as well.

Histologically, the epidermis may be atrophic or necrotic. Hyaline thickening of the blood vessel walls is seen. Thrombi may be present. Direct immunofluorescence of perilesional skin may be positive for complement C3 and immunoglobulin (IgM) in dermal blood vessels.

Livedoid vasculopathy can be difficult to treat. Treatment is aimed at reducing clotting and improving blood flow and includes antiplatelet drugs (low-dose aspirin, dipyridamole), anticoagulants, and vasodilating agents (nifedipine). Pentoxifylline two or three times daily may help by altering blood viscosity. A recent literature search reports success in topical dapsone applied to lesions twice daily under occlusion. Leg elevation and compression stockings help healing. Livedoid vasculopathy may have periods of activity and remission.

The case and photo were submitted by Dr. Bilu Martin.

Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at mdedge.com/edermatologynews.com. To submit a case for possible publication, send an email to [email protected]

Livedoid vasculopathy, also known as atrophie blanche and PURPLE (painful purpuric ulcers with reticular pattern of the lower extremities) is a rare, chronic vascular condition. It is a vasculopathy rather than a vasculitis as the former is caused by occlusion of blood vessels and the latter results from inflammation of the vessels. Middle-aged women tend to be affected more frequently. Although the exact cause is unclear, systemic diseases, such as hypercoagulable states, may predispose vessels to develop occlusion. Associated disorders include antiphospholipid syndrome, protein C deficiency, factor V mutation, arteriosclerosis, hyperhomocysteinemia, and hepatitis C.

Typically, lesions begin as painful purpura or reticulated macules on the lower extremities that ulcerate and heal very slowly. Ankles, particularly malleoli, are more frequently affected. When they heal, they form painless white stellate scars typical of atrophie blanche. Surrounding erythema, telangiectasias, and sclerosis may be present; livedo reticularis may be seen as well.

Histologically, the epidermis may be atrophic or necrotic. Hyaline thickening of the blood vessel walls is seen. Thrombi may be present. Direct immunofluorescence of perilesional skin may be positive for complement C3 and immunoglobulin (IgM) in dermal blood vessels.

Livedoid vasculopathy can be difficult to treat. Treatment is aimed at reducing clotting and improving blood flow and includes antiplatelet drugs (low-dose aspirin, dipyridamole), anticoagulants, and vasodilating agents (nifedipine). Pentoxifylline two or three times daily may help by altering blood viscosity. A recent literature search reports success in topical dapsone applied to lesions twice daily under occlusion. Leg elevation and compression stockings help healing. Livedoid vasculopathy may have periods of activity and remission.

The case and photo were submitted by Dr. Bilu Martin.

Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at mdedge.com/edermatologynews.com. To submit a case for possible publication, send an email to [email protected]

Livedoid vasculopathy, also known as atrophie blanche and PURPLE (painful purpuric ulcers with reticular pattern of the lower extremities) is a rare, chronic vascular condition. It is a vasculopathy rather than a vasculitis as the former is caused by occlusion of blood vessels and the latter results from inflammation of the vessels. Middle-aged women tend to be affected more frequently. Although the exact cause is unclear, systemic diseases, such as hypercoagulable states, may predispose vessels to develop occlusion. Associated disorders include antiphospholipid syndrome, protein C deficiency, factor V mutation, arteriosclerosis, hyperhomocysteinemia, and hepatitis C.

Typically, lesions begin as painful purpura or reticulated macules on the lower extremities that ulcerate and heal very slowly. Ankles, particularly malleoli, are more frequently affected. When they heal, they form painless white stellate scars typical of atrophie blanche. Surrounding erythema, telangiectasias, and sclerosis may be present; livedo reticularis may be seen as well.

Histologically, the epidermis may be atrophic or necrotic. Hyaline thickening of the blood vessel walls is seen. Thrombi may be present. Direct immunofluorescence of perilesional skin may be positive for complement C3 and immunoglobulin (IgM) in dermal blood vessels.

Livedoid vasculopathy can be difficult to treat. Treatment is aimed at reducing clotting and improving blood flow and includes antiplatelet drugs (low-dose aspirin, dipyridamole), anticoagulants, and vasodilating agents (nifedipine). Pentoxifylline two or three times daily may help by altering blood viscosity. A recent literature search reports success in topical dapsone applied to lesions twice daily under occlusion. Leg elevation and compression stockings help healing. Livedoid vasculopathy may have periods of activity and remission.

The case and photo were submitted by Dr. Bilu Martin.

Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at mdedge.com/edermatologynews.com. To submit a case for possible publication, send an email to [email protected]