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74


Spiradenomas

Tran, Kathleen; Defelice, Taylor; Robinson, Maria; Patel, Rishi R; Sanchez, Miguel
We report a 52-year-old man with a one-year history of multiple, firm, skin-colored nodules on the vertex of the scalp. Histopathologic examination was consistent with a spiradenoma, which is a rare, benign adnexal tumor of controversial histogenesis. Multiple spiradenomas may arise in association with Brooke-Spiegler syndrome, which is an autosomal dominant condition of multiple cyclindromas, trichoeptheliomas, and cyclindromas.
PMID: 23286805
ISSN: 1087-2108
CID: 211312

Brooke-Spiegler syndrome

Trufant, Joshua; Robinson, Maria; Patel, Rishi
A 50-year-old woman presented with a long history of multiple, flesh-colored papules and nodules on her central face, neck, and upper shoulders, as well as pink papules on her scalp. They were neither pruritic nor tender, but the patient desired treatment for cosmesis. Histopathologic examination of a representative facial lesion was consistent with a trichoepithelioma and an upper forehead papule was consistent with cylindroma. She reported a positive family history of similar lesions.
PMID: 23286806
ISSN: 1087-2108
CID: 211322

Serratia marcescens folliculitis and concomitant acne vulgaris

Lehrhoff, Stephanie; Yost, John; Robinson, Maria; Patel, Rishi; Sanchez, Miguel
We present a unique case of S. marcescens folliculitis of the trunk in a 46-year-old woman with a history of facial acne vulgaris during her teen years. Her eruption occurred at the time of elective ambulatory surgery when she was treated with pre and post-operative antibiotics. The diagnosis of S. marcescens folliculitis was made on the basis of histopathologic features and tissue culture of a skin biopsy specimen of a pustule after her eruption was unresponsive to conventional treatment for inflammatory acne vulgaris. The history and pathophysiology of gram-negative folliculitis in the setting of acne vulgaris is reviewed.
PMID: 23286809
ISSN: 1087-2108
CID: 211332

Poikilodermatous mycosis fungoides

Bloom, Bradley; Marchbein, Shari; Fischer, Max; Kamino, Hideko; Patel, Rishi; Latkowski, Jo-Ann
Poikilodermatous mycosis fungoides (MF) is a variant of MF, formerly referred to as poikiloderma vasculare atrophicans. The lesions are classically characterized by large plaques of hypopigmentation and hyperpigmentation with atrophy and telangiectases. The plaques may be asymptomatic or mildly pruritic and typically involve the major flexural areas and trunk. Poikilodermatous MF has an early stage (IA-IIA) at diagnosis and a male predominance. Poikilodermatous MF shows an atypical T-cell infiltrate in the papillary dermis with evidence of epidermotropism, epidermal atrophy, dilated blood vessels in the dermis, melanophages, and melanin incontinence. Recent studies suggest a predominance of a CD8+, CD4- immunophenotype. Treatment modalities are similar to classic MF with phototherapy being the most common first-line therapy. Poikilodermatous MF has an excellent prognosis.
PMID: 23286794
ISSN: 1087-2108
CID: 211202

Dissecting cellulitis of the scalp

Mundi, Jyoti P; Marmon, Shoshana; Fischer, Max; Kamino, Hideko; Patel, Rishi; Shapiro, Jerry
Dissecting cellulitis of the scalp is a chronic, relapsing, inflammatory disease of the scalp that results in scarring alopecia. We present a case of a 32-year-old man with recalcitrant disease who is now responding to treatment with isotretinoin. The pathogenesis, clinical presentation, disease associations, and histopathological findings are reviewed. Treatment can be challenging. The literature on medical and surgical therapeutic options is reviewed.
PMID: 23286798
ISSN: 1087-2108
CID: 211242

Eruptive vellus hair cysts

Patel, Utpal; Terushkin, Vitaly; Fischer, Max; Kamino, Hideko; Patel, Rishi
Eruptive vellus hair cyst (EVHC), described initially in 1977, is a benign dermatologic condition that is characterized by the sudden appearance of monomorphic, follicular, asymptomatic, small papules in children and young adults. The diagnosis is based on the histopathologic findings of stratified-squamous epithelium with a granular layer that surrounds a cystic space filled with laminated keratin and a variable number of vellus hair cysts. EVHC can be associated with steatocystoma multiplex. A current hypothesis suggests that EVHC originates from a cystic change at the insertion of the pilosebaceous duct. EVHC is primarily treated for cosmesis with retinoids, surgery, and lasers.
PMID: 23286797
ISSN: 1087-2108
CID: 211232

Verrucous lymphangioma circumscriptum

Terushkin, Vitaly; Marmon, Shoshana; Fischer, Max; Patel, Rishi; Sanchez, Miguel R
A 19-year-old woman with a seven-year history of pruritic, grouped, verrucous papules and plaques on the left arm presented for consultation. The lesion slightly flattened in appearance with topical glucocorticoid treatment. The histopathological features were consistent with lymphangioma circumscriptum. This entity is the most common subtype of lymphangioma and presents with grouped, clear vesicles that may appear pink to purple. However, a rare verrucous type can present a diagnostic challenge. Whereas the definitive treatment option is surgical excision, other treatments, which include sclerotherapy, radiotherapy, and laser therapy, have been reported to cause resolution or improvement. In the future, propranolol may hold promise as a medical therapy for lymphangioma.
PMID: 23286799
ISSN: 1087-2108
CID: 211252

Koebner phenomenon to heat in cutaneous (discoid) lupus erythematosus (lupus ab-igne)

Berger, Emily; Robinson, Maria; Patel, Rishi; Franks, Andrew G Jr
A 63-year-old woman with a ten-year history of systemic lupus erythematosus presented with a three-year history of a large, pruritic lesion that was located on the back and a pruritic lesion that was located on the right thumb. She was a baker by profession for many years during which time she often stood with her back to a hot oven. Physical examination of the lower back showed a large, atrophic, and reticulated plaque with a hyperpigmented border. On the right thumb was a well-demarcated, erythematous plaque with adjacent loss of the medial nail plate on the affected finger. Histopathology of both skin lesions showed cutaneous lupus erythematosus. This patient displays the Koebner phenomenon to heat in cutaneous (discoid) lupus erythematosus. We propose the term lupus ab-igne to describe her skin lesions. There are a few reports of the Koebner phenomenon occurring in patients with lupus erythematosus in response to contact dermatitis, tattoos, zoster, operative scars, scratching, or pressure from clothing. To our knowledge, this is the first report of heat as the physical factor leading to the formation of cutaneous lupus lesions.
PMID: 23286807
ISSN: 1087-2108
CID: 210962

Antiphospholipid-antibody-associated panniculitis

Hunt, Raegan D; Robinson, Maria; Patel, Rishi; Franks, Andrew G Jr
A 60-year-old man presented with intermittent, tender, erythematous nodules on the legs that were associated with mild arthralgias. He was otherwise asymptomatic but reported a history of lupus anticoagulant antibodies that were discovered incidentally on laboratory screening at the approximate time that his lesions first occurred. A biopsy specimen showed a septal and lobular panniculitis with neutrophils, histiocytes, numerous eosinophils, foci of fibrosis, and fat necrosis but no vascular pathology. An elevated activated partial thromboplastin time (PTT), appreciably elevated levels of anti-beta-2 glycoprotein I antibody (IgM and IgG), and moderately elevated levels of anticardiolipin antibody (IgM and IgG) were present. The onset and recurrence of his skin condition coincided with increased antiphospholipid antibody levels and treatment with 81 mg aspirin daily was associated with improvement.
PMID: 23286808
ISSN: 1087-2108
CID: 210952

Anetoderma secondary to antiphospholipid antibodies

Eungdamrong, John; Fischer, Max; Patel, Rishi; Meehan, Shane; Sanchez, Miguel
Anetoderma is an elastolytic disorder that is associated with a number of infectious and autoimmune disorders. We present a case of a patient with generalized anetoderma, who was later found to have positive antinuclear antibodies and antiphospholipid antibodies (APAs). Numerous other cases have been reported in literature and some authors have suggested that anetoderma is a highly specific sign of APAs, with or without other manifestations of systemic lupus erythematosus or antiphospholipid syndrome [14]. Thus, work up for connective-tissue disorders should be considered in any patients who present with this skin finding.
PMID: 23286816
ISSN: 1087-2108
CID: 211402