Faculty Bibliography

Steatocystoma multiplex is a rare condition that is characterized by cutaneous cysts and may be inherited in an autosomal dominant manner or may occur sporadically. The pathogenesis is hypothesized to involve mutations in the keratin 17 gene. There are no internal manifestations. The lesions are usually asymptomatic. However, a suppurative variant exists in which the lesions become inflamed and suppurative after minor trauma. Treatments include cryosurgery, aspiration, surgical excision, laser therapy, and modified surgical incision techniques. This report presents a case of steatocystoma multiplex, the suppurative variant, in a 26-year-old woman with involvement of rare locations on the buttocks, groin, and extremities.

Granuloma annulare (GA) is a benign, granulomatous disease with several clinical manifestations, which include localized, generalized, perforating, subcutaneous, patch, papular, and linear forms. We report a case of papular GA of the dorsal aspects of the hands that arose after repeated, direct trauma to the site of subsequent involvement. Although multiple etiologies for GA have been proposed, which include ultraviolet light, arthropod bites, trauma, tuberculin skin tests, viral infections, and PUVA photochemotherapy, the underlying pathogenesis of the disorder remains unclear. However, owing to the key histopathologic findings of focal collagen and elastic fiber degeneration and mucin deosition in GA, it is not surprising that cutaneous trauma may have played a role in connective tissue injury, subsequent degeneration, and the production of a granulomatous response with increased mucin deposition.

We present a 67-year-old man with an ulcerated, indurated plaque on the right mid back with a presumed diagnosis of morphea that was complicated by an allergic contact dermatitis. Further clinical and histopathologic data elucidated the diagnosis of fluoroscopy-induced radiation dermatitis. We present a brief review of the common locations, clinical characteristics, pathophysiology, and management options for fluoroscopy-induced radiation dermatitis.

We present a case of acrokeratosis verruciformis of Hopf in a 58-year-old woman with multiple, skin-colored, flat-topped, hyperkeratotic papules on the dorsal aspects of the hands and feet. Her lesions appeared at age 13 and her son developed similar lesions at about the same age. Acrokeratosis verruciformis of Hopf is a rare genodermatosis, which is distinct from keratosis follicularis; sporadic cases have also been described. Management options include cryotherapy, keratolytics, superficial ablation with CO2 or Nd:YAG lasers, topical retinoids, and acitretin.

Patients with human immunodeficiency virus are known to have an increased risk for development of cutaneous malignant conditions. We present a 55-year-old man with a history of an isolated squamous-cell carcinoma five years prior, who presented with 27 cutaneous lesions, which had developed over the course of the preceding year. This occurred in the context of a new diagnosis of human immunodeficiency virus (HIV) infection and acquired immune deficiency syndrome (AIDS). The initiation of anti-retroviral therapy and subsequent restoration of CD4 counts was associated with an increase in the number of lesions. Histopathologic examination of representative tumors showed basal-cell carcinomas, which lead to the diagnosis of eruptive basal-cell carcinomas in the setting of HIV-related immunosuppression. The incidence and epidemiology of malignant conditions that are related and non-related to AIDS are reviewed as well as discussion of the current literature regarding presentation, treatment, and prevention of non-melanoma skin cancers in patients with HIV infection.

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.

Purpura annularis telangiectoides of Majocchi (PATM) is a clinical variant of pigmented purpuric dermatoses. It is characterized by asymptomatic or mildly pruritic, erythematous, annular patches that vary in size. The patches exhibit central clearing and peripheral cayenne pepper petechiae. It classically occurs on the lower legs but may be more generalized. Histopathologic features include a lymphocytic capillaritis with subsequent capillary leakage and extravasated erythrocytes, which is typical of all pigmented purpuric dermatoses. The pathogenesis of PATM is unknown, but it may uncommonly be associated with underlying diseases such as hematologic disorders and rarely cutaneous T-cell lymphoma. There is no standard effective treatment.

A 25-year-old man presented with a 13-year history of an erythematous, papular eruption of his face and trunk, which was treated in the past as acne and psoriasis with isotretinoin and methotrexate, respectively. Histopathologic examination demonstrated an infiltrate of Langerhans cells, which was consistent with Langerhans cell histiocytosis. The epidemiology, clinical presentation, and treatment options of this disease are reviewed.

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.

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.