Faculty Bibliography

We report the case of a 60-year-old man with penile-scrotal extramammary Paget disease (EMPD). The patient initially underwent Mohs micrographic surgery, but the margins remained positive after several sections; multiple scouting punch biopsies used to define the extent of the tumor were also positive. Because of concerns about functional impairment and cosmesis associated with wide local excision, the patient instead chose treatment with topical 5 percent imiquimod cream as a cytoreductive and margin-defining treatment. Owing to the association between EMPD and underlying malignant conditions, a thorough metastatic evaluation is necessary, particularly to rule out genitourinary cancer in the setting of penile-scrotal EMPD. Management of EMPD is complicated by the multifocal, non-contiguous nature of the disease and the presence of clinically occult extensions. As a result, recurrence rates after surgery are high. Several non-surgical modalities have been used to treat EMPD, which include radiotherapy, topical imiquimod, topical 5-fluorouracil, topical bleomycin, photodynamic therapy, CO2 laser ablation, and topical retinoids. Systemic chemotherapy also has been used to treat advanced EMPD. However, because EMPD is so uncommon, clinical trials comparing the various methods of treatment are lacking. Regardless of the mode of treatment, long-term follow up is essential, given the high rate of recurrence.

Graham-Little-Piccardi-Lassueur syndrome (GLPLS) traditionally describes patients with a triad of a multifocal cicatricial alopecia of the scalp, noncicatricial alopecia of the axillae and groin, and a lichenoid follicular eruption. The entity has caused much controversy with respect to both etiology and clinical definition. We present a patient with lichen planopilaris, lichen planus pigmentosus, and nonscarring alopecia of the genitals, who is reminiscent of GLPLS. Recent work shows evidence for autoimmunity in GLPLS. Further elucidation of underlying mechanisms can improve categorization and treatment options in this rare and controversial syndrome.

JA Stein was supported by the Irwin I. Lubowe Fellowship in Dermatology

The authors have indicated no significant interest with commercial supporters

A 24-year-old woman presented with hypopigmented papules of the abdomen that had been present for four years without a family history of similar cutaneous findings or associated medical problems. Histopathologic features confirmed the diagnosis of a connective-tissue nevus that was composed of collagen. Eruptive collagenomas are a rare form of acquired collagenomas, which are characterized by the sudden appearance of asymptomatic papules and nodules on the lower trunk and extremities; the lesions are composed of haphazardly arranged collagen fibers. The pathogenesis is unknown, lesions are persistent, and therapeutic options have not been reported

We describe a 73-year-old woman with a long-standing history of annular, hyperkeratotic papules that began on the palms and soles and gradually spread to her trunk, extremities, and face. The clinical presentation and biopsy findings were consistent with PPPD, which is a rare subtype of porokeratosis that begins on the palms and soles and gradually spreads to the trunk and extremities. Owing to the risk of malignant degeneration in porokeratosis, patients should be closely monitored with total body skin examinations. There is no definitive treatment for PPPD. Oral retinoids are sometimes helpful although relapses are common after discontinuation of therapy

Pemphigoid gestationis is a rare autoimmune blistering disease of pregnancy. It is characterized by pruritic, urticarial plaques with the development of tense vesicles and bullae within the lesions. Pemphigoid gestationis has been associated with premature delivery, small-for-gestational-age infants. Recurrences with subsequent pregnancies are often more severe. Oral glucocorticoids are the mainstay of therapy. Differentiation of pemphigoid gestationis from pruritic urticarial papules and plaques of pregnancy is essential because management and outcomes differ. In instances in which clinical diagnosis is difficult, direct immunofluorescence tests, immunoblots, or ELISA studies of anti-basement-membrane zone antibodies are useful in establishing the diagnosis

Carcinoid tumors are derived from neuroendocrine cells, and are most frequently found in the gastrointestinal tract and bronchopulmonary system. Cutaneous involvement of carcinoid tumors is relatively rare, with isolated case reports in the literature. We detail a patient with stage IV pulmonary atypical carcinoid tumor with skin metastasis. This case is unusual because the patient did not have any erythema or induration of the scalp, only a complaint of pain. On biopsy, the tumor cells had cytologic features of a carcinoid tumor, but were arranged as infiltrating cords, small aggregates and single units, rather than the organoid or trabecular pattern seen in the primary tumor. Further, along with neuroendocrine markers, the tumor cells had the staining pattern of cytokeratin 7+/cytokeratin 20-/thyroid transcription factor-1+, supporting a carcinoid tumor of lung origin. Thus, this case of skin metastasis from an atypical pulmonary carcinoid tumor illustrates a unique clinical and histologic presentation

A 79-year-old woman presented with a four-year history of generalized, erythematous, indurated plaques on the malar areas, back, and extremities. The lesions had been recalcitrant in the past to topical glucocorticoid therapy. A skin biopsy specimen was diagnostic of discoid lupus erythematosus (DLE). A minority of patients with DLE progress to develop systemic lupus erythematosus although generalized DLE is more frequently associated with systemic involvement than is limited disease. Standard therapy of cutaneous lupus includes broad spectrum sunscreens, topical and intralesional glucocorticoids, and antimalarial agents