Faculty Bibliography

A 53-year-old woman presented with a six-month history of non-pruritic, erythematous papules and papular-plaques that were localized to the anterior and lateral aspects of the neck. A biopsy specimen showed elastolysis and granuloma formation, which were consistent with a diagnosis of annular elastolytic giant-cell granuloma. This is one of the few reported cases of this entity that consists predominantly of papular lesions rather than annular plaques.

Disseminated superficial actinic porokeratosis, which was described in 1966, is characterized by small, atrophic patches with distinctive keratin rims that occur on sun-exposed areas of the extremities, shoulders, and back. The diagnosis is based on the histopathologic finding of a cornoid lamella, absence of a granular layer, and often a thin epidermis. It is associated with exposure to ultraviolet radiation. Gene studies suggest a pathway defect in which several mutations in keratinocyte proliferation and differentiation lead to development of porokeratosis.

A 41-year-old woman with a prior diagnosis of lupus erythematous presented with a five-year history of small, erythematous, flesh-colored papules and nodules that coalesced into symmetrically-distributed plaques on her upper back. A biopsy specimen showed an interstitial, granulomatous mixed-cell dermatitis with eosinophils. These clinicopathologic findings are consistent with a diagnosis of lupus erythematous-associated interstitial granulomatous dermatitis.

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.

We present a 58-year-old Jamaican man with an eight-month history of an isolated, vegetative, eroded, and crusted plaque on the posterior aspect of the scalp. One month prior to referral to the Charles C. Harris Skin and Cancer Pavilion he developed a few, scattered erosions on the extremities. A biopsy specimen showed suprabasal acantholysis along with deposition of C3 and IgG on a direct immunofluorescence test. Localized pemphigus is a rare variant of pemphigus vulgaris. Localized pemphigus presents with a localized plaque that remains localized for an extended period of time without generalized bullae formation. Our patient represents an unusual case of localized pemphigus of the scalp with vegetative features.

Sarcoidosis is a multi-system, granulomatous disease, which affects the skin in approximately 20 to 30 percent of cases. Recognition of cutaneous sarcoidosis can be challenging because of the wide range of skin lesion morphologies. Ulcerative sarcoidosis is uncommon. We present a 35-year-old woman with pretibial ulcerative sarcoidosis, indurated tattoos, and hilar lymphadenopathy.

The leishmaniases are a group of diseases caused by species of Leishmania and transmitted by the bite of the female sandfly. The major clinical forms include localized or disseminated cutaneous, mucocutaneous, and visceral disease. Localized cutaneous leishmaniasis is most frequently caused by L. major and L. tropica in the Old World and by L. braziliensis, L. mexicana, and related species in the New World. L. donovani generally causes visceral disease in the Old World. We describe a case of disseminated cutaneous leishmaniasis caused by L. donovani in a traveller returning to the United States from Italy. Dermatologists should be aware of the clinical manifestations of leishmaniasis.

Fox-Fordyce disease (FFD) is a rare inflammatory disorder that affects the apocrine sweat glands. Clinically, lesions are equidistant, smooth, uniform, firm, folliculocentric papules, which can range in color from flesh-colored to red-brown to slightly yellow. Whereas the axillae are most commonly involved, FFD also can involve the anogenital and periareolar areas, lips, umbilicus, sternum, perineum, and upper medial aspects of the thighs. The underlying etiology of FFD remains unclear although epidemiologic data support a hormonal component because women are more commonly affected than men. Moreover, symptoms initially present after the onset of puberty, flare perimenstrually, and often resolve during pregnancy and after menopause. Histopathologic findings include the obstruction of the apocrine duct by a hyperkeratotic plug in the follicular infundibulum, which is believed to represent the primary pathophysiologic process; subsequent ductal rupture and resulting inflammatory response produce the typical clinical picture. Treatment of FFD is difficult because no one agent has proven particularly effective. Topical and interlesional glucocorticoids are often considered the first-line pharmacologic agents, although use is often limited by concerns for atrophy. Other agents that have shown some success include topical and systemic retinoids, topical clindamycin, topical pimecrolimus cream, benzoyl peroxide, and oral contraceptives. For medication-refractory cases, mechanical destruction or removal of the apocrine glands has been efficacious in small case series.

Morbihan disease, which consists of solid facial edema, is a rare complication of rosacea, a common cutaneous disorder in middle-aged individuals. The characteristic features of Morbihan disease are its chronic course, typical clinical picture, lack of specific laboratory and histopathologic findings, and refractoriness to therapeutic measures. Since its initial description in 1957, only a small number of cases have been reported in the dermatologic literature. We report a 54-year-old man who developed a two-year duration of erythema and edema that affects the upper and mid face, with accentuation in the periorbital region. Patch tests excluded an allergic contact dermatitis and histopathologic investigation showed small, nodular clusters of epithelioid cells in the dermis that were consistent with sarcoidal granulomata. A diagnosis of Morbihan disease was made owing to the combination of clinical and histopathologic findings. Therapeutic options for the disease remain unsatisfactory and treatments reported in the literature include systemic glucocorticoids, oral tetracyclines, thalidomide, isotretinoin, ketotifen, and clofazimine. Our patient failed a six-to-seven months course of minocycline prior to presentation and has since experienced improvement on gradually-increasing doses of isotretinoin.

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.