Faculty Bibliography

We present a 57 year-old man presented with generalized hyperhidrosis and widespread, smooth, flesh colored papules on the torso and extremities.Histological examination from multiple biopsies demonstrated morphologic alteration of the eccrine glands with an apocrine phenotype, suggesting eitherapocrine metaplasia or the presence of "apoeccrine glands." The morphologic similarities between eccrine, apocrine, and apoeccrine as they relate to ourpatient's histologic findings are discussed. We consider secondary causes of generalized hyperhidrosis, which may also play a role in this patient's presentation. Treatment and further workup are discussed, whilemanagement of this patient remains in progress.

Lichen myxedematosus is condition characterized by localized areas of dermal deposition of mucin, presenting with firm papules localized to few areas of the body. The condition needs to be excluded from scleromyxedema, which, in addition to the firm papular eruption, has areas of induration and is usually associated with a monoclonal gammopathyand systemic symptoms. We present a 62-year-old woman with a several-year history of asymptomatic, firm papules over the face and arms with no evidence of thyroid disease or a monoclonal gammopathy,which is consistent with a diagnosis of localized lichen myxedematosus, the discrete papular variant. The patient is being treated with a topical calcineurininhibitor.

BACKGROUND: Little is known regarding the clinical practice of immunohistochemistry in the diagnosis of melanoma. We aimed to assess the incidence of immunostain usage by referring pathologists and dermatopathologists in melanoma cases sent for consultative review. As a secondary objective, associations between immunostain use and specific melanoma characteristics were also evaluated. METHODS: This is a retrospective review of consultation reports of referred melanomas at a tertiary academic center in New York, New York from 2001-2015. Univariate regression analysis was performed on melanomas with accompanying immunostains and on characteristics such as Breslow's depth, location, prognostic factors, and morphologic subtypes. Associations between immunostain usage and these characteristics were analyzed using Fisher's exact test. RESULTS: Immunostain use significantly increased over the study period (p<0.001) and was more likely to be associated with melanomas that were thicker (OR=2.5; 1.7-3.6); located on the head and neck (OR=1.6; 1.4-1.9) or acral sites (OR=1.5; 1.1-2.0); had ulceration (OR=2.1; 1.6-2.8), dermal mitoses (OR=1.3; 1.1-1.5), or perineural invasion (OR=3.6; 2.0-6.5); or were of desmoplastic (OR=7.4; 4.5-12), amelanotic (OR=7.1; 3.6-14), or nevoid subtypes (OR=4.0; 1.7-8.9). CONCLUSIONS: Immunostain use in the diagnosis of melanoma has increased significantly in the past 15 years for reasons that remain unclear.

Gardner-Diamond syndrome, which also is knownas autoerythrocyte sensitization disorder, is a raresyndrome of inflammatory, edematous papulesthat evolve into painful ecchymoses on the trunkand lower legs after a period of stress with no priorhistory of trauma. This syndrome usually occurs inwomen with a history of psychiatric disorders, themost common one being depression. Although theexact mechanism of injury is not well understood,it is hypothesized that these patients haveautoantibodies to phosphatidylserine, which is aphospholipid membrane component in erythrocytes.Treatment for this disorder includes symptomatictherapies and psychotropic medications to treat theunderlying psychiatric disorder.

Frontal fibrosing alopecia is a scarring alopecia thatis characterized by recession of the frontotemporalhairline with the frequent loss of eyebrows. Itpredominantly affects postmenopausal womenand only rarely affects men. We report the caseof a 46-year-old man with a ten-year history of anerythematous patch with perifollicular erythemaat the superior aspect of the forehead andfrontotemporal hairline. A skin biopsy specimenshowed a perivascular, lymphocytic infiltrate withperiinfundibular fibrosis. These findings establisheda diagnosis of frontal fibrosing alopecia. Thepathogenesis of this condition is poorly understoodbut may be hormonally-mediated.

Sarcoidosis is a multiorgan inflammatory diseasewith variable clinical presentations and the commonhistopathologic finding of noncaseating granulomas.The etiology of the disease is not known, butevidence suggests both environmental and geneticcontributions to the pathogenesis. Depending onthe severity of cutaneous disease and extent ofextracutaneous involvement, therapies range fromtopical and intralesional glucocorticoids to systemicimmunomodulatory and immunosuppressiveagents. We present the case of a patient withcutaneous sarcoidosis with prominent necrosis onhistopathologic examination in the setting of severepulmonary sarcoidosis.

Lichen planus is an inflammatory skin conditionwith characteristic histopathological findings thathas many clinical variants. Recently, there have been25 cases reported in the literature of lichen planuspigmentosus (LPPi) that preceded the developmentof frontal fibrosing alopecia (FFA), which is a variantof lichen planopilaris (LPP). We review the literatureregarding LPPi, LPP, and FFA and present a case of a40-year-old African American woman with LPPi andLPP.

We present a 58-year-old woman with bipolardisorder and with a longstanding history of yellowbrown,hyperkeratotic papules in a seborrheicdistribution and nail changes. Her father andpaternal grandmother had similar eruptions and alsohad psychiatric disease. Histopathologic examinationshowed acantholysis and dyskeratosis, which wasconsistent with Darier-White disease. Darier-Whitedisease is a rare, hereditary disorder that is inheritedin an autosomal dominant manner and that usuallypresents in adolescence or early adulthood dueto mutations in the SERCA2 calcium pump. Theresultant alterations in calcium homeostasis affectdesmosome assembly and lead to acantholysis andapoptosis, which creates the characteristic eruption.Genetic linkage analysis of affected families showsthat a susceptibility locus for bipolar disorder cosegregateswith genetic markers that are found inthe Darier-White disease region. Treatments, whichinclude topical emollients, mild keratolytics, andtopical or oral retinoids, are aimed at improvingthe appearance of skin, relieving symptoms, andpreventing or treating infectious complications.