Faculty Bibliography

White fibrous papulosis of the neck is a rare diagnosisthat typically presents with white, 2-to-3-mmpapules on the posterior aspect of the neck. Wepresent an elderly woman with diffuse involvementon her abdomen and back.

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.

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.

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.

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.

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.

BACKGROUND: While infrequent, nodal metastases in cutaneous squamous cell carcinoma (cSCC) can result in death from disease. Identification of those at risk for metastases is key to improved prognostication and treatment. OBJECTIVE: To review metastatic cSCC at the study institution. METHODS AND MATERIALS: Sixteen patients with metastatic cSCC were identified at the New York University Dermatologic Associates and Cancer Associates from 1998 to 2013. Patients were staged with American Joint Committee on Cancer (AJCC) and modified Brigham and Women's Hospital (BWH) criteria and compared to 32 control subjects. RESULTS: Seven of 16 patients were identified as Stage T2 by AJCC criteria and Stage T2b by BWH criteria; two patients were on Stage T1, three patients were on more advanced T stages, and four patients lacked primary tumor data. Five patients had hematologic malignancy, and one patient had a solid-organ transplant. CONCLUSION: The modified BWH criteria aims to better prognosticate the large group of T2 AJCC tumors, resulting in the majority of mortality. In the experience of the authors, the majority of patients with metastatic disease were on T2, stratifying to stage T2b by BWH criteria, or more advanced T stages. The findings of this study support BWH stratification of T2 tumors and also indicate that hematologic malignancy is a significant comorbidity associated with a poor outcome.

Vegetative pyoderma gangrenosum is a rare, superficial variant of pyoderma gangrenosum that is more commonly found on the trunk as single or multiple, non-painful lesions. There is typically no associated underlying systemic disease. Compared to classic pyoderma gangrenosum, vegetative lesions are more likely to heal without the use of systemic glucocorticoids, although up to 39% of patients required a short course of prednisone in a review of 46 cases. Treatments for vegetative pyoderma gangrenosum include topical and intralesional glucocorticoids, minocycline or doxycycline, dapsone, colchicine, and, rarely, alternative steroid-sparing immunosuppressants. We present a case of multiple vegetative pyoderma gangrenosum lesions arising in prior surgical sites in a patient found to have IgA monoclonal gammopathy and abnormal urinary protein electrophoresis.