Faculty Bibliography

Perniosis is a localized, inflammatory reaction that ischaracterized by erythematous papules and nodulesthat often are located on the acral surfaces in youngwomen. The lesions of perniosis are thought to bedue to cold-induced vasoconstriction that leadsto hypoxemia and inflammation of the vessel wall.Histopathologic and laboratory studies are indicatedfor patients with suspected perniosis to distinguishbetween idiopathic perniosis and secondaryperniosis. Treatment includes adequate heating andclothing, proper food intake, nifedipine, ultravioletA1 phototherapy, topical glucocorticoids, andvasodilators.

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.

We present a 42-year-old transgender womanwith woody induration over her buttocks andlower extremities as well as persistent ulcers of thebuttocks. The lesions developed ten years prior to herpresentation and approximately five years after shereceived illegal silicone injections to her buttocks.Histopathologic examination was consistent witha silicone granuloma. Silicone granuloma is a notan uncommon side effect of silicone injections andmore often occurs when the filler is administeredby non-physician practitioners, as is the case in thispatient. Ulcerative silicone granulomas, however,rarely are reported. In this case, the patient'shemodialysis treatments, which required longperiods of weight bearing on her buttocks, may havepredisposed her to ulcers in this area, and the ulcersmay have been in part due to poor vascular supplyas well as physical pressure. Treatment of this patientis relatively challenging, owing to her multiplecomorbidities that include end-stage renal diseaseand congestive heart failure.

Chronic arsenic exposure is known to inducepunctate keratoses with an increased risk ofprimary squamous-cell carcinoma. Drinking wateris currently the major source of arsenic exposureworldwide and is considered one of the mostsubstantial environmental carcinogens. We describethe case of a 61-year-old Hungarian woman withscattered, acral, hyperkeratotic papules and a historyof five palmoplantar squamous-cell carcinomasas well as two other extremity non-melanomaskin cancers. Prior to immigration, she had livedin a county of Southern Hungary that is known tohave elevated concentrations of inorganic arsenicin the drinking water above the World HealthOrganization's current maximum threshold forsafety. To date, this report is the first to describethe phenomenon of palmoplantar squamouscellcarcinomas in a patient from this region andunderscores the importance of vigilant screening inthose individuals who have spent substantial time inhigh-risk regions internationally and domestically.

Postirradiation morphea (PIM) is a rare and potentially disabling cutaneous complication of radiotherapy, affecting approximately 2 in every 1000 patients treated with radiotherapy. Only 67 cases are reported in the literature to date, and treatment data is reported in only roughly half of cases. The objective of this retrospective cohort study was to characterize the nature and treatment of patients with PIM. Using two medical record databases at New York University, we reviewed all charts with ICD-9 code 701.0 to identify all patients with PIM at a large tertiary care center from 2007 to 2015. Nine patients with PIM were identified. All were female and had a history of radiotherapy for breast cancer. Mean age of onset was 58 years. Where data were available, 40% (n = 2) developed PIM within 1 year of first radiation exposure, 40% (n= 2) within 1-5 years, and 20% (n =1) after 5 years. Sixty seven percent (n = 6) had PIM extending beyond the irradiation field to sites including the contralateral breast, abdomen, back, groin, and extremities. Fifty-six percent (n = 5) were asymptomatic, 33% (n = 3) had pruritus, and 11% (n = 1) had pain. One patient had a history of radiation dermatitis. None had a history of connective tissue disease. All were referred to a dermatologist. Where treatment data were available, all (n = 8) were treated with topical agents including dapsone, corticosteroids, calcipotriol, azeilaic acid, and tacrolimus. Only 1 patient had substantial improvement with topical therapy alone (dapsone); the remainder had no or partial improvement. The majority of patients (n=5) required systemic agents including methotrexate, etanercept, doxycycline, colchicine, calcitriol, pentoxifylline, and/or phototherapy. MTX, although only utilized in 2 patients, led to substantial improvement in both. Overall, PIM was treatment refractory with 50% (n = 4) of patients requiring trials of three or more treatment regimens and the majority (n = 5) requiring treatment for greater than six months. In conclusion, this study represents the largest PIM cohort since 1989 and the largest study to date to report on PIM therapy. This study highlights the recalcitrant and potentially chronic nature of PIM, as well as the diverse nature of PIM in terms of symptoms, latency period from radiotherapy, and areas of involvement. Additional study is needed to further characterize PIM

IMPORTANCE: Patients with cutaneous lupus erythematosus (CLE) experience significant morbidity and poor quality of life. In the absence of a dermatologist's examination, no reliable tool exists to confirm whether a patient has CLE for use in epidemiologic studies. OBJECTIVE: To determine whether the Cutaneous Lupus Screening (CLUSE) tool can detect cases of CLE by measuring its performance in individuals with dermatologist-diagnosed CLE compared with individuals without CLE. DESIGN, SETTING, AND PARTICIPANTS: The CLUSE tool is a novel, self-administered questionnaire with 15 closed-ended questions derived from the Delphi method. It includes features of disease validation for CLE as well as its most common phenotypes. This pilot study was administered during a 1-year period (July 1, 2011, to June 30, 2012) in outpatient dermatology clinics at an academic medical center. Data analysis was performed July 1, 2012, to November 30, 2013. Participants were individuals 18 years or older who had a definitive diagnosis of CLE or any other non-CLE dermatologic condition as established by a board-certified dermatologist. Eligible patients were recruited consecutively, and no individual approached declined to participate. MAIN OUTCOMES AND MEASURES: Sensitivity and specificity of the individual questions from the CLUSE tool in predicting CLE, comparisons between summary scores for the dichotomous questions between the CLE cases and non-CLE controls, and 9 scoring algorithms that assign a diagnosis of CLE and its subtypes depending on an individual's response to each question. RESULTS: A total of 133 patients were given the CLUSE tool; 16 participants were excluded. Responses from 117 individuals were collected for analysis and included 24 CLE cases and 93 non-CLE cases. In the 117 questionnaires analyzed, mean (SD) and median (interquartile range) CLUSE scores differed in the CLE (5.6 [2.1] and 5.5 [3-10], respectively) vs non-CLE (0.96 [1.6] and 0 [0-7], respectively) groups (all P < .001). Of the 9 algorithms, algorithm 9, used for diagnosing CLE regardless of subtype, demonstrated the highest sensitivity (87.5%) and high specificity (96.8%). CONCLUSIONS AND RELEVANCE: A combination of questions and representative photographs can ascertain cases of CLE with high sensitivity and specificity. The CLUSE tool is a brief, self-administered questionnaire with low respondent burden used for the identification of CLE. In the future, this questionnaire will be administered to large, established patient databases to gather epidemiologic data on this disease.