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Systemic lupus erythematosus-associated neutrophilic dermatosis with palmoplantar involvement
Quatrano, Nicola A; Criscito, Maressa C; Femia, Alisa N; Brinster, Nooshin K
PMCID:4992001
PMID: 27570816
ISSN: 2352-5126
CID: 2227302
Primary cutaneous nodular amyloidosis in association with Sjogren's syndrome
Mazori, Daniel R; Femia, Alisa N
PMID: 26494586
ISSN: 1778-7254
CID: 1810612
Epidemiology and treatment of postirradiation morphea: A retrospective analysis from a large tertiary care center [Meeting Abstract]
Fruchter, R; Mazori, D R; Femia, A N
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
EMBASE:72275333
ISSN: 0190-9622
CID: 2151272
Characteristics and treatment of adult-onset linear morphea: A retrospective cohort study of 61 patients at 3 tertiary care centers
Mazori, Daniel R; Wright, Natalie A; Patel, Mital; Liu, Stephanie W; Ramachandran, Sarika M; Franks, Andrew G Jr; Vleugels, Ruth Ann; Femia, Alisa N
PMID: 26892661
ISSN: 1097-6787
CID: 1949872
Epidemiology and Treatment of Eosinophilic Fasciitis: An Analysis of 63 Patients From 3 Tertiary Care Centers
Wright, Natalie A; Mazori, Daniel R; Patel, Mital; Merola, Joseph F; Femia, Alisa N; Vleugels, Ruth Ann
PMID: 26559760
ISSN: 2168-6084
CID: 1834802
Development and Pilot Testing of the Cutaneous Lupus Screening Tool
Lam, Christina; Liu, Stephanie W; Townsend, Henry B; Femia, Alisa N; Qureshi, Abrar A; Vleugels, Ruth Ann
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.
PMID: 26509244
ISSN: 2168-6084
CID: 2029322
Epidemiology and treatment of refractory cutaneous lupus erythematosus [Meeting Abstract]
Fruchter, R; Kurtzman, D; Lin, J; Femia, AN; Vleugels, R
ISI:000380028800176
ISSN: 1523-1747
CID: 2216112
Focal full-thickness epidermal necrosis in association with adalimumab in a patient with ulcerative colitis [Meeting Abstract]
Fruchter, R; Lopatin, S; Malter, LB; Femia, AN
ISI:000380028800142
ISSN: 1523-1747
CID: 2216182
Mycobacterium avium complex infection
Vedvyas, Chetan; Shvartsbeyn, Marianna; Brinster, Nooshin; Femia, Alisa
We report the case of a 30-year-old woman with a three-year history of papules, nodules, and multiple ulcers of the left lower extremity. A skin biopsy specimen showed dermal fibrosis and patchy granulomatous inflammation in the upper-to-mid dermis that was associated with a perivascular, lymphohistiocytic infiltrate along with granulomas and necrosis in the subcutaneous fat. Tissue culture showed Mycobacterium avium complex infection. Clinical manifestations of cutaneous nontuberculous mycobacterial infections are discussed as well as their potential association with pedicures.
PMID: 26990341
ISSN: 1087-2108
CID: 2051342
Primary diffuse macular amyloidosis
Kanchanapoomi, Melissa; Rosenstein, Rachel; Shvartsbeyn, Marianna; Meehan, Shane A; Dolly, Naomi; Femia, Alisa
We present a 53-year-old woman with diffuse macular amyloidosis. We discuss the clinical manifestations, pathophysiologic mechanisms, and associations of cutaneous macular amyloidosis.
PMID: 26990346
ISSN: 1087-2108
CID: 2051382