Eosinophilic fasciitis is an uncommon connective tissue disorder that affects patients of all ages, resulting in significant morbidity. Systemic corticosteroids can induce remission of disease. However, there is no universally accepted treatment ladder for eosinophilic fasciitis. This case series evaluates treatment efficacy in patients with eosinophilic fasciitis seen at Wake Forest University Department of Dermatology outpatient clinics. Patient charts were screened using ICD-9 diagnosis code 710.9 (unspecified diffuse connective tissue disease) to identify patients with eosinophilic fasciitis (n=10) seen at our institution. Patients were treated for an average 24 months with a combination of methotrexate and prednisone therapy, unless one or both were contraindicated, with each medication tapered conservatively to prevent disease flares. Alternate treatments included mycophenolate mofetil with prednisone, azathioprine with prednisone, prednisone monotherapy, and methotrexate monotherapy. Disease remission off therapy and on low-dose therapy was 66 and 70%, respectively. Our first-line therapy of concomitant methotrexate and prednisone is well-tolerated and effective for managing patients with eosinophilic fasciitis. Our study was limited to cases seen at a single academic institution.
Lichen sclerosus et atrophicus (LSA) is a chronic inflammatory scarring disease with a predilection for the anogenital area; however, 15%-20% of LSA cases are extragenital. The folliculocentric variant is rarely reported and less well understood. The authors report a rare case of extragenital, folliculocentric LSA in a 10-year-old girl. The patient presented to the dermatology clinic for evaluation of an asymptomatic eruption of the arms and legs, with no vaginal or vulvar involvement. Physical examination revealed the presence of numerous 2-4 mm, mostly perifollicular, hypopigmented, slightly atrophic papules and plaques. Many of the lesions had a central keratotic plug. Cutaneous histopathological examination showed features of LSA. Based on clinical and histological findings, folliculocentric extragenital LSA was diagnosed.
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