McEvoy, Marian

Background: Folliculotropic mycosis fungoides (FMF) is a variant of cutaneous T-cell lymphoma that has clinical overlap with a variety of inflammatory follicular unit disorders. However, we describe distinctive presentations of FMF with acneiform features that can be diagnostically challenging, leading to diagnostic delay. Objective: To highlight the importance of histopathologic and immunohistochemical evaluation for diagnostic confirmation of presumed inflammatory follicular unit–based disorders that are unusual in presentation or unresponsive to standard therapies. Methods: A cross-sectional retrospective study of 5 consecutive patients with a histopathologic diagnosis of FMF was conducted. The clinical, histopathologic, immunophenotypic, and molecular genetic features of cases are presented. Results: We describe 5 patients with clinical and histopathologic presentations of FMF masquerading as hidradenitis suppurativa, furunculosis, or acne vulgaris (age range 34–66 years, 4:1 female to male). Clinical morphologies included open and closed comedones, inflammatory pustules, papules and nodules, follicular papules with keratotic plugging, cysts, and scarring involving the face, trunk, and intertriginous areas. All patients failed to respond to standard therapies, including topical and oral antibiotics, topical and oral retinoids, or topical corticosteroids, before receiving the diagnosis of FMF. Lesional skin biopsies showed a perifollicular CD4-positive T-lymphocytic infiltrate with pilotropism, intrafollicular mucin deposition, foreign-body granulomatous inflammation, acute inflammation, and follicular epithelial necrosis. None had concurrent systemic mycosis fungoides. Limitations: Small retrospective cohort study. Conclusion: We present these cases to expand the clinical and histopathologic spectrum of FMF that may strikingly resemble acneiform disorders and to highlight the importance of diagnostic reconsideration with histopathologic evaluation.

Summary Background/Purpose Ultraviolet A1 ( UVA 1) phototherapy has been used for over 15 years in the United States, primarily for the treatment of localized sclerosis and various sclerosing disorders. The objective was to describe use of UVA 1 for dermatoses beyond localized sclerosis at two academic institutions. Methods Data from 83 patients treated with low‐ (20–40 J/cm 2 ), medium‐ (>40–80 J/cm 2 ), and high‐ (>80–120 J/cm 2 ) dose UVA 1 phototherapy was retrospectively analyzed. The mean individual treatment dose (J/cm 2 ), the mean number of sessions, and the mean total dose (J/cm 2 ) were evaluated. Effectiveness was assessed by reviewing clinical examination notes from office visits. Results Good therapeutic efficacy was seen in patients with systemic sclerosis ( SS , 16 patients), graft‐versus‐host disease ( GVHD , 25 patients), and nephrogenic systemic fibrosis ( NSF , 17 patients). A statistically significant a dose–response association was observed in the cases of SS , GVHD and NSF . Likelihood of clinical improvement from UVA 1 phototherapy was very likely for medium‐ and high‐dose regimens in SS , while this level of improvement was only observed in GVHD and NSF patients receiving high‐dose UVA 1. Conclusion UVA 1 phototherapy is effective and safe in the treatment of GVHD , NSF , SS , and mast cell disorders. High‐dose regimens appear to be more effective than medium‐ and low‐dose regimens for NSF and GVHD , while medium‐ and high‐dose regimens outperform low‐dose UVA 1 in SS .