Baykal Can, Atci Tugba, Ozturk Sari Sule, Polat Ekinci Algun, Buyukbabani Nesimi
Department of Dermatology and Venereology, Istanbul Medical Faculty, Istanbul University, Istanbul, Turkey.
Department of Pathology, Istanbul Medical Faculty, Istanbul University, Istanbul, Turkey.
J Dtsch Dermatol Ges. 2017 Mar;15(3):289-299. doi: 10.1111/ddg.12976. Epub 2017 Feb 27.
A rare variant of mycosis fungoides (MF), folliculotropic MF (FMF) is characterized by a broad clinical spectrum that primarily includes follicle-based lesions but also many atypical clinical manifestations. The objective of the present study was to conduct a clinical analysis of patients with FMF, with a particular focus on highlighting underrecognized dermatological features.
Overall, 27 FMF patients enrolled in our department';s MF registry, which includes 572 patients, were retrospectively reevaluated with regard to demographics, clinical features, treatment modalities, follow-up, and outcomes.
Besides the well-known clinical features of FMF, we found lichen spinulosus-like lesions in association with hypopigmentation (n = 3) and alopecia (n = 2), infiltrated/elevated erythematous facial plaques initially considered to be lupus tumidus (n = 2), pseudotumoral lesions clinically mimicking tumor-stage MF (n = 1), persistent excoriations (n = 1) and erythematous facial papules mimicking rosacea (n = 1), as well as white dome-shaped asymptomatic papules/nodules filled with mucin (on histology) (n = 2) that overlay other disease-related lesions. Various therapeutic methods were used with variable results. Eight (29.6 %) patients had late-stage disease.
Awareness of underrecognized clinical manifestations may be key to reducing delayed diagnosis of this aggressive MF variant.
毛囊性蕈样肉芽肿（FMF）是蕈样肉芽肿（MF）的一种罕见变异型，其临床谱广泛，主要包括基于毛囊的病变，但也有许多非典型临床表现。本研究的目的是对FMF患者进行临床分析，特别关注突出那些未被充分认识的皮肤特征。
总体而言，对纳入我科MF登记册（共572例患者）的27例FMF患者进行回顾性重新评估，内容包括人口统计学、临床特征、治疗方式、随访及结局。
除了FMF的众所周知的临床特征外，我们还发现了与色素减退（3例）和脱发（2例）相关的棘状苔藓样病变、最初被认为是肿胀性狼疮的浸润性/隆起性面部红斑（2例）、临床上模仿肿瘤期MF的假肿瘤性病变（1例）、持续性搔抓（1例）以及模仿酒渣鼻的面部红斑丘疹（1例），还有覆盖在其他疾病相关病变之上的、组织学上显示充满粘蛋白的白色圆顶状无症状丘疹/结节（2例）。采用了各种治疗方法，结果各异。8例（29.6%）患者处于疾病晚期。
认识到未被充分认识的临床表现可能是减少这种侵袭性MF变异型延迟诊断的关键。
