Rajalakshmi T, Inchara Y K, Antony Meryl
Departments of Pathology, St. John's Medical College and Hospital, Bangalore - 560 034, India.
Indian J Med Paediatr Oncol. 2009 Jul;30(3):108-12. doi: 10.4103/0971-5851.64257.
Follicular Mycosis Fungoides (FMF) is an under-recognized disease in India. Its clinical mimics include Hansen's disease and Sarcoidosis.
To describe the clinical and pathological features of FMF.
All cases of FMF between January and December 2007 were retrieved. Cases of conventional epidermotropic MF with a minor follicular component were excluded. Slides were reviewed by two observers. The following criteria were assessed: degree and density of folliculotropism of lymphocytes, location of folliculotropism (infundibular / isthmic / bulbar), follicular mucin, eosinophils, granulomas, and conventional epidermotropism. Each feature was assigned a semi-quantitative grade.
There were four cases of FMF, with an equal gender distribution and a mean age of 17.5 years. All lesions were on the face. They presented as: hypopigmented patches (2) and erythematous plaques (2). Alopecia was seen in two cases. The clinical diagnosis was Hansen's disease in all four, with a differential of Alopecia mucinosa / Sarcoidosis in two cases.The histological features seen were: disproportionate folliculotropism, lymphocyte tagging with haloes, follicular mucin, and nucleomegaly / convolution in all four cases, prominent eosinophils (2), epithelioid granulomas (1), eccrine infiltration (4), parakeratosis at the follicular ostia (2), and sebaceotropism (1). The infiltrate was bulbar (4) and isthmic (2). The rest of the epidermis showed no hint of conventional MF.
The preferential features for FMF were involvement of face, dominant folliculotropism, nuclear atypia and convolution, and follicular mucin. Presence of granulomas and eosinophils necessitated exclusion of infectious causes. The absence of findings of MF in the rest of the epidermis should not deter pathologists from rendering this diagnosis.
毛囊蕈样肉芽肿（FMF）在印度是一种未得到充分认识的疾病。其临床相似疾病包括麻风病和结节病。
描述FMF的临床和病理特征。
检索2007年1月至12月期间所有FMF病例。排除伴有少量毛囊成分的传统亲表皮性蕈样肉芽肿病例。两名观察者对切片进行复查。评估以下标准：淋巴细胞对毛囊的亲嗜程度和密度、亲嗜毛囊的位置（漏斗部/峡部/球部）、毛囊黏蛋白、嗜酸性粒细胞、肉芽肿以及传统亲表皮性特征。每个特征赋予一个半定量等级。
有4例FMF病例，性别分布均等，平均年龄17.5岁。所有皮损均位于面部。表现为：色素减退斑（2例）和红斑性斑块（2例）。2例出现脱发。所有4例临床诊断均为麻风病，2例鉴别诊断为黏液性脱发/结节病。组织学特征如下：所有4例均有不成比例的亲毛囊性、淋巴细胞带晕、毛囊黏蛋白以及核肿大/核卷曲，2例有显著嗜酸性粒细胞，1例有上皮样肉芽肿，4例有汗腺浸润，2例毛囊口有角化不全，1例有亲皮脂腺性。浸润位于球部（4例）和峡部（2例）。其余表皮未显示传统蕈样肉芽肿的迹象。
FMF的特征性表现为面部受累、显著的亲毛囊性、核异型性和核卷曲以及毛囊黏蛋白。肉芽肿和嗜酸性粒细胞的存在需要排除感染性病因。其余表皮未发现蕈样肉芽肿的表现不应妨碍病理学家做出此诊断。
