Castano Ekaterina, Glick Sharon, Wolgast Lucia, Naeem Rizwan, Sunkara Jaya, Elston Dirk, Jacobson Mark
Department of Pathology, Montefiore Medical Center, Bronx, NY, USA.
J Cutan Pathol. 2013 Nov;40(11):924-34. doi: 10.1111/cup.12217.
Patients with hypopigmented mycosis fungoides (HMF) present at a younger age than those with classic MF. Our goal was to describe the clinical presentation, histopathologic features and long-term outcome in patients who developed HMF before the age of 21. It was observed that among 69 pediatric patients diagnosed with MF between 1992 and 2010, 50 had HMF. Thirty-five patients had clinical follow-up. There were 37 males and 32 females with a mean age of 13.6 years. Most patients were African American or Hispanic and presented with multiple hypopigmented patches. All biopsies showed epidermotropism of T-lymphocytes, whereas fibroplasia and lichenoid infiltrate were variable. All specimens tested were CD8+. Treatment modalities included topical steroids, narrow band ultraviolet B and psoralen and ultraviolet A. HMF patients were followed for <1-12 years. Most children responded to treatment, but recurrence rates were high. One patient progressed to plaque/tumor stage. Others did not progress; however, many were lost to follow-up. We present a large cohort of children with HMF and report on the features of disease and progression. A major difference in histology of HMF was lack of fibroplasia and lichenoid infiltrate, probably because of presentation in the early patch stage. Most patients have a waxing-and-waning course and relapse after discontinuation of therapy, requiring repetitive treatment.
色素减退性蕈样肉芽肿（HMF）患者比经典蕈样肉芽肿（MF）患者发病年龄更小。我们的目标是描述21岁前发生HMF的患者的临床表现、组织病理学特征和长期预后。据观察，在1992年至2010年间诊断为MF的69例儿科患者中，有50例患有HMF。35例患者进行了临床随访。其中男性37例，女性32例，平均年龄13.6岁。大多数患者为非裔美国人或西班牙裔，表现为多处色素减退斑。所有活检均显示T淋巴细胞亲表皮现象，而纤维组织增生和苔藓样浸润情况各异。所有检测标本均为CD8 +。治疗方式包括外用类固醇、窄谱中波紫外线和补骨脂素及长波紫外线。HMF患者随访时间为<1至12年。大多数儿童对治疗有反应，但复发率很高。1例患者进展为斑块/肿瘤期。其他患者未进展；然而，许多患者失访。我们报告了一大群患有HMF的儿童，并阐述了疾病特征和进展情况。HMF组织学的一个主要差异是缺乏纤维组织增生和苔藓样浸润，这可能是因为其处于早期斑片期。大多数患者病情呈反复波动过程，停药后复发，需要反复治疗。
