Kempf Werner, Kazakov Dmitry V, Broekaert Sigrid M C, Metze Dieter
*Kempf und Pfaltz, Histologische Diagnostik, Zürich, Switzerland; †Department of Pathology, Faculty of Medicine in Pilsen, Charles University in Prague, Prague, Czech Republic; and ‡Department of Dermatology, University of Münster, Münster, Germany.
Am J Dermatopathol. 2014 Jul;36(7):598-602. doi: 10.1097/DAD.0000000000000011.
Mycosis fungoides (MF) displays a broad spectrum of clinical, histological, and phenotypic variants with different prognostic impact. CD8⁺CD56⁺MF is very rare. So far, only 5 patients with CD8⁺CD56⁺ MF were reported to date. All of the previously described patients with this phenotypic variant were adults at the time of diagnosis and often manifested with widespread poikilodermatous skin lesions. Here we describe the clinicopathological features of a juvenile form of CD8⁺CD56⁺ non-poikilodermatous MF in a 10-year-old girl. In our patient, the disease displayed an indolent course and excellent prognosis with complete remission after treatment with UV light, topical steroids, and methotrexate. The differential diagnosis of CD8⁺ epidermotropic lymphocytic infiltrates in children is discussed, and the cases of CD8⁺CD56⁺ MF reported in the literature are reviewed.
蕈样肉芽肿（MF）表现出广泛的临床、组织学和表型变异，具有不同的预后影响。CD8⁺CD56⁺MF非常罕见。迄今为止，迄今仅报道了5例CD8⁺CD56⁺MF患者。所有先前描述的具有这种表型变异的患者在诊断时均为成年人，且常表现为广泛的皮肤异色性皮肤病变。在此，我们描述了一名10岁女孩的幼年型CD8⁺CD56⁺非皮肤异色性MF的临床病理特征。在我们的患者中，疾病呈惰性病程，预后良好，经紫外线、外用类固醇和甲氨蝶呤治疗后完全缓解。讨论了儿童CD8⁺亲表皮淋巴细胞浸润的鉴别诊断，并复习了文献中报道的CD8⁺CD56⁺MF病例。
