Whitmore S E, Simmons-O'Brien E, Rotter F S
Department of Dermatology, Johns Hopkins Medical Institutions, Baltimore, Md.
Arch Dermatol. 1994 Apr;130(4):476-80.
Only five cases of purely macular hypopigmented mycosis fungoides have been published in the literature. These patients all had clinical evidence of disease 7 months to 10 years prior to histologic diagnosis, suggesting that this clinical presentation of mycosis fungoides is easily misdiagnosed.
Three African-American males, ages 9, 15, and 22 years, were found to have mycosis fungoides on evaluation of skin biopsy specimens after initially being clinically diagnosed with, and treated for, pityriasis alba. All three had typical histopathologic changes and cell marker studies showing a relative loss of the CD7 antigen, which is normally present on the majority of T cells. One patient who was treated with methoxsalen plus UV light (PUVA) therapy had clearance of his disease clinically and histologically.
From the literature, it would appear that macular hypopigmented mycosis fungoides is exceptionally rare. This perceived rarity may be inaccurate due to either relative underreporting, incorrect diagnoses, or both. From the eight patients now reported to date, it may be said that hypopigmented mycosis fungoides may be seen in nonwhite individuals and more often than not, has onset before age 20 years.
文献中仅发表过5例单纯性黄斑色素减退性蕈样肉芽肿病例。这些患者在组织学诊断前7个月至10年均有疾病的临床证据，提示蕈样肉芽肿的这种临床表现容易被误诊。
3名非裔美国男性，年龄分别为9岁、15岁和22岁，最初临床诊断为白色糠疹并接受治疗，在对皮肤活检标本进行评估时发现患有蕈样肉芽肿。所有3例均有典型的组织病理学改变，细胞标志物研究显示CD7抗原相对缺失，而CD7抗原通常存在于大多数T细胞上。1例接受甲氧沙林加紫外线光疗（PUVA）的患者临床和组织学上疾病均已清除。
从文献来看，黄斑色素减退性蕈样肉芽肿似乎极为罕见。由于报告相对不足、诊断错误或两者兼而有之，这种罕见情况可能并不准确。从目前报告的8例患者来看，可以说色素减退性蕈样肉芽肿可见于非白人个体，且通常在20岁之前发病。
