Lehman Julia S, Cook-Norris Robert H, Weed Brent R, Weenig Roger H, Gibson Lawrence E, Weaver Amy L, Pittelkow Mark R
Department of Dermatology, MayoClinic, Rochester, Minnesota, USA.
Arch Dermatol. 2010 Jun;146(6):607-13. doi: 10.1001/archdermatol.2010.101.
To clarify clinicopathologic features and reconcile discrepancies in previous studies of folliculotropic mycosis fungoides (FMF).
A single-center retrospective clinicopathologic study and a systematic review of FMF.
Tertiary referral center in the midwestern United States.
Patients with clinical and histopathologic evidence of FMF seen at the tertiary referral center during a 12(1/2)-year period.
Clinicopathologic features of FMF.
Fifty patients (32 male [64%] and 18 female [36%]) met study criteria for the clinicopathologic review. Pruritic patches, plaques, and folliculocentric lesions (milia, cysts, and alopecia) on the head, neck, and trunk were common clinical findings. The mean time to diagnosis of FMF was 5.0 years. Diagnostic latency did not affect risk of death. One-year and 5-year overall survival rates were 96% and 62%, respectively. Frequent microscopic features were follicular mucinosis (74%) and epidermotropism (54%). Systematic review of 186 additional patients confirmed male predominance (ratio of men to women, 3.2:1.0), prevalent pruritus (73%), frequent follicular mucinosis (69%) and epidermotropism (37%) microscopically, and common head, neck, and trunk involvement. Combined data demonstrated that 6% of patients with FMF had concurrent non-mycosis fungoides hematologic malignant neoplasms and that the 5-year overall survival rate was 62% to 64%.
Folliculotropic mycosis fungoides has distinct clinical and microscopic features and is associated with a poor 5-year overall survival rate.
阐明亲毛囊性蕈样肉芽肿（FMF）的临床病理特征，并解决既往研究中的差异。
一项单中心回顾性临床病理研究及FMF的系统评价。
美国中西部的三级转诊中心。
在该三级转诊中心12.5年期间有FMF临床和组织病理学证据的患者。
FMF的临床病理特征。
50例患者（32例男性[64%]和18例女性[36%]）符合临床病理回顾的研究标准。头颈部和躯干的瘙痒性斑块、斑片及毛囊中心性损害（粟丘疹、囊肿和脱发）是常见的临床发现。FMF的平均诊断时间为5.0年。诊断延迟不影响死亡风险。1年和5年总生存率分别为96%和62%。常见的显微镜特征为毛囊黏蛋白沉积症（74%）和向表皮性（54%）。对另外186例患者的系统评价证实男性占优势（男女比例为3.2:1.0）、普遍存在瘙痒（73%）、显微镜下常见毛囊黏蛋白沉积症（69%）和向表皮性（37%），以及常见头颈部和躯干受累。综合数据显示，6%的FMF患者同时患有非蕈样肉芽肿性血液系统恶性肿瘤，5年总生存率为62%至64%。
亲毛囊性蕈样肉芽肿具有独特的临床和显微镜特征，且5年总生存率较低。
