Templeton S F, Wiegand S E
Department of Dermatology, Emory University School of Medicine, Atlanta, GA 30322, USA.
Am J Dermatopathol. 1997 Apr;19(2):180-4. doi: 10.1097/00000372-199704000-00013.
Pachyonychia congenita (PC) is a rare genodermatosis with characteristic nail abnormalities and occasional palmoplantar keratoderma and leukokeratosis oris; alopecia may occur (10% of patients). This report is the first microscopic description of a patient with PC-associated alopecia. Transverse section histologic features include diminished follicular density with preservation of follicular units, prominent miniaturization of follicles, dyskeratosis of outer root sheath keratinocytes, and moderate parakeratotic and orthokeratotic follicular hyperkeratosis. These microscopic features may be seen individually in other nonscarring alopecias, but the combination may be unique to PC-associated alopecia. Differential diagnoses include alopecia areata, androgenetic alopecia and traction alopecia/ trichotillomania.
先天性厚甲症（PC）是一种罕见的遗传性皮肤病，具有特征性的指甲异常，偶尔伴有掌跖角化病和口腔黏膜白斑；可能会出现脱发（10%的患者）。本报告是首例对先天性厚甲症相关脱发患者的微观描述。横切面组织学特征包括毛囊密度降低，但毛囊单位保留，毛囊显著变小，外根鞘角质形成细胞角化异常，以及中度的毛囊角化不全和正角化过度。这些微观特征在其他非瘢痕性脱发中可能单独出现，但这种组合可能是先天性厚甲症相关脱发所特有的。鉴别诊断包括斑秃、雄激素性脱发和牵拉性脱发/拔毛癖。
