棘状秃发性毛发角化病

Keratosis follicularis spinulosa decalvans:the first case report in China

报告1例棘状秃发性毛发角化病。患者男,22岁。因头皮弥漫性毛囊角化性丘疹伴脱发10余年就诊。皮肤科检查:头皮弥漫性瘢痕性脱发和毛囊角化性丘疹,并可见散在分布细小和稀疏的头发。组织病理检查可见表皮毛囊角化过度和轻度的毛囊周围纤维化,毛囊和血管周围有少量慢性炎性细胞浸润。结合临床和组织病理检查,符合棘状秃发性毛发角化病的诊断。临床上,该病需与棘状秃发性毛囊炎、毛囊性鱼鳞病伴脱发和畏光综合征(IFAP)、角膜炎-鱼鳞病-耳聋综合征(KID综合征)和无萎缩脱毛性毛发角化病等病鉴别。

A case of keratosis follicularis spinulosa deealvans is reported. A 22-year-old man presented with a lO-year history of diffuse follicular hyperkeratosis and alopecia of the scalp. Physical examination revealed extensive cicatrical alopecia and follicular keratotie papules on the scalp with fine and sparse hair. Biopsy specimen from the scalp showed perifollicular hyperkeratosis and fi- brosis. There were moderately dense chronic inflammation of the hair follicle and blood vessel. The diagnosis of keratosis follicularis spinulosa decalvans was made according to the clinical and histopathologic manifestations. Differential diagnosis must be made with folliculitis spinulosa decalvans, ichthyosis follicularis alopecia photophobia, and keratosis-ichythyosis-deafness syndrome.