Lim T W, Paik J H, Kim N I
Department of Dermatology, College of Medicine, Kyung Hee University, Seoul, Korea.
J Dermatol. 1999 Oct;26(10):677-81. doi: 10.1111/j.1346-8138.1999.tb02071.x.
Pachyonchia congenita (PC) is an uncommon autosomal dominant genodermatosis affecting the nails and other ectodermal tissues. The most striking features are symmetrically thickened dysmorphic nails and hyperkeratotic skin lesions. We report a case of pachyonychia congenita in a 30-year-old male patient who had thickening and gray-brown discoloration of all nails and many nodules on his back and neck. He also had hyperkeratotic skin lesions on both feet. His tongue had irregularly-shaped, whitish plaques. Histology of these nodules revealed the characteristic features of steatocystoma multiplex. After treatment with oral retinoic acid, his hyperkeratotic skin lesions improved.
先天性厚甲症（PC）是一种罕见的常染色体显性遗传性皮肤病，累及指甲和其他外胚层组织。最显著的特征是对称性增厚的畸形指甲和角化过度的皮肤病变。我们报告一例30岁男性先天性厚甲症患者，其所有指甲增厚并呈灰棕色，背部和颈部有许多结节。他的双脚也有角化过度的皮肤病变。他的舌头有形状不规则的白色斑块。这些结节的组织学检查显示为多发性皮脂囊肿的特征性表现。口服维甲酸治疗后，他的角化过度皮肤病变有所改善。
