Paller A S, Moore J A, Scher R
Department of Dermatology, Children's Memorial Hospital of Northwestern University Medical School, Chicago, IL 60614.
Arch Dermatol. 1991 May;127(5):701-3.
Pachyonychia congenita is an autosomal dominant disorder that usually develops in early infancy. We have observed five patients with the onset of the typical subungual hyperkeratoses of pachyonychia during the teenage years. Leukokeratosis and keratoderma of the palms and soles were associated. The family history of three of the patients suggests that pachyonychia congenita tarda is also inherited in an autosomal dominant manner.
先天性厚甲症是一种常染色体显性疾病,通常在婴儿早期发病。我们观察到5例患者在青少年时期出现典型的先天性厚甲症甲下过度角化。同时伴有掌跖部白角化病和角化皮病。其中3例患者的家族史表明迟发性先天性厚甲症也以常染色体显性方式遗传。