van der Steen P H, Mittag H, Küster W, Happle R
Department of Dermatology, University of Marburg, Germany.
Dermatology. 1996;193(2):147-8. doi: 10.1159/000246233.
A 40-year-old woman with focal dermal hypoplasia had a palmar papillomatous lesion showing a linear arrangement and histopathological features compatible with a diagnosis of epidermal nevus. This unusual manifestation of focal dermal hypoplasia is difficult to categorize. Undoubtedly the lesion reflects functional X-chromosome mosaicism. Although the mechanism of lyonization may give rise to true epidermal nevi such as CHILD nevus, we prefer to classify the present skin lesion as a nevoid disorder and not as an epidermal nevus.
一名患有局灶性真皮发育不全的40岁女性手掌出现乳头状病变，呈线性排列，组织病理学特征符合表皮痣的诊断。局灶性真皮发育不全的这种不寻常表现难以归类。毫无疑问，该病变反映了功能性X染色体嵌合体。尽管莱昂化机制可能导致真正的表皮痣，如CHILD痣，但我们更倾向于将目前的皮肤病变归类为痣样疾病而非表皮痣。
