Pereira Marcela A C, Mesquita Lismary A de F, Budel Anelise R, Cabral Carolina S P, Feltrim Amanda de S
Hospital Universitário Evangélico de Curitiba, PR, Brazil.
An Bras Dermatol. 2010 May-Jun;85(3):372-5. doi: 10.1590/s0365-05962010000300013.
Incontinentia pigmenti is a rare X-linked genodermatosis that affects mainly female neonates. Skin manifestations are the most common and occur in four quite distinct phases. A female infant presented vesiculobullous lesions on trunk and limbs, and a verrucous lesion on the right palm. Biopsy revealed eosinophil exocytosis and pigment incontinence, confirming the clinical hypothesis. Although uncommon, incontinentia pigmenti should be taken into consideration as a possible differential diagnosis when vesiculobullous and verrucous lesions are present in childhood.
色素失禁症是一种罕见的X连锁遗传性皮肤病，主要影响女性新生儿。皮肤表现最为常见，且发生在四个截然不同的阶段。一名女婴躯干和四肢出现水疱大疱性皮损，右手掌出现疣状皮损。活检显示嗜酸性粒细胞外渗和色素失禁，证实了临床诊断。尽管色素失禁症不常见，但当儿童期出现水疱大疱性和疣状皮损时，应将其作为可能的鉴别诊断加以考虑。
