Resumo

SABIDO TOLEDO, Lisbeth; MARTINEZ GARCIA, Gretter  e  LUNA CEBALLOS, Elsa Juana. Incontinentia pigmenti. A case report. Rev.Med.Electrón. [online]. 2021, vol.43, n.4, pp. 1099-1107.  Epub 31-Ago-2021. ISSN 1684-1824.

Pigmentary incontinence, also known as Bloch-Sulzberger syndrome, is a rare X chromosome-linked genodermatosis, located in Xq28. It affects the female sex and has different clinical manifestations in the same family. Ii is a multi-systemic disease characterized by affecting, in a variable way, the tissues derived from the neuroectoderm, the skin, the eyes, the teeth and the central nervous system. Skin lesions are the most significant ones since birth time, and skin biopsy confirms the diagnosis. Due to the rareness of this entity, we presented the case of a nursing female infant aged one month, with a family history of pigmentary incontinence, who presented typical lesions in the skin, since his first week of life, in different phases, following the lines of Blaschko. Ocular manifestations and eosinophilia were confirmed.

Palavras-chave : pigmentary incontinence; Bloch-Sulzberger syndrome; X chromosome-linked genodermatosis.

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