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March 1975


Arch Dermatol. 1975;111(3):389. doi:10.1001/archderm.1975.01630150109015

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Incontinentia Pigmenti. Presented by Andrew Segal, MD, Lawrence Krugel, MD.  A 3½-month-old girl had an eruption beginning three weeks after birth. Examination showed brownish hyperkeratotic papular lesions involving the upper part of the trunk and the extensor surface of the arms. The papules were covered by an ichthyotic-type adherent scale. There was no evidence of other cutaneous disorder or ocular or central nervous system abnormalities. Histologic examination showed moderate papillomatous acanthosis with hypergranulosis and hyperkeratosis, multiple foci of liquefaction degeneration of basal cells, and diffuse loss of pigment into macrophages in the upper corium. The acanthotic epidermis showed a few dyskeratotic cells with brightly eosinophilic cytoplasm and perinuclear halo.

Discussion  Dr. Thomas Chappel: Incontinentia pigmenti of the Bloch-Sulzberger type is a hereditary disorder transmitted as a dominant trait and occurring principally in female patients. It is uncertain whether the condition is sex-linked or autosomal. It is postulated that the disorder

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