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November 1958

Incontinentia PigmentiReport of a Family

Author Affiliations

U. S. N. R.; Indianapolis; Albany, Ga.

From the Department of Dermato-Syphilology, Indianapolis General Hospital (Dr. John Eric Dalton, Chairman).

AMA Arch Derm. 1958;78(5):607-611. doi:10.1001/archderm.1958.01560110053007

Incontinentia pigmenti usually begins in early childhood as a series of linear and grouped vesicles on an erythematous base, which, when ruptured, tend to become impetiginized. These may disappear and recur for weeks or months, gradually giving way to an intermediate temporary stage of linear verrucous lesions, or the vesicles may lead directly to the third or pigment stage, characterized by a reticulated pattern of pigmented macules, whorls, lines, and patches. Many years later, in some patients, a final stage may occur in which the lesions fade, leaving either no blemishes or slightly atrophic, slightly depigmented areas to mark the sites. Many cases are associated with mild to severe ectodermal and mesodermal defects.2,3,6,11

Since the literature, pathogenesis, and characteristics of the disease have been surveyed in detail by previous papers on the subject,2,5,6,9,11 only some of the more pertinent cases will be reviewed

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