The rarity of incontinentia pigmenti, this mixed ectodermal and mesodermal syndrome, with its many associated defects, makes this entity of considerable interest to both the dermatologist and the pediatrician. This disorder was first described by Bardach1 as a systematized nevus, and received the name ``incontinentia pigmenti'' in reports by Bloch2 in 1926, and in 1927 by Sulzberger.3 The earlier case reports were limited to descriptions of the pigmentary phase and the associated developmental abnormalities. It remained for Carney4 in 1951 to describe first the initial vesicular stage, which often precedes the better-known bizarre pigmentary whorls. He showed that in the early months, inflammatory lesions, particularly vesicles and bullae, may come and go, with associated blood eosinophilia. At a later time, linear verrucous lesions may develop and, eventually, the typical pigmentation. Various ectodermal and mesodermal defects have been recorded in
CRAMER JA, SCHMIDT WJ. Incontinentia PigmentiReport of Six Cases. AMA Arch Derm. 1955;71(6):699–702. doi:10.1001/archderm.1955.01540300021006
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