ONE OF the most fascinating aspects of dematology is the manner in which the skin and its appendages often reflect the presence of pathologic processes in other organs or tissue systems. Such interrelated findings may be acquired or developmental. An example of such a developmental relationship is incontinentia pigmenti.
Incontinentia pigmenti is a rare congenital anomaly that was first fully described by Sulzberger1 in 1927, although it had originally been presented by Bloch2 in 1925. Siemens3 in 1929 described the same condition as "melanosis corii degenerativa." Since this time the syndrome has been adequately described by several authors both in this country and abroad. The condition is rare. The number of reported cases is estimated as between 60 and 70.
Incontinentia pigmenti represents another example of what Wiener4 has termed a "dermadrome." As is true of some other dermadromes, the skin manifestations of
PHILPOTT JA, WOODBURNE AR, PHILPOTT OS. INCONTINENTIA PIGMENTI. AMA Arch Derm. 1955;71(2):214–218. doi:10.1001/archderm.1955.01540260072015
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