[Incontinentia pigmenti]

Incontinentia Pigmenti (IP) originally described by Bloch in 1992 and by Sultzberger in 1928 is also known as the Bloch-Sultzberger syndrome. IP is a rare genodermatosis occurring in most races, but almost exclusively among females. IP is manifested as a skin disease with blisters, warts and hyperpigmentation. About 80 percent of IP patients develop systematic manifestations in the form of serious complications involving the eyes, teeth, skeleton as well as alopecia and CNS dysfunction. IP is inherited as an X-linked dominant trait, though its aetiology is unknown at present. The article consists in a review of the most important literature on IP and includes a report of a case from the Nordic IP Association archives. As a large Nordic research project on IP is being planned the association is keen to contact more people with this rare disease.