Incontinentia pigmenti

Incontinentia pigmenti (IP) is a rare X-linked dominant multi-systemic ectodermal dysplasia usually lethal in males and presenting neonatally in females with a bullous rash along Blashko's lines (BL) followed by verrucous plaques evolving over time to hyperpigmented swirling patterns. It is further characterized by teeth abnormalities, alopecia, nail dystrophy and affects occasionally the retina and the central nervous system (CNS).

Elevated alkaline phosphatase

Abnormally increased serum levels of alkaline phosphatase activity.


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PMID (PMCID)
1157442
FEMALE
Incontinentia pigmenti with unusual features.
Khan AJ, Mitra N, Evans HE, Elguezabal A.
Clin Pediatr (Phila). 1975;14(9):874-6.
A case is presented of incontinentia pigmenti with severe neurological handicaps and unusual features such as single umbilical artery, pigmented lesion at birth, and biochemical changes including elevated alkaline phosphatase and immunoglobulin M.