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).

Abnormality of the skin

An abnormality of the skin.


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PMID (PMCID)
11507587
FEMALE Infant, Newborn
Laser photocoagulation in preproliferative retinopathy of incontinentia pigmenti.
Nguyen JK, Brady-Mccreery KM.
J AAPOS. 2001;5(4):258-9.
Incontinentia Pigmenti is a rare, X-linked, dominant disorder in which affected female infants develop characteristic abnormalities of the skin, central nervous system, hair, teeth, and eyes.