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).
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ADULT syndrome
MONDO:0007072
autosomal dominant palmoplantar keratoderma and congenital alopecia
MONDO:0007083
ameloonychohypohidrotic syndrome
MONDO:0007095
ankyloblepharon-ectodermal defects-cleft lip/palate syndrome
MONDO:0007124
anonychia with flexural pigmentation
MONDO:0007131
Böök syndrome
MONDO:0007207