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).

Skin rash

A red eruption of the skin.

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PMID (PMCID)
26777982	FEMALE	Infant, Newborn
	Effectiveness of Corticosteroid Therapy for Acute Neurological Symptoms in Incontinentia Pigmenti.
	Tomotaki S, Shibasaki J, Yunoki Y, Kishigami M, Imagawa T, Aida N, Toyoshima K, Itani Y. Pediatr Neurol. 2016;56:55-58.
	Incontinentia pigmenti is a rare neurocutaneous disorder that may result in neurological symptoms in addition to its characteristic skin rashes.