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).

Macule

A flat, distinct, discolored area of skin less than 1 cm wide that does not involve any change in the thickness or texture of the skin.

Total: 1

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PMID (PMCID)
15575852	FEMALE	Child
	Pulmonary tuberculosis and cutaneous mycobacterial infection in a patient with incontinentia pigmenti.
	Senturk N, Aydin F, Haciomeroglu P, Yildiz L, Totan M, Canturk T, Turanli AY. Pediatr Dermatol. 2004;21(6):660-3.
	A 12-year-old girl hospitalized for pulmonary tuberculosis presented with bizarre-shaped brown macules following Blaschko lines on the left deltoid area, compatible with incontinentia pigmenti, which had appeared following BCG vaccination at the age of 7 years.