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).

Fever

Elevated body temperature due to failed thermoregulation.

Total: 2

(per page)

PMID (PMCID)
27396921	MALE	Infant
	Incontinentia Pigmenti Coxsackium.
	Jefferson J, Grossberg A. Pediatr Dermatol. 2016;33(5):e280-1.
	Late recurrences of first-stage inflammatory vesiculobullous lesions of incontinentia pigmenti (IP) are uncommon but have been reported to occur in the setting of infections, fevers, and vaccinations.
8520067	FEMALE	Child
	Recurrent inflammation in incontinentia pigmenti of a seven-year-old child.
	Pfau A, Landthaler M. Dermatology. 1995;191(2):161-3.
	The combination of recurrent inflammatory skin manifestations within the hyperpigmented skin areas of incontinentia pigmenti and fever is unique.