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).

Papule

A circumscribed, solid elevation of skin with no visible fluid, varying in size from a pinhead to less than 10mm in diameter at the widest point.

Total: 2

(per page)

PMID (PMCID)
1628974	FEMALE	Infant, Newborn
	[Onset of disseminated Bloch-Sulzberger incontinentia pigmenti].
	Schaller J, Kock M, Goos M. Hautarzt. 1992;43(6):383-5.
	The case report of a 2-week-old girl suffering from incontinentia pigmenti (Bloch-Sulzberger Syndrome) with disseminated urticarial erythemas, papules and vesicles at the onset is presented.
1628974	FEMALE	Infant, Newborn
	[Onset of disseminated Bloch-Sulzberger incontinentia pigmenti].
	Schaller J, Kock M, Goos M. Hautarzt. 1992;43(6):383-5.
	The case report of a 2-week-old girl suffering from incontinentia pigmenti (Bloch-Sulzberger Syndrome) with disseminated urticarial erythemas, papules and vesicles at the onset is presented.