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).

Scarring



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PMID (PMCID)
14576683
 FEMALE Child
Whorled scarring alopecia: a rare phenomenon in incontinentia pigmenti?
Chan YC, Happle R, Giam YC.
J Am Acad Dermatol. 2003;49(5):929-31.
Two girls, 1 Malay and 1 Chinese, with incontinentia pigmenti of the Bloch-Sulzberger type had a whorled pattern of scarring alopecia.
7962735
 FEMALE Infant, Newborn
Incontinentia pigmenti: three cases with unusual features.
Sahn EE, Davidson LS.
J Am Acad Dermatol. 1994;31(5 Pt 2):852-7.
This child's mother, who also had incontinentia pigmenti, had identical atrophic streaks on the legs, as well as irregular axillary pigmentation, scarring alopecia, and dental abnormalities.