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).

Hemangioma

A hemangioma is a benign tumor characterized by blood-filled spaces lined by benign endothelial cells. A hemangioma characterized by large endothelial spaces (caverns) is called a cavernous hemangioma (in contrast to a hemangioma with small endothelial spaces, which is called capillary hemangioma).


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(per page)
PMID (PMCID)
20548858
OTHER
A case of incontinentia pigmenti associated with multiorgan abnormalities.
Chung WK, Lee DW, Chang SE, Lee MW, Choi JH, Moon KC.
Ann Dermatol. 2009;21(1):56-9.
We describe an 11-month-old girl with incontinentia pigmenti associated with a ventricular septal defect, left hemiatrophy, hemangiomas, an abnormal labial frenum, and spastic cerebral palsy manifested as left hemiplegia and developmental delay.
16516787
MIXED_SAMPLE Infant
Refractive laser surgery in children with coexisting medical and ocular pathology.
Astle WF, Papp A, Huang PT, Ingram A.
J Cataract Refract Surg. 2006;32(1):103-8.
Underlying medical and ocular conditions were as follows: upper eyelid hemangioma with oblique myopic astigmatism, Pelizaeus-Merzbacher leukodystrophy with nystagmus, Klippel-Trenaunay-Weber syndrome with glaucoma, incontinentia pigmenti with unilateral optic nerve atrophy, and Goldenhar syndrome with unilateral optic nerve hypoplasia.