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).
A disordered proliferation of mature tissues that is native to the site of origin, e.g., exostoses, nevi and soft tissue hamartomas. Although most hamartomas are benign, some histologic subtypes, e.g., neuromuscular hamartoma, may proliferate aggressively such as mesenchymal cystic hamartoma, Sclerosing epithelial hamartoma, Sclerosing metanephric hamartoma.
[Dragged disk syndrome. Review of etiologies. Apropos of a case].
Barreau E, Cohen SY, Coscas G. J Fr Ophtalmol. 1996;19(1):58-63.
Different aetiologies were discussed including retinopathy of prematurity, familial exudative vitreoretinopathy, congenital retinal folds, incontinentia pigmenti, Eales' disease, toxocariasis, combined hamartoma of the retina and the pigment epithelium.
