<prompt> True about incontinenta pigmenti include the following except aEURX-linked dominantPrimary skin abnormalityAvascularity of peripheral retinaOcular involvement is seen in almost 100 cases and is typically unilateral  <response>: Ocular involvement is seen in almost 100 cases and is typically unilateral Incontinentia pigmenti Incontinentia Pigmenti IP, also referred to as Bloch- Sulzberger syndrome is an unusual inherited disorder of skin pigmentation that is associated with abnormalties of :- i Skin -- 100 of affected people ii Teeth --80-90 of affected people iii Bones -- 30-40 of affected individuals iv CNS i.e., brain spinal cord 30-40 of affected individuals v Eyes -- 25-35 of affected individuals Incontinentia pignzenti is an X- linked dominant disorder - Usually occurs in .females as dominant disease is lethal in males. have only one X chromosome. Dermatological manifestations There is characteristic neonatal rash along the line of Blaschko which passes through four stages:- i Vesicular stage --f Present at bih ii Verrucous stage -- Between the age of 2-8 weeks. iii Hyperpigmented stage -- Between the age of 12-40 weeks. iv Hypopigmented stage -- From infancy through adulthood Hair Scarring alopecia Nails --Onychodystrophy or nail dysplasia, subungual keratotic tumor. Teeth Jaw -Delayed eruption, - Paial anodontia, - Microdontia, - Conical or pegged teeth. - Micrognathia, prognathia. Bone - Hemiveebrae, - Scoliosis, - Spinabifieda, - Syndactyly, - Acheiria Congenital absence of hands, extra ribs. CNS - Microcephaly, - Mental retardation, - Spasticity, - Seizures, - Ataxia, - Encephalopathy, - Hyperactivity Eves 20-35 case - Involvement is assymetrical. - The two pathognomic chages are :? - Retinal pigmentary changes with mottled diffuse hypopigmentation. - Abnormal peripheral retinal vessels with areas of nonperfusion avascularity of peripheral retina - Other changes of eye are - Microphthalmia, - Retrolental mass psedoglioma or retinoblastoma with intraocular calcification, - Cataract, - Leukocoria, - Band Keratopathy, - Strabismus, - Congenital glaucoma, - Blue sclera, Exudative retinal detachement 