D i.e. Ocular involvement is seen in almost 100% cases and is typically unilateral - Incontinetia pigmenti (Bloch-Sulzberger Syndrome) is X linked dominant disorder primarily affecting females as it is believed to be embryonically (in utero) lethal in malesQ. - It is primarily skin abnormality presenting with 4 overlaping stages of linear or whorled cutaneous lesions along lines of BlaschkoQ. Linear streaks & plaques of erythematous vesicles & papules (stage 1, bih - few months); thickened verrucous was & whorled plaquesQ (Stage 2, 2 weeks - months) both usually on extremities > trunk. Linear streaks, whorls, flacks & reticulated patches of hyperpigmentation mainly on trunk > extremitiesQ (stage 3, first few months to adolescence) and anhidrotic, hairless, atrophic, hypopigmented linear patches or streaks usually on calves (stage 4, adolescence to adultohood). Dental/teeth (80-90%), bones (30-40%), CNS /neurological (30-40%) and ocular/ophthalmic (25-35%) involvement may be associated with skin involvement in few cases (but not in all cases)Q. Retinal vasoocclusive ischemia is primary mechanismQ. Icontinentia pigmenti is an X-linked dominant disorder primarily affecting femalesQ and belived to be embryonic lethal in males. It primarily involves skin (in 100% cases) and presents with typical skin lesions along the lines of Blaschko often at bih, usually before 1st week and rarely after the fist 2 monthsQ. Four cutaneous stages, some times with some overlap are Inflammatory macule, papule, vesicle and pustules (vesicular stage) from bih or sholy there after. Hyperkeratotic and verrucous lesions (stage) between 2-8 weeks of age Blue-Gray or slate to brown, bizarre Chinese letter pattern hyperpigmentation (several months of age into adolescence) Atrophic hypopigmented, and depigmented, hariless, anthidrotic bands/streaks that fail to tan on sun exposure (from infancy through adulthood).