<prompt> True about incontinenta pigmenti include the following except:X-linked dominantPrimary skin abnormalityAvascularity of peripheral retinaOcular involvement is seen in almost 100 cases and is typically unilateral  <response>: 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. Dentalteeth 80-90, bones 30-40, CNS neurological 30-40 and ocularophthalmic 25-35 involvement may be associated with skin involvement in few cases but not in all casesQ. 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 bandsstreaks that fail to tan on sun exposure from infancy through adulthood. 