True about incontinenta pigmenti include the following except:
Correct Answer: Ocular involvement is seen in almost 100% cases and is typically unilateral
Description: 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).
Category:
Skin
Get More
Subject Mock Tests
Practice with over 200,000 questions from various medical subjects and improve your knowledge.
Attempt a mock test nowMock Exam
Take an exam with 100 random questions selected from all subjects to test your knowledge.
Coming SoonGet More
Subject Mock Tests
Try practicing mock tests with over 200,000 questions from various medical subjects.
Attempt a mock test now