Clinical manifestation

Cutaneous changes:

Stage 1: linear, red papules and vesicles grouped on an erythematous base, mainly on the extremities

Stage 2: linear, verrucous plaques on an ery-thematous base

Stage 3: streaks and whorls of brown or slate-gray pigmentation along the lines of Blaschko, particularly on the trunk Stage 4: hypopigmented, atrophic, reticulated patches, mostly on the lower extremities; lusterless, thin hair; nail dystrophy, ranging from mild pitting or ridging to severely thickened, abnormally ridged nails; dental abnormalities

• Ocular findings: retinal detachment; pro-liferative retinopathy; fibrovascular retro-lental membrane; cataracts; atrophy of the ciliary body

• Neurologic findings: seizures; developmental delay; mental retardation; ataxia, spasticity; microcephaly; cerebral atrophy; hypoplasia of the corpus callosum; periven-tricular cerebral edema

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