Unilateral hyperkeratotic plaques along blaschko lines.

Epidermolytic ichthyosis (or epidermolytic hyperkeratosis) classically presents with erythroderma and increased fragility (blistering) at birth or soon thereafter. In later life, erythroderma and blistering improve gradually and the clinical picture is dominated by hyperkeratotic plaques in flexures and around joints. Linear epidermolytic hyperkeratosis is a unique, uncommon clinical variant and the absence of erythroderma and blistering are its hallmark. Linear lesions may be localized or generalized andunilateral or bilateral. Herein we report a 6-year-old girl with unilateral epidermolytic ichthyosis.