Hyperkeratotic plaques on the trunk and extremities

An 8-year-old white boy presented for a lifelong skin condition. At birth he had widespread blisters and erosions and later developed generalized, thick scaly plaques on his body and thinner scaling on his face. Biopsy specimens obtained for electron microscopy performed at birth revealed dyscohesive keratinocytes and clumped keratin intermediate filaments of suprabasal keratinocytes (Fig 1). The physical examination revealed generalized, thick, scaly plaques predominantly involving the flexural surfaces and brown hyperkeratotic, verrucous, scaly plaques on his trunk, legs, and arms (Fig 2).

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