Progressive symmetric erythrokeratoderma with nephrotic syndrome: Coincidence or new association?
A15-year-old boy born of second-degree consanguineous marriage presented with asymptomatic scaly skin lesions on the extremities since 5 years. Lesions initially started on dorsum of feet, and gradually progressed to involve knees and hands. These lesions were persistent and nonmigratory. There was no history of similar skin eruptions in the family or siblings. He had steroid-dependent nephrotic syndrome from infancy.\nCutaneous examination showed well-demarcated, hyperpigmented, hyperkeratotic scaly plaques distributed symmetrically on the dorsum of hands and feet, elbows, knees, and tendoachillies [Figures –]. Palms and soles were normal. There was no abnormality of hair, nails, or mucous membrane. There was no musculoskeletal or ocular abnormality. He had puffiness of face with cushingoid features and pedal edema. Skin biopsy revealed hyperkeratosis, focal parakeratoses, acanthosis, normal granular layer, and a moderate mononuclear infiltration in upper dermis []. Based on clinical and histopathological findings, diagnosis of PSEK was made. He was treated with emollients, topical steroids, and keratolytics with marked improvement.