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A 5-year-old boy presented with multiple keratotic whitish plaques that were asymptomatic and randomly distributed on the buttocks, abdomen, and upper and lower limbs and had been increasing in number since birth (Figure 1, A and B). Dermoscopy of a lesion on the right leg revealed a homogeneous white pattern with well-defined borders. A lesion on the abdomen presented a homogeneous brown pigmentation, with hyperpigmented and well-defined borders, resembling leaf-like areas described in basal cell carcinoma (Figure 1, C and D). The clinical differential diagnosis included mainly common keratinocytic nevi, viral warts, flat warts, and mosaicism of waxy keratosis of childhood, which differs clinically in shape, pattern of distribution, and number of lesions. Biopsy taken from the lesion on the abdomen showed compact orthokeratotic hyperkeratosis, acanthosis with broad and rectangular rete ridges, and a basal cell layer with a striking palisaded arrangement of basal cell nuclei (Figure 2). With these features, PENS was diagnosed. Neurological examination of the patient was normal, and the follow-up to date has not shown neurological disorders. There is no history of similar lesions in the patient’s family.

A Pediatric Case of Papular Epidermal Nevus With “Skyline” Basal Cell Layer (PENS)