Verruciform xanthoma overlying inflammatory linear verrucous epidermal nevus and in broad segmental distribution

A 17-year-old woman presented with moderately itchy, non-progressive, linearly arranged verrucous plaques over dorsum of left foot since early childhood. Two years ago, she developed slowly increasing, verrucous exophytic growth in posterior most aspect of linear verrucous plaque. One year ago, she also developed multiple, linearly arranged, fleshy plaques with surface crusting over lateral aspect of right leg extending to thigh. Biopsy from both verrucous lesion on left foot and fleshy plaque on right leg showed an exophytic growth with significant papillomatosis, neutrophils in stratum corneum, acanthosis and infiltration of papillary dermis with foamy macrophages that were CD 68 positive, features compatible with verruciform xanthoma (VX). Biopsy from linear verrucous plaque over left foot was consistent with inflammatory linear verrucous epidermal nevus (ILVEN). A diagnosis of segmental VX and VX overlying ILVEN was made.