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Journal Compilation © 2020 Acta Dermato-Venereologica. doi: 10.2340/00015555-3599 Phacomatosis pigmentokeratotica (PPK) is clinically defined by the co-occurrence of an organoid naevus of sebaceous differentiation and a speckled lentiginous naevus. Based on clinical associations and mutational analysis, PPK is currently considered a clinical variant of Schimmelpenning-Feuerstein-Mims syndrome (SFMS). Neoplasia arising in sebaceous naevi are not uncommon with age, but are rarely malignant.

Gene Mutation Mapping in a Fatal Case of Phacomatosis Pigmentokeratotica Happle