Operative Management of Phakomatosis Pigmentokeratotica Sequelae

Phakomatosis pigmentokeratotica (PPK) is a rare variant of epidermal nevus syndrome, classically characterized by the coexistence of nevus sebaceus and nevus spilus. Secondary neoplasms arising within nevus sebaceus, including syringocystadenoma papilliferum (SPAP), can cause persistent irritation, discomfort, and bleeding that negatively impact quality of life, particularly in patients with extensive disease. Due to the rarity of this condition, no standardized management protocol exists for symptomatic SPAPs, which poses a therapeutic challenge for this patient population. Herein, we present a case of multiple SPAPs in a patient with PPK successfully managed using multimodal therapy involving dermatologic surgery and ablative CO2 laser, adding to the limited literature on the management of recalcitrant SPAPs.

Abstract from PubMed