Pilomatricoma in Syndromic Contexts: A Literature Review and a Report of a Case in Apert Syndrome

This literature review and case report explore the occurrence of pilomatricomas, benign tumors from hair follicle matrix cells, in syndromic contexts, particularly highlighting a novel association with Apert syndrome. A systematic review of 81 full-text articles identified 96 cases of pilomatricomas linked to genetic syndromes, with a slight female predominance and frequent occurrence in pediatric patients. The study emphasizes the importance of genetic analysis for accurate diagnosis and management, as pilomatricomas can be the first sign of underlying genetic syndromes. Notably, no reports exist of Apert syndrome patients without pilomatricomas, underscoring the significance of this association.