Abstract
Gorlin-Goltz syndrome also known as nevoid basal cell carcinoma syndrome (NBCCS) is a rare, complex hereditary disorder characterized by phenotypic developmental anomalies and postnatal tumors especially basal cell carcinomas (BCCs) and medulloblastomas. It is inherited as autosomal dominant disorder with inactivating mutation in PTCH 1 gene. Reticulate acropigmentation of Kitamura, a rare pigmented genodermatosis has not been described in the literature in association with NBCCS and may be a new finding. Considering the rarity of the disease and its wide clinical spectrum and complications, it is of paramount importance to identity these atypical presentations of the syndrome at an early stage for holistic approach, early intervention, monitoring of basal cell carcinoma, and genetic counselling.
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