Tuberous sclerosis complex (TSC) is an autosomal dominant neurocutaneous syndrome that leads to hamartoma formation in multiple organs, including the skin (Curatolo et al., 2008). Cutaneous hamartoma formation occurs secondary to loss of function of either the TSC1 or TSC2 gene in fibroblast-like cells and subsequent dysregulation of the mechanistic target of rapamycin complex 1, or mTORC1 (Li et al., 2008; Tyburczy et al., 2014). Angiofibromas are among the most well recognized TSC-related skin hamartomas and consist of multiple pink papules on the central face (Little, 1909). Here we describe angiofibromas of the nipple-areolar complex.

Between October 2012 and January 2015, 53 TSC patients (50 women, 3 men) participated in studies of lymphangioleiomyomatosis (LAM), a TSC-associated lung disease with female predominance, at the National Institutes of Health in Bethesda, Maryland. Written informed consent was obtained according to IRB-approved protocols 00-H-0051, 95-H-0186 and/or 82-H-0032.