Fibrous Papule

Fibrous papule is a form of angiofibroma typically occurring in adulthood with a predilection for the central face. Histologically, they are composed of vessels with dilated lumens set in a fibroblastic, collagenous stroma. Multiple fibrous papules can be seen in patients with tuberous sclerosis (TS). TS is an autosomal dominant disease caused by mutations in TSC1 and TSC2. Multiple organs are affected by neoplasia in TS including the brain, skin, heart, eyes, lungs, liver and kidneys. Skin lesions are present in almost all patients and include angiofibromas, shagreen patches and ash-leaf spots (hypopigmented macules).