Fibrous papule of the face with granular cells

Fibrous papule of the face is a common benign lesion located most often on the nose. It presents usually as a single small, firm, skin-coloured papule and is often misdiagnosed as melanocytic naevus, wart or small nodular basal cell carcinoma. Histopathologically, the lesions are characterized by involvement of the upper dermis by a fibrovascular proliferation and scattered triangular or stellate, often multinucleated cells. Uncommon histopathologic variants of fibrous papule of the face include hypercellular, clear-cell, pleomorphic, pigmented, inflammatory and granular-cell types. We present here a patient with the syndrome of familial cancer and fibrous papule of the face with granular cells. The granules stained strongly with PAS stain, as well as with CD68 and NKI/C3 immunostains, whereas S-100 protein resulted negative. In our patient the mutations in the 2 most often affected DNA mismatch repair genes of Muir-Torre syndrome were not found, therefore the origin of the familial cancer syndrome remains unknown. Probably the occurrence of the granular-cell fibrous papule of the face was coincidental.