Dermatofibrosarcoma protuberans: Under the mask of scar

Abstract

Dermatofibrosarcoma protuberans (Darier–Ferrand) is a rare mesenchymal tumor of fibrohistiocytic genesis with moderate malignancy and low incidence of metastasis, the pathogenesis and etiology of which remain unclear. The diagnosis is based on the clinical picture of a reddish-brown dense plaque or papule that gradually becomes a painful node with ulceration. Histological examination reveals the infiltrate with a moire pattern in the dermis in the form of fusiform cells with diffuse CD34+ staining during immunohistochemical analysis. Treatment of dermatofibrosarcoma protuberans (Darier–Ferrand) is a serious problem due to the high tendency for recurrence after removal. The methods described in the literature include extensive excision, Mohs surgery, chemotherapy, radiation therapy and targeted treatment with an anticancer protein-tyrosine kinase inhibitor. The authors present their own clinical observation of a patient with dermatofibrosarcoma protuberans (Darier–Ferrand) in whom a diagnostic error by only visual method and an incorrect treatment led to the worsening of skin process. The interest of this clinical observation lies in the difficulty of diagnosis during the initial visit, in the complexity of differential and morphological diagnosis.