Acta Dermato-Venereologica issue 50:1 98-1CompleteContent | Page 45

155 Dermatofibrosarcoma Protuberans Presenting as a Large Atrophic Plaque on the Chest Ryosuke SAIGUSA, Takuya MIYAGAWA*, Satoshi TOYAMA, Jun OMATSU, Tomomitsu MIYAGAKI, Yuri MASUI, Daisuke YAMADA and Shinichi SATO Department of Dermatology, University of Tokyo Graduate School of Medicine, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-8655, Japan. *E-mail: [email protected] Accepted Sep 19, 2017; Epub ahead of print Sep 20, 2017 Atrophic dermatofibrosarcoma protuberans (DFSP) is a very rare variant of DFSP, which presents as an atrophic, asymptomatic plaque that can be difficult to diagnose. Like classic DFSP, standard wide excision is necessary for the treatment of atrophic DFSP, thus accurate diag- nosis is indispensable. We report here a case of atrophic DFSP that presented as an unprecedentedly large atrophic plaque on the anterior chest, which was diagnosed and treated successfully. CASE REPORT A 48-year-old healthy Japanese woman presented with an 8-year history of red nodules surrounded by a palm-sized (13×12 cm) area of red atrophic plaque on the anterior chest wall (Fig. 1). She had no history of surgery or trauma to the chest. She had noticed the atrophic plaque at the age of 40 years. The plaque expanded gradually, fol- lowed by development of red nodules. The results of la- boratory investigations, including anti-nuclear antibody, were within normal limits. Histological analysis showed an extensive basophilic nodular area in the dermis and upper hypodermis in addition to dermal and epidermal atrophy (Fig. 2A). This nodular area was composed of SHORT COMMUNICATION Fig. 1. A palm-sized red atrophic plaque with red nodules on the anterior chest. monomorphic spindle cells with minimal cellular atypia, which were arranged in a characteristic storiform pattern (Fig. 2B). Immunohistochemical staining revealed that these spindle cells stained positively for CD34 (Fig. 2C) and negatively for S100 protein or epithelial membrane antigen (EMA), which suggested a diagnosis of DFSP, plaque-like CD34-positive dermal fibroma or solitary fibrous tumour. To confirm the diagnosis, we further Fig. 2. Histopathology, immunostaining and molecular analysis. (A, B) Haematoxylin-eosin staining of the atrophic plaque lesion (A, ×12.5; B, ×400). (C) CD34 immunostaining of the atrophic lesion (×400). (D) The detection of COL1A1–PDGFB gene fusion transcripts in the skin samples from the lesion. This is an open access article under the CC BY-NC license. www.medicaljournals.se/acta Journal Compilation © 2018 Acta Dermato-Venereologica. doi: 10.2340/00015555-2800 Acta Derm Venereol 2018; 98: 155–156