Case report: squamous cell carcinoma, radial forearm flap and Huriez syndrome. Focus on a rare pathology

• Published in: Annales de chirurgie plastique et esthétique 2013-04, Vol.58 (2), p.175-179
• Main Authors: Dumont, L A, Gahagnon, T, Martinot-Duquennoy, V, Mortier, L, Guerreschi, P
• Format: Article
• Language: fre
• Subjects: Carcinoma, Squamous Cell - etiology; Carcinoma, Squamous Cell - pathology; Carcinoma, Squamous Cell - surgery; Follow-Up Studies; Forearm - surgery; Hand; Humans; Keratosis - complications; Male; Middle Aged; Rare Diseases; Reconstructive Surgical Procedures; Scleroderma, Localized - complications; Skin Neoplasms - complications; Skin Neoplasms - etiology; Skin Neoplasms - pathology; Skin Neoplasms - surgery; Surgical Flaps; Treatment Outcome
• ISSN: 1768-319X
• DOI: 10.1016/j.anplas.2011.07.003

Huriez disease is a rare autosomal dominant pathology characterized by the triad hypoplastic nail, hyperkeratosis and scleroatrophy of distal extremities. One of its most principal complications is the development of an aggressive squamous cell carcinoma. We present a case of a 62-year old patient who had an acute two hands scleroatrophy associated with recurrent squamous cell carcinoma treated by large excision and covered by trophic and thick radial forearm flap. This flap allowed us to treat the wound and the sclerosis shrinkage with aim to give back the functional benefit to the patient. It also gave the patient an oncological treatment despite aggressive management in one step surgery. Furthermore, one year later we did not observe cutaneous flap histological modification that could have degenerated into cancer. A multidisciplinary approach with dermatologists, geneticists and plastic surgeons is essential in addition with close medical supervision because of high cancer risks.