B Bonaventura1, D Kraus2, G B Stark2, H Fuellgraf3, J Kiefer2. 1. Department of Plastic and Hand Surgery, Medical Center - University of Freiburg, Faculty of Medicine, Comprehensive Cancer Center Freiburg, University of Freiburg, Freiburg, Germany. bastian.bonaventura@uniklinik-freiburg.de. 2. Department of Plastic and Hand Surgery, Medical Center - University of Freiburg, Faculty of Medicine, Comprehensive Cancer Center Freiburg, University of Freiburg, Freiburg, Germany. 3. Institute for Surgical Pathology, Medical Center, University of Freiburg, Freiburg, Germany.
Abstract
BACKGROUND: Epidermolysis bullosa is a group of rare inherited skin diseases characterized by blister formation following mechanical skin trauma. Epidermolysis bullosa is associated with increased skin cancer rates, predominantly squamous cell carcinomas, yet to our best knowledge, there is no reported case of dermatofibrosarcoma protuberans in a patient with Epidermolysis bullosa. CASE PRESENTATION: Here, we present a 26-year-old man with junctional epidermolysis bullosa, who developed a DFSP on the neck. Initial, the skin alteration was mistakenly not considered malignant, which resulted in inadequate safety margins. The complete resection required a local flap to close the defect, which is not unproblematic because of the chronic inflammation and impaired healing potential of the skin due to Epidermolysis bullosa. CONCLUSIONS: To our best knowledge, this is the first reported case of a skin-associated sarcoma in a patient with EB; however, further investigation is required to verify a correlation.
BACKGROUND:Epidermolysis bullosa is a group of rare inherited skin diseases characterized by blister formation following mechanical skin trauma. Epidermolysis bullosa is associated with increased skin cancer rates, predominantly squamous cell carcinomas, yet to our best knowledge, there is no reported case of dermatofibrosarcoma protuberans in a patient with Epidermolysis bullosa. CASE PRESENTATION: Here, we present a 26-year-old man with junctional epidermolysis bullosa, who developed a DFSP on the neck. Initial, the skin alteration was mistakenly not considered malignant, which resulted in inadequate safety margins. The complete resection required a local flap to close the defect, which is not unproblematic because of the chronic inflammation and impaired healing potential of the skin due to Epidermolysis bullosa. CONCLUSIONS: To our best knowledge, this is the first reported case of a skin-associated sarcoma in a patient with EB; however, further investigation is required to verify a correlation.
Entities:
Keywords:
Case report; Dermatofibrosarcoma protuberans; Epidermolysis bullosa; Local skin flap; Reconstructive surgery
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