EGFR inhibition for metastasized cutaneous squamous cell carcinoma in dystrophic epidermolysis bullosa
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(2019) 14:278
LETTER TO THE EDITOR
Open Access
EGFR inhibition for metastasized cutaneous squamous cell carcinoma in dystrophic epidermolysis bullosa Andrea Diociaiuti1, Holger Steinke2, Alexander Nyström2, Agnes Schwieger-Briel2,3, Frank Meiss2, Christina Pfannenberg4, Leena Bruckner-Tuderman2, Juri Ruf5, Rita De Vito6, May El Hachem1 and Dimitra Kiritsi2* Abstract Dystrophic epidermolysis bullosa (DEB) is a hereditary skin fragility disorder, characterized by trauma-induced blistering followed by soft tissue fibrosis. One of the most feared complications is the early development of aggressive cutaneous squamous cell carcinomas (SCC). For patients with locally advanced or metastasized SCCs treatment with cetuximab, a monoclonal antibody against epidermal growth factor receptor (EGFR), has been proposed and so far, treatment of five DEB patients with cetuximab has been published. With this report, we extend the spectrum of EB patients treated with cetuximab by adding two additional patients. Taking together all DEB cases treated with cetuximab, we propose that cetuximab should be administered as early as possible, since it seems to be more efficient and is accompanied by rather mild adverse effects. We also show that EGFR is frequently expressed in DEB-associated SCCs, although there were noticeable differences in the level of expression, which may influence responsiveness to EGFR-targeting therapies. Although only limited experiences with targeted cancer treatments in EB exist, such reports highlight the treatments’ effects in this specific cohort and assist our therapeutic decisions. Keywords: Collagen VII, Skin fragility, Skin cancer, Cetuximab, Immunotherapy Dystrophic epidermolysis bullosa (DEB) is a hereditary skin fragility disorder, characterized by trauma-induced blistering followed by soft tissue fibrosis [1]. One of the most feared complications is the development of aggressive cutaneous squamous cell carcinomas (SCCs) [2, 3]. Patients suffering from the most severe generalized recessive DEB subtype (RDEB-gen sev) have the highest risk (rising up to 90.1% by the age of 55) [4]. Metastatic SCCs also represent the most common cause of death in adults with RDEB [4]. Moreover, although SCCs are usually well differentiated, they tend to relapse [5]. The standard treatment of EB-associated SCC is wide local excision. In some cases amputation is necessary [6]. Treatment with cetuximab, a monoclonal antibody against epidermal growth factor receptor (EGFR), has * Correspondence: [email protected] 2 Department of Dermatology, Medical Center – University of Freiburg, Faculty of Medicine, University of Freiburg, Hauptstrasse 7, 79104 Freiburg, Germany Full list of author information is available at the end of the article
been proposed for patients with advanced or metastasized SCCs [6, 7]. Although not every determinant of cetuximab response or resistance has been identified, this treatment is associated with better responses, if EGFR is expressed in the tumor [8]. Besides the fact that no co
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