Cetuximab Therapy of Metastasizing Cutaneous Squamous Cell Carcinoma in a Patient with Severe Recessive Dystrophic Epidermolysis Bullosa

Vol. 219, No. 1, 2009

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Case Report

Cetuximab Therapy of Metastasizing Cutaneous Squamous Cell Carcinoma in a Patient with Severe Recessive Dystrophic Epidermolysis Bullosa
Andreas W. Arnold^a, Leena Bruckner-Tuderman^c, Christina Züger^b, Peter H. Itin^a

^aDepartment of Dermatology, University Hospital Basel, Basel, and
^bDepartment of Oncology, Kantonsspital Glarus, Glarus, Switzerland;
^cDepartment of Dermatology, University Medical Center Freiburg, Freiburg, Germany

Address of Corresponding Author

Dermatology 2009;219:80-83 (DOI: 10.1159/000218714)

Key Words

Epidermolysis bullosa dystrophica
Cetuximab, adverse reactions
Squamous cell carcinoma
Biological therapy

Abstract

Dystrophic epidermolysis bullosa (EB) is a genetic skin fragility condition with propensity to skin cancer. The severest subtype is the generalized recessive dystrophic EB (RDEB), in which mechanically induced skin blisters and erosions heal with severe scarring. A common and potentially life-threatening complication in RDEB is cutaneous squamous cell carcinoma (SCC). In spite of generally good differentiation of SCC associated with RDEB (RDEB-SCC), the tumours behave in an aggressive manner and metastasize early. Despite the high prevalence of RDEB-SCC, systematic studies on therapeutic options have not been reported. Wide surgical excision is recommended, and unresectable cases have been treated with radiotherapy. Here we report the first treatment of metastasized RDEB-SCC with the epidermal growth factor receptor antagonist cetuximab (Erbitux®). The response was favourable, and adverse events were similar to those in patients without EB. Notably, no additional negative cutaneous effects, such as severer skin or mucosal blistering, occurred.

Author Contacts

Dr. Andreas W. Arnold
Department of Dermatology, University Hospital Basel
Petersgraben 4
CH–4031 Basel (Switzerland)
Tel. +41 61 265 4095, Fax +41 61 265 4200, E-Mail aarnold@uhbs.ch

Article Information

Received: March 26, 2009
Accepted: March 27, 2009
Published online: May 13, 2009
Number of Print Pages : 4
Number of Figures : 5, Number of Tables : 0, Number of References : 11

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