John Libbey Eurotext

European Journal of Dermatology

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Multiple and aggressive keratoacanthomas associated with Ferguson-Smith syndrome, successfully treated by cetuximab and cisplatin Article à paraître

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Auteurs
1 Service de dermatologie, Centre Hospitalier de Valence, Valence, France
2 Service de chirurgie otorhinolaryngologique, Centre Hospitalier de Valence, Valence, France
3 Service de génétique, Centre Hospitalier de Valence, Valence, France
4 Service d’anatomopathologie, Centre Hospitalier de Valence, Valence, France
5 Service de dermatologie, Hôpitaux Drôme Nord, Romans sur Isère, France

Multiple self-healing squamous epitheliomas of the skin, or Ferguson-Smith syndrome, is a rare autosomal dominant genodermatosis due to loss-of-function mutations in the tumour suppressor gene TGFBR1. Patients develop multiple well-differentiated keratoacanthomas from early adulthood, usually on sun-exposed areas. These keratoacanthomas resolve spontaneously, occasionally leaving scars and rarely causing mutilation [1]. Surgery is an effective treatment. In difficult-to-manage cases, acitretin is [...]