LAUSR

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 an option, whereas radiotherapy is contraindicated because it may exacerbate the develoment of keratoacanthomas [2-4]. In July 2016, a 78-year-old woman was referred to our unit for the management of radiotherapy-resistant mutilating keratoacanthomas of the face. Since the age of 30, her medical history revealed numerous keratoacanthomas in sun-exposed areas which were surgically treated. Her sister had a similar medical history. In July 2015, the patient developed a keratoacanthoma of the nose which was resected. In September 2015, a relapse on the nose and nasal dorsum required a new surgergical procedure. In October, a third surgery was required due to a relapse. Histological examination showed an aggressive keratoacanthoma with perineural invasion. Adjuvant radiotherapy was performed between January and February 2016 for a total of 33 sessions. From March to June 2016, the patient developed four papules on the nose, one on the white lip and one on the right cheek, which were surgically managed subsequently with a full-thickness skin graft of the right cheek Histological examination of the right cheek papule revealed a well-differentiated squamous epithelium with keratinous plugs, acanthosis and dermal invasion, corresponding to a keratoacanthoma (figure 1A). In July, multiple nodules were