British Journal of Dermatology (2021) 184, pp742–774 of-function mutations in the desmoglein 1 (DSG1) gene. This syndrome comprises severe dermatitis, multiple allergies and metabolic wasting (SAM) and can manifest as ichthyosiform erythroderma at birth. 1 Additional variable features include palmoplantar keratoderma, hypotrichosis, recurrent skin infections, malabsorption and failure to thrive. 2 Our patient was a woman in her twenties who had been diagnosed in the neonatal period with atopic dermatitis, which continued during infancy when her weight was below the third percentile. Both parents had mild palmoplantar keratoderma that had remained undiagnosed for many years. Our patient had severe palmoplantar keratoderma and disseminated dermatitis, manifesting with erythema and lichenified plaques, accompanied by severe pruritus and multiple food allergies. 3 She developed recurrent flares of erythroderma, but was otherwise in good general condition without growth retardation or metabolic wasting (body mass index 20Á2 kg m À2). The molecular basis of the SAM syndrome in our patient was represented by the homozygous DSG1 mutation c. 2659C> T, p. R887*, which led to the absence of desmoglein-1 in her skin. 3

For a long time, cutaneous manifestations were satisfactorily treated with topical calcineurin inhibitors and intensive skincare, consisting of daily bathing with antiseptics and application of emollients at least twice a day. Palmoplantar keratoderma was alleviated with potent keratolytics and mechanical removal every 3 weeks. Our patient intermittently