Defects in desmoglein 1 (DSG1) or desmoplakin (DSP), structural desmosomal proteins, cause severe dermatitis, multiple allergies, and metabolic wasting (SAM) syndrome, a severe multisystemic disease resembling IPEX syndrome at least clinically106,107. The gene discussed is DSG1; the disease is immune dysregulation-polyendocrinopathy-enteropathy-X-linked syndrome.