Severe dermatitis, numerous allergies, and metabolic wasting (SAM) syndrome is a genodermatosis caused by a mutation of the DSG1 gene. A 50-year-old Japanese lady with congenital skin erosion and palmoplantar hyperkeratosis was diagnosed with SAM syndrome in this study. The woman had no allergies, a history of other diseases, or a family history of sickness. Her entire body was covered with erythema and erosions with pruritus, as well as edema in the lower limbs, according to a physical examination. The findings of the skin biopsy revealed a subcorneal split with minor acantholysis and inflammatory infiltration of the upper dermis with eosinophils. Serum albumin levels were somewhat lower, whereas thymus and activation-regulated chemokine (TARC) levels and immunoglobulin (Ig) E levels were higher. Multiple allergen simultaneous tests revealed IgE antibodies specific for 32 of the allergens.

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