Toxic epidermal necrolysis from graft-vs-host disease. Occurrence in a patient with thymic hypoplasia.
An infant with diarrhea, failure to thrive, and a seborrhea-like skin eruption was thought to have fatal familial Leiner's syndrome. Treatment with nonirradiated plasma was followed by graft-vs-host disease and fatal toxic epidermal necrolysis; thymic hypoplasia was found at autopsy. Accurate diagnosis of immunodeficiency syndromes is essential to avoid potentially harmful therapy.
D004062:DiGeorge Syndrome / Q000150:complications*; D006087:Graft vs Host Reaction; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000150:complications*; D007223:Infant; D008297:Male; D010949:Plasma / Q000276:immunology; D013262:Stevens-Johnson Syndrome / Q000209:etiology*; D065227:Transfusion Reaction