Pyoderma gangrenosum associated with selective hereditary IgA deficiency.
A case of pyoderma gangrenosum is described in a girl aged 4. The condition was associated with selective IgA deficiency. The father and the 2 brothers suffered from the same deficiency (autosomal dominant transmission). Treatment with prednisolone and clofazimine produced an excellent clinical response.
D002675:Child, Preschool; D002991:Clofazimine / Q000627:therapeutic use; D005260:Female; D005734:Gangrene; D006801:Humans; D017098:IgA Deficiency; D011239:Prednisolone / Q000627:therapeutic use; D011711:Pyoderma / Q000188:drug therapy / Q000235:genetics*; D012867:Skin / Q000473:pathology
D011239:Prednisolone; D002991:Clofazimine