Mixed form of dermatitis herpetiformis and bullous pemphigoid

Abstract

Two cases of bullous disease had clinical and histological features suggestive of both dermatitis herpetiformis (DH) and bullous pemphigoid (BP). Immunofluorescence (IF) studies on both diseased and healthy skin tissue revealed in the one patient an intense linear deposit of IgG and C3 along the dermo-epidermal junction and in the other patient a linear deposit of IgA and C3 along the dermo-epidermal junction. No circulating anti-basement membrane antibodies were found. At the time of investigation in both cases normal jejunal mucosa was observed. Both patients carried the HLA-DRw3 antigen, which has been found associated with DH. One of them responded to treatment with diasone-sodium combined with a gluten-free diet (GFD). Combined treatment with a small dose of prednisone and diasone-sodium in addition to GFD controlled the disease of the other patient. Despite significant differences in typical cases of DH and BP, the diseases do overlap on rare occasions to form the so-called intermediate or mixed form.