Malignant disease in dermatitis herpetiformis

Abstract

An increased incidence of gastrointestinal malignancies, particularly lymphoma, has been documented in several studies of celiac disease (l-3). In one series of 210 patients followed up in Birmingham, England, a statistically significant increase in deaths from malignancy was observed (1). Among the patients were 13 with lymphoma and 6 with gastrointestinal carcinoma. Another study in the United Kingdom identified 259 malignancies in 235 patients with celiac disease; 133 malignancies were lymphomas and the most common site of the lesion was the small intestine (3). After the association between dermatitis herpetiformis (DH) and celiac disease was recognized, it seemed natural to look also at the occurrence of lymphoma and other malignancies in DH. Several case reports and small series were published, and in 1983 Leonard et al (4) confirmed statistically that DH patients had a risk for developing lymphoma similar to that of patients with celiac disease. Although it is now well established that linear immunoglobulin A (IgA) disease is not caused by gluten (5,6), it remains of interest that the incidence of lymphoproliferative malignancies also seems to be increased in this disease (4,7).