MALIGNANCY AND SURVIVAL IN DERMATITIS-HERPETIFORMIS - A COMPARISON WITH CELIAC-DISEASE

Background-Dermatitis herpetiformis is a lifelong, gluten sensitive sk in disease. Patients with dermatitis herpetiformis, similar to patient s with coeliac disease not adhering to a gluten free diet, seem to hav e increased risk for lymphoma. Aims-This study looked at the occurrenc e of malignancy and survival of patients with dermatitis herpetiformis and compared the results with those seen in patients with coeliac dis ease or in the general population. Patients-A total of 305 adult patie nts with dermatitis herpetiformis diagnosed at the University Hospital of Tampere in 1970-1992 were studied. Most patients started a gluten free diet and at the end of the study 93% of the patients were adherin g to the diet. A control group comprised 383 adult patients with coeli ac disease, 81% of them adhered to a gluten free diet, 6% had a normal diet, and in 13% the diet history remained unknown. Methods-The occur rence of malignant diseases and survival of the patients were assessed up to the end of 1993. Standardised incidence ratios (SIR) with 95% c onfidence intervals were used for the malignant diseases. The survival of the patients was compared with that of the general population. Res ults-Thirteen (4.3%) patients with dermatitis herpetiformis developed 14 malignant disorders during the follow up (SIR 1.25; 95% confidence intervals 0.68 to 2.09). A non-Hodgkin's lymphoma occurred in four pat ients with dermatitis herpetiformis, significantly more than expected (SIR 10.3; 2.8-26.3). Thirteen (4.3%) patients with dermatitis herpeti formis died during the follow up but there was no increased general mo rtality. In coeliac disease, 13 (3.4%) patients developed malignancy ( SIR 1.16; 0.62 to 1.97), 31 (8.1%) patients died but the survival rate did not differ from that in the general population. Conclusions-The i ncidence of non-Hodgkin's lymphoma was significantly increased in pati ents with dermatitis herpetiformis. The results also confirm that the patients with dermatitis herpetiformis treated mainly with a gluten fr ee diet have no increased general mortality.