cqvip:Background: The risk for lymphoma is increased in both dermatitis herpetiformis (DH) and in coeliac disease. The lymphoma most associated with coeliac disease is enteropathy- associated T- cell lymphoma. Objectives: To study the occurrence and type of lymphoma in a large series of patients with DH and their first- degree relatives. Methods: The occur- rence of lymphoma was studied in 1104 patients consecutively diagnosed with DH in two university hospitals during 1969/2001. A questionnaire was sent to 341 patients to examine the occurrence of lymphoma in their 1825 first- degree relatives. To analyse whether the DH patients with lymphoma had adhered to a gluten- free diet similarly to the patients without lymphoma, two age- and sex- matched patients with DH served as controls for each index case. Data on the gluten- free diet were collected from prospectively completed dietary forms and also from medical records. Results: Eleven (1% ) patients contracted lymphoma 2- 31 years after the diagnosis of DH. Eight had B- cell- type lymphoma, two enteropathy- associated T- cell lymphoma and one remained unclassified due to missing material. Three (0.2% ) of the first- degree relatives contracted lymphoma, all B- cell type. The 11 DH patients with lymphoma had adhered to a gluten- free diet significantly less strictly than the DH controls without lymphoma. Conclusions: The present study documents that patients with DH can have both B- and T- cell lymphoma. The DH patients with lymphoma had not adhered as strictly to the gluten- free diet as the control patients without lymphoma. The occurrence of lymphoma in the first- degree relatives was lower than in the patients with DH.
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