Lymphoma in patients with dermatitis herpetiformis and their first-degree relatives.
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.


OBJECTIVE
To study the occurrence and type of lymphoma in a large series of patients with DH and their first-degree relatives.


METHODS
The occurrence 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.
Hervonen
K
K
Department of Dermatology, Tampere University Hospital, PO Box 2000, FIN-33521 Tampere, Finland. Kaisa.Hervonen@pshp.fi
Vornanen
M
M
Kautiainen
H
H
Collin
P
P
Reunala
T
T
D000328:Adult; D000368:Aged; D016022:Case-Control Studies; D003874:Dermatitis Herpetiformis / Q000150:complications* / Q000178:diet therapy; D005260:Female; D005983:Glutens / Q000008:administration & dosage; D006801:Humans; D016393:Lymphoma, B-Cell / Q000209:etiology / Q000235:genetics; D008228:Lymphoma, Non-Hodgkin / Q000209:etiology* / Q000235:genetics; D016399:Lymphoma, T-Cell / Q000209:etiology / Q000235:genetics; D008297:Male; D008875:Middle Aged; D010349:Patient Compliance
D005983:Glutens