Dermatitis herpetiformis: clinical presentations are independent of manifestations of celiac disease.

BACKGROUND Dermatitis herpetiformis (DH) is a skin manifestation of celiac disease (CD), and is often the presenting and only complaint. There are few data comparing those with DH who present only with DH and those with DH who present mainly due to CD. OBJECTIVE We compared the prevalence of features usually associated with CD in those who presented with DH with patients in whom DH was part of a typical CD presentation. METHODS A cross-sectional study of a prospectively maintained database of 1,050 patients with CD was analyzed. Only biopsy-diagnosed patients were analyzed for small bowel findings. All patients were included in the analysis of autoimmune diseases and lymphoma incidence. Small bowel biopsies were classified into mild and severe. RESULTS The prevalence of villous atrophy was significantly higher in the patients who presented with CD than in those who presented with DH alone (61.8 vs. 12.5%; p = 0.005). However, the prevalence of nutritional deficiencies, autoimmune diseases, and lymphoma occurred at a similar rate in patients with DH and patients with CD without DH. CONCLUSIONS Patients who present with CD and concurrent DH are more likely to have more severe pathology than those with predominantly DH, although nutritional deficiencies are similar between the two groups. It is important to screen for nutritional deficiencies in patients with DH, irrespective of the presence of typical CD manifestations.