Celiac disease evolving into dermatitis herpetiformis in patients adhering to normal or gluten-free diet

Abstract

Objective. Dermatitis herpetiformis (DH) is a cutaneous form of celiac disease affecting ∼17% of celiac disease patients. The aim was to determine how often celiac disease precedes the development of DH, and what is the impact of gluten-free diet (GFD) in this phenotype change. Material and methods. Our prospectively collected DH series from 1970 comprised 514 patients. We analyzed all DH patients who at least 2 years earlier had been diagnosed with celiac disease. DH diagnosis was confirmed by showing immunoglobulin A deposits in dermis. Serological and small bowel mucosal findings were analyzed, and the strictness of GFD treatment before and after the diagnosis of DH was evaluated. Results. Twenty (4%) DH patients had a prior diagnosis of celiac disease. The median time interval between celiac disease and DH detection was 9.5 years. Before DH appeared 4 patients had been on a normal gluten-containing diet, 10 had dietary lapses on a GFD, and 6 were on a strict GFD. Celiac autoantibodies were positive in 7 out of 19 patients, and 5 out of 7 undergoing small bowel biopsy had partial villous atrophy. Following DH diagnosis the rash was controlled after a median of 6 months on a strict GFD. Conclusions. Patients with celiac disease may develop DH by time. This is most often an indicator of poor adherence to GFD, and a rigorous dietary intervention is necessary. In the majority of cases, DH will be detected without prior celiac disease diagnosis, but the physicians should recognize this phenotype alteration.

Key Words:

• celiac disease
• dermatitis herpetiformis
• follow-up
• gluten-free diet

Acknowledgments

This study was financially supported by the grants from the Academy of Finland, the Sigrid Juselius Foundation, Finnish Medical Foundation, Seppo Nieminen Fund and the Competitive State Research Financing of the Expert Responsibility area of Tampere University Hospital (Grant number 9P060, 9P008 and 9R018) and Seinäjoki Central Hospital (VTR16).

Declaration of interest: The authors report no conflict of interest. The authors alone are responsible for the content and writing of the paper.