Cross-sectional Study of Dermatitis Herpetiformis with Focus on Neurological Comorbidities

Dermatitis herpetiformis (DH) is a chronic autoimmune blistering skin disease strongly associated with coeliac disease. This cross-sectional study investigated the clinical, serological, and neurological characteristics of 114 DH patients (70 males, 44 females; mean age: 50.2 years; 13 newly, 101 previously diagnosed) at Semmelweis University, Budapest, between 2020 and 2023. Diagnosis was confirmed by direct immunofluorescence microscopy. Serum autoantibodies against transglutaminase 2 (TG2), 3 (TG3), and 6 (TG6) were quantified using immunoassays. While at the time of evaluation, 65.8% of patients adhered to a strict gluten-free diet (GFD), 34.2% followed an incomplete or no GFD. Autoantibodies against TG2 and TG3 were each detected in 24.6% of patients, while TG6 autoantibodies were detected in 53.5%. Neurological comorbidities were reported in 43.9%, with headache and radiculopathy being the most prevalent. Hashimoto's thyroiditis was present in 19.3%, and anaemia in 32.5% of patients. These findings, derived from a globally relevant patient cohort, underscore the systemic nature of DH and its strong association with transglutaminase autoimmunity, neurological and autoimmune comorbidities, emphasizing the need for early diagnosis, strict adherence to a GFD, and a multidisciplinary approach to optimize patient outcomes.