Dermatitis herpetiformis (DH)

Overview

A long-lasting, very itchy skin condition that causes small bumps and blisters, often on the elbows, knees, buttocks, or scalp.

Doctors now recognize Dermatitis herpetiformis as a form of celiac disease that shows up in the skin rather than the gut. Eating gluten sets off the immune system, leading to IgA antibodies that mistakenly target a skin protein called transglutaminase-3. These antibodies collect in the skin and cause the rash. Many people with DH don’t notice digestive problems, but most still have changes in the small intestine that match celiac disease.

Common Symptoms

Red or skin-colored bumps, small blisters, severe itching, and burning are the hallmarks of DH. The rash usually appears in clusters on both sides of the body, most often on the elbows, knees, buttocks, back, and scalp. Scratching may cause scabs or scars. When the rash heals, it can leave areas of darker or lighter skin. Some people also develop changes in tooth enamel, such as grooves or discoloration.

Coexisting Diseases and Conditions

DH is a form of celiac disease and improves with a strict gluten-free diet. Even without gut symptoms, most people with DH have changes in the small intestine when biopsied. Other autoimmune conditions sometimes seen with DH include thyroid disease (Hashimoto’s thyroiditis or Graves’ disease), type 1 diabetes, and connective tissue diseases such as lupus. DH has also been linked to pernicious anemia and, more rarely, other autoimmune skin diseases like linear IgA disease or bullous pemphigoid.

Risk Factors and Prevalence

  • Most common in adults between ages 30 and 40, though it can appear at any age.

  • Highest prevalence in Northern Europe (especially Finland and the UK) and among people of Northern European ancestry.

  • Family history of celiac disease or DH increases risk.

  • Autoimmune thyroid disease is more common in people with DH.

Sources

  1. Sources
    1. Dermatitis Herpetiformis. (n.d.). NORD (National Organization for Rare Disorders). Retrieved July 12, 2021, from https://rarediseases.org/rare-diseases/dermatitis-herpetiformis/

    2. Dermatitis Herpetiformis | NIDDK. (n.d.). National Institute of Diabetes and Digestive and Kidney Diseases. Retrieved July 12, 2021, from https://www.niddk.nih.gov/health-information/professionals/clinical-tools-patient-management/digestive-diseases/dermatitis-herpetiformis

    3. Persechino, F., Galli, G., Persechino, S., Valitutti, F., Zenzeri, L., Mauro, A., Corleto, V. D., Parisi, P., Ziparo, C., Evangelisti, M., Quatrale, G., & Di Nardo, G. (2021). Skin Manifestations and Coeliac Disease in Paediatric Population. Nutrients13(10), 3611. https://doi.org/10.3390/nu13103611