Dermatitis Herpetiformis: Skin Manifestation of Celiac Disease

On this page:

• Symptoms
• Causes
• Diagnosis
• Treatment
• Acknowledgments

Dermatitis herpetiformis (DH) is a chronic, intensely itchy, blistering skin manifestation of gluten sensitive enteropathy, commonly known as celiac disease. DH affects 15 to 25 percent of people with celiac disease.¹ Most people with DH have no other symptoms of celiac disease. DH is found mainly in adults and is more common in men and people of northern European descent.

Symptoms

DH is characterized by small, clustered papules and vesicles that erupt symmetrically on the elbows, knees, buttocks, back, or scalp. Men may also have oral or genital lesions. A burning sensation may precede lesion formation. Lesions are usually scratched off by the time a patient comes in for a physical exam.

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Causes

DH is caused by the deposit of immunoglobulin A (IgA) in the skin, which triggers further immunologic reactions resulting in lesion formation. DH is an external manifestation of an abnormal immune response to gluten, in which IgA antibodies form against the skin antigen epidermal transglutaminase.

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Diagnosis

A skin biopsy is the first step in diagnosing DH. Direct immunofluorescence of clinically normal skin adjacent to a lesion shows granular IgA deposits in the upper dermis. Histology of lesional skin may show microabscesses containing neutrophils and eosinophils but may reveal only excoriation due to the intense itching patients experience.

Skin biopsies performed on the affected skin are nearly always positive for IgA deposition.² Blood tests for antiendomysial or anti-tissue transglutaminase antibodies may also suggest celiac disease.

A positive biopsy and serology confirm celiac disease. In the absence of these results, patients should be referred to a gastroenterologist for a definitive diagnosis via intestinal biopsy.³

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Treatment

Dapsone, a sulfone, provides immediate relief of symptoms. For patients who cannot tolerate dapsone, sulfapyridine or sulfamethoxypyridazine may be used, although these drugs are less effective than dapsone. A strict gluten-free diet is the only treatment for the underlying disease. Even with a gluten-free diet, drug therapy may need to be continued for 1 to 2 years.⁴

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Acknowledgments

This fact sheet was reviewed by John J. Zone, M.D., University of Utah School of Medicine.

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The Celiac Disease Awareness Campaign

To meet the need for comprehensive and current information about celiac disease, the National Digestive Diseases Information Clearinghouse (NDDIC), a service of the National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK), launched the Celiac Disease Awareness Campaign. The Awareness Campaign is the result of the combined ideas and efforts of the professional and voluntary organizations that focus on celiac disease, along with the NIDDK, the National Institutes of Health, and the Centers for Disease Control and Prevention.

Download this fact sheet and learn more about the Awareness Campaign at www.celiac.nih.gov.

Celiac Disease Awareness Campaign
c/o National Digestive Diseases Information Clearinghouse
2 Information Way
Bethesda, MD 20892–3570
Phone: 1–800–891–5389
TTY: 1–866–569–1162
Fax: 703–738–4929
Email: nddic@info.niddk.nih.gov
Internet: www.digestive.niddk.nih.gov

The National Digestive Diseases Information Clearinghouse (NDDIC) is a service of the National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK). The NIDDK is part of the National Institutes of Health of the U.S. Department of Health and Human Services. Established in 1980, the Clearinghouse provides information about digestive diseases to people with digestive disorders and to their families, health care professionals, and the public. The NDDIC answers inquiries, develops and distributes publications, and works closely with professional and patient organizations and Government agencies to coordinate resources about digestive diseases.

Publications produced by the Clearinghouse are carefully reviewed by both NIDDK scientists and outside experts.

This publication is not copyrighted. The Clearinghouse encourages users of this publication to duplicate and distribute as many copies as desired.

This publication may contain information about medications. When prepared, this publication included the most current information available. For updates or for questions about any medications, contact the U.S. Food and Drug Administration toll-free at 1–888–INFO–FDA (463–6332) or visit www.fda.gov. Consult your doctor for more information.

NIH Publication No. 09–6432
January 2009