www.celiac.nih.gov

 

 

 

 

 

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proviDer Points

Dermatitis Herpetiformis:
 

Skin Manifestation of Celiac Disease 

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. 

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. 

By permission of Mayo Foundation for Medical Education and Research. 

All rights reserved. 

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. 

2 Zone JJ. Skin manifestations of celiac disease. Gastroenterology. 

2005;128:s87–91. 

1 Rodrigo L. Celiac disease. World Journal of Gastroenterology. 

2006;12(41):6585–6593. 

www.celiac.nih.gov
 

 

proviDer Points

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 

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 

aCknowleDgments 

This fact sheet was reviewed by John J. Zone, M.D., 

University of Utah School of Medicine. 

3 Abenavoli L, Proietti I, Leggio L, et al. Cutaneous manifestations 

in celiac disease. World Journal of Gastroenterology. 2006;12(6): 

843–852. 

4 Nicolas MEO, Krause PK, Gibson LE, Murray JA. Dermatitis 

herpetiformis. International Journal of Dermatology. 2003;42:588–600. 

THe CeliaC DiSeaSe awareneSS CaMpaign 

To meet the need for comprehensive and current information about 

celiac disease, the National Digestive Diseases Information Clearinghouse, 

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 

www.celiac.nih.gov