Dermatitis Herpetiformis CDAC PP 508

Provider Points
Dermatitis Herpetiformis:
Skin Manifestation of Celiac Disease
D ermatitis herpetiformis (DH) is a chronic,

intensely itchy, blistering skin manifestation of
gluten-sensitive enteropathy, commonly known as
celiac disease. DH is a rash that affects about
10 percent of people with celiac disease.1 DH is
found mainly in adults and is more common in
men and people of northern European descent; DH
is rarely found in African Americans and Asian
Americans.2 By permission of Mayo Foundation for Medical Education and Research.
All rights reserved.
symptoms disease.4 Various other autoimmune diseases are associated

Dermatitis herpetiformis is characterized by small, clustered with DH, the most common being hypothyroidism.
papules and vesicles that erupt symmetrically on the elbows,
knees, buttocks, back, or scalp. The face and groin can also be Diagnosis
involved. A burning sensation may precede lesion formation. A skin biopsy is the first step in diagnosing DH. Direct
Lesions are usually scratched off by the time a patient comes immunofluorescence of clinically normal skin adjacent to
in for a physical exam, and the rash may appear as erosions a lesion shows granular IgA deposits in the upper dermis.
and excoriations. Histology of lesional skin may show microabscesses
Patients with DH may also experience dental enamel defects containing neutrophils and eosinophils. However, histology
to permanent teeth, which is another manifestation of may reveal only excoriation due to the intense itching that
celiac disease. Less than 20 percent of people with DH have patients experience.
symptoms of celiac disease.3 Blood tests for antiendomysial or anti-tissue transglutaminase
antibodies may also suggest celiac disease. Blood tests for
Causes epidermal transglutaminase antibodies are positive in more
Dermatitis herpetiformis is caused by the deposit of than 90 percent of cases.5 All of these tests will become
immunoglobulin A (IgA) in the skin, which triggers further negative with prolonged adherence to a gluten-free diet.
immunologic reactions resulting in lesion formation. DH is an A positive biopsy and serology confirm DH and should be
external manifestation of an abnormal immune response to taken as indirect evidence of small bowel damage. A biopsy
gluten, in which IgA antibodies form against the skin antigen of the small bowel is usually not needed for DH diagnosis.
epidermal transglutaminase. However, if clinical signs of gastrointestinal disease are
Family studies show that 5 percent of first-degree relatives of evident on examination, further workup may be required.2
a person with DH will also have DH. An additional 5 percent Whether or not intestinal damage is evident, a gluten-free
of first-degree relatives of a person with DH will have celiac diet should be implemented because the rash of DH is gluten
sensitive.4
www. celiac. nih. g o v

Provider Points
TreaTmenT
The sulfone dapsone can provide immediate relief of The celiac disease awareness campaign
symptoms. For patients who cannot tolerate dapsone, The National Institutes of Health Celiac Disease Awareness
sulfapyridine or sulfamethoxypyridazine may be used, Campaign provides current, comprehensive, science-based
although these medications are less effective than dapsone. information about the symptoms, diagnosis, and treatment of celiac
disease, also known as celiac sprue, nontropical sprue, and gluten-
A strict gluten-free diet is the only treatment for the
sensitive enteropathy. The Awareness Campaign is an initiative of
underlying disease. Even with a gluten-free diet, medication the National Digestive Diseases Information Clearinghouse, a service
therapy may need to be continued from a few months to of the National Institute of Diabetes and Digestive and Kidney
2 years. Diseases.
DH can go into remission, which is defined as absence of Download this publication and learn more about the Awareness
skin lesions and symptoms of DH for more than 2 years while Campaign at www.celiac.nih.gov.
not taking sulfones or other treatments and not adhering celiac disease awareness campaign
to a gluten-free diet. Cohort studies showing DH remission c/o National Digestive Diseases Information Clearinghouse
provide support for reducing sulfone therapy and weaning 2 Information Way
from a gluten-free diet in patients with well-controlled DH.6 Bethesda, MD 20892–3570
Phone: 1–800–891–5389
TTY: 1–866–569–1162
references Fax: 703–738–4929
1. Ruiz AR. Celiac sprue. The Merck Manual website. Email: [email protected]
www.merckmanuals.com/professional/gastrointestinal_ Internet: www.digestive.niddk.nih.gov
disorders/malabsorption_syndromes/celiac_disease.html. The National Digestive Diseases Information Clearinghouse (NDDIC)
Updated August 2012. Accessed December 4, 2013. is a service of the National Institute of Diabetes and Digestive and
2. Bolotin D, Petronic-Rosic V. Dermatitis herpetiformis: Kidney Diseases (NIDDK). The NIDDK is part of the National
Institutes of Health of the U.S. Department of Health and Human
part II. Diagnosis, management, and prognosis. Journal of
Services. Established in 1980, the Clearinghouse provides
the American Academy of Dermatology. 2011;64(6):1027–1033. information about digestive diseases to people with digestive
3. Alonzo-Llamazares J, Gibson LE, Rogers RS. Clinical, disorders and to their families, health care professionals, and the
pathologic, and immunopathologic features of dermatitis public. The NDDIC answers inquiries, develops and distributes
publications, and works closely with professional and patient
herpetiformis: review of the Mayo Clinic experience.
organizations and Government agencies to coordinate resources
International Journal of Dermatology. 2007;46(9):910–919. about digestive diseases.
4. Sapone A, Bai JC, Ciacci C, et al. Spectrum of gluten- This publication is not copyrighted. The Clearinghouse encourages
related disorders: consensus on new nomenclature users of this publication to duplicate and distribute as many copies
and classification. BioMed Central website. as desired.
www.biomedcentral.com/1741-7015/10/13.
This publication may contain information about medications and,
Published February 7, 2012. Accessed July 26, 2013.
when taken as prescribed, the conditions they treat. When
5. Rose C, Armbruster FP, Ruppert J, Igl B-W, Zillikens D, prepared, this publication included the most current information
Shimanovich I. Autoantibodies against epidermal available. For updates or for questions about any medications,
transglutaminase are a sensitive diagnostic marker in contact the U.S. Food and Drug Administration toll-free at 1–888–
INFO–FDA (1–800–463–6332) or visit www.fda.gov. Consult your
patients with dermatitis herpetiformis on a normal or
health care provider for more information.
gluten-free diet. Journal of the American Academy of
Dermatology. 2009;61(1):39–43.
6. Paek SY, Steinberg SM, Katz SI. Remission in dermatitis
herpetiformis: a cohort study. Archives of Dermatology.
2011;147(3):301–305.
acknowledgmenTs
Publications produced by the Clearinghouse are carefully
reviewed by both NIDDK scientists and outside experts. This NIH Publication No. 13–6432
September 2013
publication was reviewed by John J. Zone, M.D., Professor and
Chairman, Department of Dermatology, University of Utah The NIDDK prints on recycled paper with bio-based ink.
School of Medicine.
www.celiac.nih.gov
www. celiac. nih. g o v

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Provider Points

Skin Manifestation of Celiac Disease

D ermatitis herpetiformis (DH) is a chronic,

symptoms disease.4 Various other autoimmune diseases are associated

www. celiac. nih. g o v

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