Dermatitis 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. DH is found mainly in adults and is more common in men and people of northern European descent. Learn more.
Micrograph of dermatitis herpetiformis
Image by Beatriz Di Martino Ortiz, Hugo Macchi, Celeste Valiente Rebull, María Lorena Re Dominguez, Guadalupe Barboza/Wikimedia
Summary
Dermatitis Herpetiforme 4
Image by BallenaBlanca/Wikimedia
Dermatitis Herpetiforme 4
Dermatitis herpetiforme - abdomen
Image by BallenaBlanca/Wikimedia
What Is Dermatitis Herpetiformis?
Dermatitis herpetiformis is a rare, chronic, skin disorder characterized by groups of severely itchy blisters and raised skin lesions. These are more common on the knees, elbows, buttocks and shoulder blades. The slow onset of symptoms usually begins during adulthood, but children can also be affected. Other symptoms may include fluid-filled sores; red lesions that resemble hives; and itchiness, redness and burning. The exact cause of this disease is not known, but it is frequently associated with the inability to digest gluten. People with this disease are typically treated with the drug dapsone.
Source: Genetic and Rare Diseases (GARD) Information Center
Additional Materials (7)
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Dermatitis herpetiformis on the leg
Watercolour drawing of the front of the leg of a woman aged 37 years, showing several shallow ulcers covered by crusts, together with some small bullae, occurring during an attack of dermatitis, giving rise to the condition known as dermatitis herpetiformis. From an out-patient in the Department of Diseases for the Skin.
Medical Photographic Library
Keywords: Mark, Leonard Portal
Image by /Wikimedia
Dermatitis herpetiformis on the abdomen of a woman
Watercolour drawing of the front of the abdomen of a woman aged 36, suffering from dermatitis herpetiformis. Drawing shows a number of small shallow ulcers covered by yellowish crusts, and scattered irregularly over the skin. They are rather more numerous on the right side than on the left, but occur on both sides of the middle line.
Medical Photographic Library
Keywords: Mark, Leonard Portal
Image by /Wikimedia
Micrograph of dermatitis herpetiformis
Micrograph of dermatitis herpetiformis: Subepidermal vesicles, with papillary neutrophil microabcesses, with neutrophil, eosinophil and lymphocytes infiltrates in the superficial dermis.
Image by Beatriz Di Martino Ortiz, Hugo Macchi, Celeste Valiente Rebull, María Lorena Re Dominguez, Guadalupe Barboza/Wikimedia
Dermatitis Herpetiformis
Video by Medical Education for Visual Learners/YouTube
Medicoapps Masterclass - Dermatitis herpetiformis
Video by Medicoapps/YouTube
Cross-reactivity hypothesis for the onset of dermatitis herpetiformis in patients with celiac disease
Gliadin proteins in gluten are absorbed by the gut and enter the lamina propria where they need to be deamidated by tissue transglutanimase (tTG). tTG modifies gliadin into a more immunogenic peptide. Classical dendritic cells (cDCs) endocytose the immunogenic peptide and if their pattern recognition receptors (PRRs) are stimulated by pathogen-associated molecular patterns (PAMPs) or danger-associated molecular pattern (DAMPs), the danger signal will influence them to secrete IL-8 (CXCL8) in the lamina propria, recruiting neutrophils. Neutrophil recruitment results in a very rapid onset of inflammation. Therefore, co-infection with microbes that carry PAMPs may be necessary for the initial onset of symptoms in gluten sensitivity, but would not be necessary for successive encounters with gluten due to the production of memory B and T cells (discussed below). In celiac disease, tTG is treated as an autoantigen, especially in people with certain HLA-DQ2 and HLA-DQ8 alleles and other gene variants that cause atopy. tTG is up-regulated after gluten absorption. cDCs endocytose tTG-modified gliadin complexes or modified gliadin alone but they only present gliadin to CD4+ T cells on pMHC-II complexes. These T cells become activated and polarised into type I helper T (Th1) cells. Th1 cells against gliadin have been discovered, but none against tTG. A naive B cell sequesters tTG-modified gliadin complexes from the surface of cDCs in the lymph nodes (LNs) before they become endocytosed by the cDCs. The B cell receptor (membrane bound antibody; BCR) is specific to the tTG portion of the complex. The B cell endocytoses the complex and presents the modified gliadin to the activated Th1 cell's T cell receptor (TCR) via pMHC-II. Thus, the B cell presents the foreign peptide (modified gliadin) but produces antibodies specific for the self-antigen (tTG). Once the B cell becomes activated, it differentiates into plasma cells that secrete autoantibodies against tTG, which may be cross-reactive with epidermal transglutanimase (eTG). Class A antibodies (IgA) deposit in the gut. Some may bind to the CD89 (FcaRI) receptor on macrophages (M1) via their Fc region (constant region). This will trigger endocytosis of the tTG-IgA complex, resulting in the activation of macrophages. Macrophages secrete more IL-8, propagating the neutrophil-mediated inflammatory response. In dermatitis herpetiformis, the purportedly cross-reactive autoantibodies may migrate to the skin. IgA deposits may form if the antibodies cross-react with epidermal transglutanimase (eTG). Macrophages may be stimulated to secrete IL-8 by the same process as is seen in the gut, causing neutrophils to accumulate at sites of high eTG concentrations in the dermal papillae of the skin. Neutrophils produce puss in the dermal papillae, generating characteristic blisters. IL-31 accumulation at the blisters may intensify itching sensations. Memory B and T cells may become activated in the absence of PAMPs and DAMPs during successive encounters with tTG-modified gliadin complexes or modified gliadin alone, respectively. Symptoms of dermatitis herpetiformis are often resolved if patients avoid a gluten-rich diet.
Image by LBNBM OBIBT/Wikimedia
Coeliac disease and dermatitis herpetiformis in the UK: occurrence and pregnancy outcome
Video by Coeliac UK/YouTube
Sensitive content
This media may include sensitive content
Dermatitis herpetiformis on the leg
/Wikimedia
Dermatitis herpetiformis on the abdomen of a woman
/Wikimedia
Micrograph of dermatitis herpetiformis
Beatriz Di Martino Ortiz, Hugo Macchi, Celeste Valiente Rebull, María Lorena Re Dominguez, Guadalupe Barboza/Wikimedia
1:33
Dermatitis Herpetiformis
Medical Education for Visual Learners/YouTube
2:13
Medicoapps Masterclass - Dermatitis herpetiformis
Medicoapps/YouTube
Cross-reactivity hypothesis for the onset of dermatitis herpetiformis in patients with celiac disease
LBNBM OBIBT/Wikimedia
41:02
Coeliac disease and dermatitis herpetiformis in the UK: occurrence and pregnancy outcome
Coeliac UK/YouTube
Causes
Rheumatoid Factor Molecules (IgM, IgG, and IgA)
Image by TheVisualMD
Rheumatoid Factor Molecules (IgM, IgG, and IgA)
The rheumatoid factor test detects the presence of a protein called rheumatoid factor (RF), an antibody of the type known as immunoglobulin M (IgM, top molecule), which is produced by the immune system. (There are five classes of immunoglobulins produced by the immune system; the job of these antibodies is to identify "foreign" cells or substances, but sometimes the antibodies make mistakes and instead attack the body's own tissues.) In addition to RF, other antibodies, such as cyclic citrullinated peptide (CCP), are also used to diagnose RA. Although the IgM class is the most common RF, immunoglobulin G (IgG, lower right) and immunoglobulin A (IgA, lower left) RFs have been reported.
Image by TheVisualMD
What Causes Dermatitis Herpetiformis?
Dermatitis herpetiformis 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.
Family studies show that 5 percent of first-degree relatives of a person with DH will also have DH. An additional 5 percent of first-degree relatives of a person with DH will have celiac disease. Various other autoimmune diseases are associated with DH, the most common being hypothyroidism.
Source: National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)
Additional Materials (2)
Immunglobulin A as a Dimer
Immunglobulin A as a Dimer
Immunglobulin A as a Dimer
1 H-chain
2 L-chain
3 J-chain
4 secretory component
Image by User:Lennert B
Immunoglobulin A
Image by OpenStax College
Immunglobulin A as a Dimer
User:Lennert B
Immunoglobulin A
OpenStax College
Symptoms
Dermatitis Herpetiforme 5
Image by BallenaBlanca/Wikimedia
Dermatitis Herpetiforme 5
Dermatitis herpetiforme - manos
Image by BallenaBlanca/Wikimedia
What Are the Symptoms of Dermatitis Herpetiformis?
Dermatitis herpetiformis is characterized by small, clustered papules and vesicles that erupt symmetrically on the elbows, knees, buttocks, back, or scalp. The face and groin can also be involved. A burning sensation may precede lesion formation. Lesions are usually scratched off by the time a patient comes in for a physical exam, and the rash may appear as erosions and excoriations.
Patients with DH may also experience dental enamel defects to permanent teeth, which is another manifestation of celiac disease. Less than 20 percent of people with DH have symptoms of celiac disease.
Source: National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)
Additional Materials (4)
Dermatitis Herpetiforme 3
Dermatitis herpetiforme - piernas
Image by BallenaBlanca/Wikimedia
Dermatitis herpetiforme - mano izquierda 1
Dermatitis herpetiforme en manos
Image by BallenaBlanca/Wikimedia
Dermatitis herpetiformis
Image by Madhero88/Wikimedia
Dermatitis-herpetiformis
en:Dermatitis herpetiformis
Image by Madhero88/Wikimedia
Dermatitis Herpetiforme 3
BallenaBlanca/Wikimedia
Dermatitis herpetiforme - mano izquierda 1
BallenaBlanca/Wikimedia
Dermatitis herpetiformis
Madhero88/Wikimedia
Dermatitis-herpetiformis
Madhero88/Wikimedia
Diagnosis
Skin punch biopsy
Image by BruceBlaus
Skin punch biopsy
Skin punch biopsy
Image by BruceBlaus
How Is Dermatitis Herpetiformis Diagnosed?
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. However, histology may reveal only excoriation due to the intense itching that patients experience.
Blood tests for antiendomysial or anti-tissue transglutaminase antibodies may also suggest celiac disease. Blood tests for epidermal transglutaminase antibodies are positive in more than 90 percent of cases. All of these tests will become negative with prolonged adherence to a gluten-free diet.
A positive biopsy and serology confirm DH and should be taken as indirect evidence of small bowel damage. A biopsy of the small bowel is usually not needed for DH diagnosis. However, if clinical signs of gastrointestinal disease are evident on examination, further workup may be required. Whether or not intestinal damage is evident, a gluten-free diet should be implemented because the rash of DH is gluten sensitive.
Source: National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)
Treatment
Dapsona 50mg
Image by Docnoriega/Wikimedia
Dapsona 50mg
Tableta de Dapsona 50 mg, comparación de tamaño de la tableta.
Image by Docnoriega/Wikimedia
How Is Dermatitis Herpetiformis Treated?
The sulfone dapsone can provide immediate relief of symptoms. For patients who cannot tolerate dapsone, sulfapyridine or sulfamethoxypyridazine may be used, although these medications are less effective than dapsone. A strict gluten-free diet is the only treatment for the underlying disease. Even with a gluten-free diet, medication therapy may need to be continued from a few months to 2 years.
DH can go into remission, which is defined as absence of skin lesions and symptoms of DH for more than 2 years while not taking sulfones or other treatments and not adhering to a gluten-free diet. Cohort studies showing DH remission provide support for reducing sulfone therapy and weaning from a gluten-free diet in patients with well-controlled DH.
Source: National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)
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Dermatitis Herpetiformis
Dermatitis 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. DH is found mainly in adults and is more common in men and people of northern European descent. Learn more.