- cells - cdc.gov
Dermatitis herpetiformis is an autoimmune disorder characterized by chronic, intensely pruritic (itchy) symmetric groups of vesicles, papules, and wheals (hives), that may occur on the elbows, knees, arms, legs, shoulders, scalp, buttocks, neck, and face. Dermatitis herpetiformis, which is also known as Duhrings’s disease, Brocq-During disease, and dermatitis multiformis, usually occurs in people with celiac disease and well as milder forms of gluten sensitivity, and less often, in people with autoimmune thyroid disorders.
Dermatitis herpetiformis has also been reported to occur in people with vitiligo, type I diabetes, Sjogren’s syndrome, dermatomyositis, and rheumatoid arthritis.
Who is Affected
Dermatitis herpetiformis has a typical onset in the late teens and early twenties, or in the third or fourth decades of life, although it can affect people of all ages. Males are affected twice as often as females, and it occurs more often in whites than in people of Asian or African descent.
Symptoms
Dermatitis herpetiformis causes small blisters, discrete papules (bumps, pimples), itchy smooth lesions resembling hives, and waxy lesions. Blisters and papules frequently appear on the face and and may cause intense itching. Clinical signs are often highly variable ranging from groups of papulovesicles with excoriations or eczema-like lesions to minimal variants of discrete redness with small water blisters or areas of small purpura (purple bruise-like lesions).
Untreated, dermatitis herpetiformis tends to wax and wane although with a constant diet of gluten, symptoms persist. It can take a few weeks to several years for symptoms to clear with a gluten-free diet with longer periods required for patients who have had symptoms for a long time before restricting gluten.
Although lesions in the oral mucosa are rare, there are reports of oral lesions occurring early in the stages of dermatitis herpetiformis. These lesions may also be caused by the apthous ulcers (oral blisters) that frequently occur in people with celiac disease.
Environmental Triggers
Environmental triggers include gluten, which is found in wheat, rye, and barley, and other grains that are contaminated with wheat during harvest. Gluten is also found in hydrolyzed vegetable protein, artificial colorings, malts, malt ales, hydrolyzed plant protein, monosodium glutamate, preservatives, modified food starches, vegetable gum, beer, and vinegar. Iodide in iodized salt and foods high in iodine and halide are suspected of causing disease flares.
Diagnosis
Biopsy of the lesions in dermatitis herpetiformis shows dermal papillary microsabscesses of neutrophilic white blood cells with deposits of immunoglobulin A (IgA) and complement at the junction of the dermal and epidermal layers of the skin.
Treatment
Treatment for dermatitis herpetiformis includes: the antibiotics dapsone and sulfapyridine, and a gluten free diet. Dapsone effectively reduces the rash within several days. However, long-term use can result in anemia. A gluten-free diet generally limits the need for continued dapsone use. Often, dapsone is used initially to reduce inflammation and symptoms are controlled, often resolving after 18 months of a gluten-free diet. However, symptoms often return over time when gluten is resumed. In autoimmune thyroid disease, reduction of thyroid antibodies offers benefits. Recent studies show the effectiveness of selenium in reducing thyroid peroxidase (TPO) antibody titers.
Although dermatitis herpetiformis usually occurs for life once it appears, permanent remission is reported to occur in 10-20 percent of patients, usually after long-term adherence to a gluten-free diet.
Resources:
Dermatitis Herpetiformis, Dermatologic Disease Database, American Osteopathic College of Dermatology, accessed May 17, 2007.
Sharon Longshore and Kenneth Tomecki, Skin Signs of Systemic Disease, August 4, 2004, Medicine Index, The Cleveland Clinic.
Nino M, Ciacci C, and Delfino M, A long-term gluten-free diet as an alternative treatment in severe forms of dermatitis herpetiformis, J Dermatolog Treat, 2007; 18(1):10-12.
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