Description, Causes and Risk Factors:
Alternative Name: Duhring disease.
Dermatitis herpetiformis is relatively more frequent than the autoimmune bullous dermatoses belongs to bullous pemphigoid group. It prevalence is estimated to be about 10 to 39 cases per 100,000. Males are affected slightly more frequently than females. The disease is less frequent in Blacks. Dermatitis herpetiformis occurs mainly between the age of 20 and 55, but occasionally seen in children, usually after age of 5.
The cause of DH is allergy to gluten, a protein found in wheat and some other grains. Usually allergies, likes hives and hay fever, are made by the body's IgE system. This can be treated with pills and shots. DH is different and is an allergy of the IgA system. IgA is an antibody produced in the lining of the intestines. The usual allergy treatments are useless.
It has been hypothesized that DH is the result of an immunologic response to chronic stimulation of the gut mucosa by dietary gluten with subsequent activation of cutaneous endothelial cells and circulating inflammatory cells, including neutrophils. Several chemicals have been associated with induction of DH, including potassium iodide and cleaning solutions.
Individuals with such a disease almost always have intestinal disease signs (celiac disease). Such individuals also may progress thyroid disease. Small blister generally develops slowly, especially, on the back, buttocks, and knees. In some cases, such blisters can break out on the neck or face. Burning and itching may be severe.
Hormonal factors may also play a role in the pathogenesis of DH. Androgens have a suppressive effect on immune activity, including decreased autoimmunity, and androgen deficient states may be a potential trigger for DH exacerbation.
The symptoms are intense burning, stinging and itching around the elbows, knees, scalp, buttocks and back. More locations can also be affected and the severity can vary.
Differential diagnosis may include:
Linear IgA bullous dermatosis.
Transient acantholytic dermatosis.
Diagnosis is usually based upon the appearance of the skin lesions. Your doctor may perform a skin biopsy (i.e., removal of a small piece of skin or other tissue) for laboratory evaluation to assist in diagnosis. Your doctor may also order blood tests to check for certain immune markers that may aid in the diagnosis.
There is a very effective treatment available for dermatitis herpetiformis. A gluten-free diet is very difficult to achieve; however, limiting intake of wheat, barley, or rye products can lessen the symptoms.
Dapsone is first choice, and reduces the itch within a day or two. Cautions and blood monitoring requirements should be noted. For those intolerant or allergic to dapsone, the following may be used:
Ultrapotent topical steroids.
Risks and benefits of the drug must be carefully discussed with your dermatologist.
NOTE: The above information is educational purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.
DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.
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