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Friday, February 10, 2012

Duhring Disease

Duhring Disease or Dermatitis herpetiformis is characterized by intensely itchy, chronic papulovesicular eruptions, usually distributed symmetrically on extensor surfaces (buttocks, back of neck, scalp, elbows, knees, back, hairline, groin, or face). The blisters vary in size from very small up to 1 cm across.
The condition is extremely itchy, and the desire to scratch can be overwhelming.This sometimes causes the sufferer to scratch the blisters off before they are examined by a doctor. Intense itching or burning sensations are sometimes felt before the blisters appear in a particular area.

Untreated, the severity of DH can vary significantly over time, in response to the amount of gluten ingested.
Dermatitis herpetiformis symptoms typically first appear in the early years of adulthood between 20 and 30 years of age.
Although the first signs and symptoms of dermatitis herpetiformis are intense itching and burning, the first visible signs are the small papules or vesicles that usually look like red bumps or blisters. Sometimes they appear on the face and along the hairline, and, on occasion, on the shoulders, the lower end of the spinal column, and within the mouth[citation needed]. The rash rarely occurs on other mucous membranes, excepting the mouth or lips. The symptoms range in severity from mild to serious, but they are likely to disappear if gluten ingestion is avoided and appropriate treatment is administered.
Dermatitis herpetiformis symptoms are chronic, and they tend to come and go, mostly in short periods of time. Sometimes, these symptoms may be accompanied by symptoms of coeliac disease, commonly includingabdominal pain bloating or loose stool and fatigue.
The rash caused by dermatitis herpetiformis forms and disappears in three stages. In the first stage, the patient may notice a slight discoloration of the skin at the site where the lesions appear. In the next stage, the skin lesions transform into obvious vesicles and papules that are likely to occur in groups. Healing of the lesions is the last stage of the development of the symptoms, usually characterized by a change in the skin color. This can result in areas of the skin turning darker or lighter than the color of the skin on the rest of the body. Because of the intense itching, patients usually scratch, which can lead to the formation of crusts.

Diagnosis

Dermatitis herpetiformis is often misdiagnosed, being confused with drug eruptions, contact dermatitis, dishydrotic eczema (dyshidrosis), and even scabies.
Diagnosis is confirmed by a simple blood test for IgA antibodies, and by a skin biopsy in which the pattern of IgA deposits in the dermal papillae, revealed by direct immunofluorescence, distinguishes it from linear IgA bullous dermatosis and other forms of dermatitis. These tests should be done before the patient starts on a gluten-free diet, otherwise they might produce false negatives. If the patient has already started a gluten-free diet (there is a strong relationship with DH and coeliac sprue), it might be necessary for them to come off it for some weeks before the tests can be done reliably.

Treatment

Dermatitis herpetiformis responds well to medication and changes in diet.
Dapsone is an effective treatment for most patients. DH responds to dapsone so quickly (itching is significantly reduced within 2–3 days) that this response may almost be considered diagnostic. However, dapsone treatment has no effect on any intestinal damage (see coeliac disease) that might be present.
Therefore, a strict gluten-free diet must also be followed, and this will usually be a lifelong requirement. This will reduce any associated intestinal damage and the risk of other complications. After some time on a gluten-free diet, the dosage of dapsone can usually be reduced or even stopped, although this can take many years.
Dapsone is an antibacterial, and its role in the treatment of DH, which is not caused by bacteria, is poorly understood. It can cause adverse effects on the blood, so regular blood monitoring is required.
Dapsone is the drug of choice, but, for patients unable to tolerate dapsone for any reason, the following can be tried, although they are less effective:

1 comment:

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