Chronic skin conditions with blistering


What is it about ?

Chronic blistering skin conditions are relatively rare autoimmune skin conditions. These are diseases in which the body produces antibodies against its own cells which ensure the firmness of the skin. The different layers of the skin therefore no longer adhere well to each other, which gives rise to blisters.

These are chronic diseases; relapses are therefore possible. There are several types, including dermatitis herpetiformis and bullous pemphigoid.

What is their frequency?

Chronic conditions with blistering are rare. Dermatitis herpetiformis, for example, affects 0.01 to 0.3% of the population; it is twice as common in men as in women.

How to recognize them?

Dermatitis herpetiformis (also called Duhring’s disease or celiac skin disease) is a skin disease that usually starts before the age of 40. The disease can be familial. Dermatitis means inflamed skin, herpetiformis means the rash of the skin looks like herpes. It usually manifests as a very badly itchy rash, with clear blisters clustered together on a red surface. These blisters can come in all kinds of shapes and are usually very fragile, so they open quickly when the person is scratching. The lesions are most often located on the extending surfaces of the knees and elbows, as well as on the buttocks and at the level of the intergluteal fold, but less often also at the level of the armpits, shoulders, face or scalp. . Scratching often causes scabs to form and causes sores and scarring, possibly with inflammation of the lesions. This disease is often associated withgluten intolerance (celiac disease) ; thus, about 1 in 10 people with dermatitis herpetiformis also have gastrointestinal symptoms. It is also found that the condition is associated more often with other autoimmune conditions such as thyroid disease, diabetes, lupus and other rheumatic diseases.

The pemphigoid is more common in older people (over 60). The disease usually begins with a generalized itchiness, followed by itchy, transparent blisters of varying size on a red surface. Unlike dermatitis herpetiformis, pemphigoid blisters are quite firm, do not open easily, and contain clear fluid that can be moved. These lesions are usually located on the trunk, thighs and upper arms. The mucous membranes are usually not affected.

How is the diagnosis made?

The doctor will suspect one of these conditions based on your story and symptoms. In this case, he will refer you to a skin specialist (dermatologist) for a definitive diagnosis and treatment. A skin biopsy (taking a piece of skin tissue) is indeed necessary to detect the antibodies that are at the origin of the disease.

In case of dermatitis herpetiformis, it is also necessary to have a blood test and a biopsy of the small intestine carried out by a doctor who specializes in the digestive system (gastroenterologist) to detect an association with celiac disease. A biopsy of the small intestine can confirm that there is damage to the intestinal lining (atrophy of the intestinal villi).

What can you do ?

The gluten sensitivity underlying Dermatitis Herpetiformis requires a lifelong gluten-free diet to reduce symptoms or even make them go away altogether. Even if the tests show that you do not have gluten intolerance (celiac disease), it is advisable to follow a gluten-free diet because it has a beneficial effect on the abnormalities of the skin.

Be careful to protect your skin. Dry yourself thoroughly after showering. It is best not to puncture the blisters unless they are very large. Do not remove the protective layer. Good wound care is very important to prevent infection (redness, pain and pus).

What can the doctor do?

The doctor will refer you to a specialist for treatment.

In case of dermatitis herpetiformis, the treatment is a gluten-free diet. It is better to be accompanied by a dietitian or a nutritionist doctor. Local cortisone ointments can be applied to relieve skin symptoms.

For the pemphigoid, the treatment consists of administering corticosteroids (by mouth or as an ointment / cream). Tetracyclines, which are antibiotics, can also be effective. Various immunosuppressive drugs can be considered, but will only be started under the supervision of a specialist. Sometimes pemphigoid is a side effect of medication. The doctor can determine which drug is responsible by stopping drugs suspected of being involved.

If the condition returns after a long period of time, the general practitioner may resume treatment.

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Source

Foreign clinical practice guide ‘Chronic bullous diseases (dermatitis herpetiformis, bullous pemphigoid)’ (2000), updated on 29.05.2017 and adapted to the Belgian context on 06.03.2019 – ebpracticenet