Dermatitis herpetiformis Duhring is a chronic disease of the skin. It is characterized by the formation of vesicles on various parts of the body and is accompanied by severe itching. Many patients with Duhring’s disease suffer from the inflammatory small bowel disease celiac disease. Dermatitis herpetiformis Duhring is the skin manifestation of this clinical picture. Treatment options include certain ointments and lotions and a lifelong diet.
What is dermatitis herpetiformis Duhring?
Dermatitis herpetiformis Duhring or Duhring’s disease is a chronic disease of the skin. Characteristic of the clinical picture are blister formations on the inner sides of the arms and legs. The disease is rather rare. It occurs more frequently in middle age. Men are more frequently affected than women. The disease is chronic. Its symptoms can reappear again and again. In many cases, the intensity of the symptoms diminishes over the years.
Causes
The causes of the disease are not yet fully understood. A genetic predisposition cannot be ruled out. The disease can be triggered by iodine or gluten. People suffering from this skin disease should definitely be tested for celiac disease, as these two diseases are associated with each other. Celiac disease, also called gluten-induced enteropathy, is an intolerance to the gluten protein found in various grains. Those affected show characteristics of an autoimmune disease and an allergy. Consumption of gluten causes chronic inflammation of the mucosa in the small intestine. This hypersensitivity reaction persists for life. Dermatitis herpetisformis Duhring is the skin manifestation of the disease.
Symptoms, complaints, and signs
Initially, many affected individuals develop hives-like rashes. Sufferers complain of severe itching in the affected areas of the skin. In addition, clearly recognizable reddening of the skin is typical of the clinical picture. Blisters gradually form on the reddened areas of skin. These can extend to neighboring skin tissues and form crusts. The nodules on the skin surface are a few millimeters wide and develop into the blisters characteristic of the skin disease after about seven to ten days. Typical body sites for blister formation are the abdomen, thighs, extensor sides of both arms, buttock region, sacrum, and shoulder girdle. Blister formation on the mucous membranes is observed rather rarely. In only a few cases are the neck, back or face affected. The blisters have an initially clear, later cloudy content and may be filled with blood. A characteristic feature of the disease is a sensitive reaction to the trace element iodine. In most cases, if a patient is exposed to iodine, this leads to a drastic worsening of symptoms. In addition, patients suffering from dermatitis herpetiformis Duhring have celiac disease. This can be asymptomatic or with the appearance of increased fatty stools.
Diagnosis
Because of the rather nonspecific symptoms, especially at the beginning of the disease, dermatitis herpetiformis Duhring is often recognized late. To make the diagnosis, similar conditions must first be ruled out. These include the so-called erythema exsudativum multiforme. This disease describes an inflammation of the dermis. To confirm the diagnosis of dermatitis herpetiformis Duhring, a biopsy is taken from healthy skin tissue. In order to be able to establish the diagnosis with certainty, it is necessary to detect so-called granular IgA deposits. The tissue taken for this purpose must be examined in a special laboratory. Since many patients suffering from Duhring’s disease are also affected by celiac disease, a diagnosis must also be made with regard to this clinical picture. For this purpose, certain antibodies are determined in the blood. In addition to the detection of gliadin and endomysium antibodies, patients are tested for transglutaminase IgA antibodies. In addition, a biopsy of the small intestine must be performed to confirm the diagnosis. Most patients suffering from Duhring’s disease show a rather harmless course of celiac disease. However, a few may develop massive fatty stools, osteoporosis and vitamin deficiency.
Complications
Dermatitis herpetiformis Duhring is a rare incurable autoimmune skin disease. The symptom is not age-related and causes men to become ill more often than women. Especially in Scandinavia, Hungary as well as England and Ireland the disease appears more often than in Germany. Therefore, there is a suspicion that dermatitis herpetiformis Duhring is a hereditary disease. Furthermore, there may be a connection with intestinal diseases due to gluten intolerance. Furthermore, tumor formation as well as infections and contact with iodine seem to promote the symptom. Dermatitis herpetiformis Duhring initially manifests itself with reddening of the skin. If the symptom is ignored by the affected person or treated incorrectly on his or her own initiative, painful swellings and unpleasant itching develop. The symptom affects the limbs, but also appears on the head, upper body, sacrum and buttocks, but very rarely on the mucous membranes. As a regular complication, the affected areas become inflamed and form abscesses. These enlarge into fluid-filled skin blisters. The blisters become encrusted due to the intensifying burning itch. The course develops chronically. With timely medical clarification, complications can be largely ruled out. In medical therapy, a lifelong gluten-free diet is recommended. Sulfone-based or cortisone-based medications and lotions are administered according to tolerance.
When should you see a doctor?
A visit to the primary care physician is recommended for hives-like rashes and other signs of dermatitis herpetiformis Duhring. Redness of the skin, severe itching and blisters are also symptoms that indicate a serious skin disease and must be clarified accordingly. If swellings and abscesses form as a result of incorrect or inadequate treatment, it is best to visit the nearest clinic. This is especially true if the blisters fill with fluid or become crusted. In the case of a chronic course, regular visits to the doctor are indicated, which is why the disease is best diagnosed and treated at an early stage. Dermatitis herpetiformis Duhring is particularly common after tumor disease as well as infections and contact with iodine. If these factors apply to you, you should consult a doctor immediately. In addition to the family doctor, the dermatologist or a specialist in internal medicine can also be contacted. In medical emergencies – for example, if sepsis develops or severe bleeding occurs – the medical emergency service is the right contact.
Treatment and therapy
The itchy regions of the skin are usually treated with the sulfone dapsone. Usually, most patients are symptom-free within a short time after this application. Dapsone may have side effects. The therapy should therefore be supported by regular blood value checks. Since the disease can be aggravated by iodine, iodine-rich foods should be avoided, especially at the beginning. These include certain types of sea fish. The affected skin areas and itching can also be treated with soothing ointments or lotions. In some cases, short-term treatment with cortisone preparations is induced. Permanent special care of the skin is not necessary in dermatitis herpetiformis Duhring. Since in most cases celiac disease underlies dermatitis herpetiformis Duhring, this disease must be treated with the same therapy. For those affected, this means avoiding gluten, a gluten protein, for the rest of their lives. Even the smallest amounts can drastically worsen the course of the disease. Gluten is the gluten protein found in certain types of cereals. Sufferers must therefore avoid the cereals wheat, rye, barley, spelt, kamut, einkorn, emmer and green spelt. Products made from these grains, such as couscous or pasta, must also be avoided. Corn, millet, rice, quinoa, soy and buckwheat are expressly permitted. Products that do not contain gluten are labeled “gluten-free” and can be consumed without concern.
Outlook and prognosis
With a consistent change in diet, patients with dermatitis herpetiformis Duhring have a good prognosis. In many cases, further medical care is usually not necessary.In the case of more severe symptoms, medications help to bring about relief from the skin changes or itching. However, these are associated with side effects. Regular blood monitoring is therefore advisable to avoid triggering further diseases. The patient can achieve lifelong freedom from the symptoms of dermatitis herpetiformis Duhring on his own and self-determined initiative. For this, the food intake must be checked. The entire diet must be gluten-free to maintain a good prognosis. No product containing even the smallest amount of gluten should be consumed for a favorable healing outlook. Several months to a year of a complete gluten-free diet is required by the body for recovery. A relapse of symptoms occurs immediately if gluten has been consumed. Therefore, at any time during life, there is a risk of experiencing a recurrence of symptoms. In particular, there is an increased risk when food is consumed in restaurants. Without abstaining from gluten, the patient may succumb to a chronic course of the disease. The general state of health is weakened and the susceptibility to disease is greatly increased. The quality of life decreases and significantly more difficult health conditions occur in everyday life.
Prevention
Prevention of the disease is possible only to a limited extent. Because dermatitis herpetiformis Duhring is associated with celiac disease and with heavy exposure to iodine, it is conceivable that a lifelong special diet could reduce the likelihood of the skin disease.
Follow-up
In dermatitis herpetiformis Duhring, there are usually no special options or measures of aftercare available to the affected person. In this case, the affected person is primarily dependent on complete medical treatment to alleviate the symptoms and avoid further complications, as this also cannot lead to independent healing. Only an early diagnosis with prompt treatment can prevent the symptoms from worsening. In most cases, treatment of dermatitis herpetiformis Duhring is carried out with the help of medications, ointments or creams. The affected person should always follow the doctor’s instructions and also pay attention to the regularity and dosage of the medication. In case of any questions or uncertainties, a doctor should always be contacted first. If the symptoms of dermatitis herpetiformis Duhring do not subside within a few days, a doctor should be consulted in any case. Since the disease can also lead to psychological upsets or depression, intensive discussions with friends or family are often very useful. In severe cases, however, professional counseling by a psychologist is advisable. As a rule, dermatitis herpetiformis Duhring does not reduce the life expectancy of the patient.
What you can do yourself
Every affected person can do some things to improve his or her quality of life despite the difficult diagnosis. Self-discipline is important, as the symptom of itching should not be indulged. Scratching leads to open sores into which germs can penetrate. In addition, skin damage increases. Therefore, for his own sake, the affected person should take care to use ointments when the itching is too strong and therefore not be tempted. The skin changes lead to an optical blemish. This can reduce self-esteem. It is advisable to inform oneself comprehensively about the disease and to find possibilities so that the aesthetic aspirations of the affected person can be maintained. Conversations and tips with other sufferers or close relatives often help. It can be helpful to find out about their attitude and perception of the skin changes. In many cases, the affected person feels the optical blemish worse than it is perceived by the close environment. A change in cognitive patterns can therefore alleviate emotional suffering. Although it has not yet been sufficiently researched scientifically, it seems that a special diet could be beneficial for one’s recovery. This must be lifelong and can contribute to a significant improvement in symptoms and increase one’s own well-being.
