Spontaneous chronic urticaria

Karoline Krause

As soon as the symptoms of spontaneous urticaria persist for more than 6 weeks, spontaneous chronic urticaria is spoken of. Then the probability starts to decrease that the disease will disappear again in a short time. Urticaria of this type, with wheals and/or angioedema appearing daily, weekly or more seldom, can last for years (and sometimes decades) and can often not be tolerated without the regular administration of drugs. There are many underlying causes for spontaneous urticaria.

In the case of severe, therapy resistant and long persisting spontaneous urticaria a thorough search into the triggers and causes is recommended. The aim is to identify them and (in as far as possible) to eliminate them. The reason behind urticaria being caused by many different causes and triggers is that mast cells can be activated by a large number of factors. These triggering factors can be categorised in 3 subgroups.

  •  auto-reactive urticaria: intolerance to the body’s own substances
  • infection urticaria: reactions to chronic focus of infection or inflammation which do not necessarily entail further symptoms, e.g. in the digestive tract
  • intolerance-related urticaria; hyper sensitivity to food additives such as colour, aroma or preservative substances and food rich in histamines or so-called histamine liberators. These substances are also found in absolutely natural food.

Drugs can also cause such incompatibility reactions, e.g. acetylsalicylic acid, among others contained in Aspirin® and Thomapyrin® but also other pain killers and drugs against absolutely different symptoms.
About one third of all the cases of spontaneous chronic urticaria can be classified in one of the groups named above. Other causes of spontaneous chronic urticaria, as for instance allergies to food, are very rare. In about one third of the patients no underlying cause can be found despite thorough examination.

In every case of a spontaneous chronic urticaria it must first be ascertained that really such a type of urticaria is concerned. Then the severity of the disease and the impairment of life quality should be determined. In addition blood analysis should be made to find out whether there are signs of a severe inflammation. If there is a severe, therapy-resistant and long lasting spontaneous chronic urticaria, underlying causes should be looked for, i.e. a check made whether an auto-reactive urticaria, and infection urticaria or an intolerance urticaria is involved.

Auto-reactive urticaria can be diagnosed quickly and safely using the autologous serum test (ASST=autologous skin serum test). For this purpose the fresh full blood serum obtained is injected in the skin of the patient in a type of allergy test. If a wheal appears on this spot, this indicates that the patient reacts “allergically” to his own serum.

Particularly infections of the gastro intestinal tract caused by helicobacter pylori, an infection in the ear, nose and throat or tooth roots are particularly frequently the cause of infection urticaria. The diagnostics of an infection urticaria concentrate on the search for infections in these areas. The diagnosis of intolerance urticaria is made by a special diet lasting 4 weeks. In patients with intolerance urticaria this leads to a clear improvement all the way to full recovery. Allergy tests are not sensible for this type of food intolerance.

Treatment aims at curing spontaneous chronic urticaria. Where possible this should ensue by curing the spontaneous chronic urticaria, i.e. by eliminating the underlying cause. For instance in the case of an infection urticaria the focus should be eliminated and in the case of intolerance urticaria the triggering substances should be avoided. If this kind of curative therapy approach is not possible or is ineffective, symptomatic treatment is administered. The international guideline for chronic urticaria recommends a three-phase therapy plan. The base therapy consists of the ingestion of non-sedating antihistamines, the so-called antihistamine of the 2nd generation. Should no relief of the symptoms be experienced the dose of the antihistamine is increased. If symptoms nevertheless appear, Omalizumab should be administered (antibodies against immune globulin E). Alternatively the immunosuppressant drug Ciclosporine A should be used (suppresses the immune-reaction of the body) or the anti-inflammation drug Montelukast (a leukotriene antagonist) is administered. Advice is explicitly against the ongoing administration of cortisone as extended therapy. Cortisone may only be administered for a few days as short-term therapy when the patient is suffering severe bouts. Cortisone in the form of ointments or creams on the other hand is ineffective when urticaria is involved. Advice is explicitly against this application. The treatment recommendations of the guideline are based on a strictly scientific evaluation of clinical studies. Therefore the experimental treatment processes are not considered in guidelines. Nevertheless the experience gained by many physicians with one or the other alternative treatment method has been good, for instance with auto-blood therapy. However, great care must be taken in general when turning to alternative therapies.

All too often absolutely ineffective or even dangerous forms of therapy are offered at considerable costs. In the case of severe spontaneous chronic urticaria, e.g. when mucous membranes swell accompanied by difficulties in swallowing and respiratory distress, patients should always have an emergency kit at hand to keep serious urticaria episodes under control. Usually such emergency kits contain a fast-acting cortisone preparation and an antihistamine.

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