As we saw in chapter " skin - mucous membranes ", ataxia telangiectasia causes accelerated aging of the skin and mucous membranes. Because of the very frequent absence of immunoglobulin IgA in patients with AT, they are less efficient as first barrier against infectious agents.
The essential doctor for apparently minor skin defects
Both the above factors are likely to explain the large number of more or less serious conditions of the skin and mucous membranes which can be seen among the patients with AT. However, there are no small pains: all must be taken seriously because of their ability to get worse. This is the role of the dermatologist. It is important that he is familiar with immune disorders and the mechanism of ataxia telangiectasia. Ideally, he should be part of a multidisciplinary team.
Dermatological evaluation
Dermatology is one of the few areas of medicine where the evolution of a disease can be visually followed. The rate of change is also an important factor, the change in appearance as well. This is why a regular auscultation through the eye of the professional seems desirable because he can determine early the danger of a defect and the actions to be taken before the problem becomes serious.
Sometimes, however, visual auscultation is not enough. It is then necessary to biopsy or, in case of doubt, to remove the lesion.
Treatments
the principles of treatments (not the treatments that only a physician should prescribe) in this paragraph relate to the common conditions encountered in the ataxia telangiectasia and exposeded in the Chapter skin - mucous membranes. The complexity of the others, the absence of treatment or even simply the fact that this chapter is not meant to be exhaustive are that they are not treated here.
Trush:
Thrush is a benign condition. Treatment requires the use of local applications of bicarbonate or drugs against fungi (antifungal).
A general treatment (tablets) is sometimes necessary when the esophagus is concerned.
Eczema:
It depends on the type of eczema. It consists of:
- For contact eczema, the suppression of contact with the allergen, but it is often difficult to obtain in practice.
- For the atopic eczema, try to obtain the removal of respiratory allergens such as dust mites.
- Regardind the allergenic foods, a special effort is required for the patient to highlight and avoid them. As for infant eczema, the goal is to make prevention by diversifying their diet.
- A special treatment is the one for the eruption. It uses antiseptics in a daily bath. The product most commonly used is potassium permanganate, or the 1% silver nitrate on the oozing lesions. But be careful because it is irritating and can aggravate skin dryness.
- Local treatment is based on corticosteroids (cortisone cream) and the hydration of the skin with emollients.
- The general treatment uses antihistamines, but also anxiolytics. Finally, we can consider in some cases as a basic treatment after an investigation allergy desensitization. For others, climate change as well as spa treatments can make a significant improvement especially when eczema is chronic.
- In case of conversion into impetigo, an antibiotic ointment should be taken, on doctor's advice of course.
Sweat sometimes aggravates eczema. It may then be necessary to avoid clothes that are too hot or too tight.
Seborrhea:
- Morning and evening cleansing of the skin with an adapted soap and careful rinsing.
- Some doctors prescribe retinoids based drugs (drugs containing derivatives of vitamin A, retinol, absolutely against-indicated in pregnant women).
- Application of cream or gel containing progesterone have been tried.
Prevention
In the general population is now well established the need to protect themselves from radiation, including ultraviolet radiation from the sun, source of premature skin aging and even cancer. This is obviously even more true in ataxia telangiectasia. (See Chapter radiation )