What is scleroderma?Scleroderma - an unusual form of arthritis, is described as a "hardening of the skin", because it is one of the main manifestations in this disease. Scleroderma - Diffuse disease of connective tissue, which in some areas of the skin, and sometimes throughout the skin, scar tissue is deposited. With the spread of the scar tissue is formed not only in the skin, but also in the locomotor system, lungs, digestive tract, heart and kidneys.
There are two main forms of disease - systemic and limited.
Systemic sclerodermaSystemic scleroderma forms are also divided into two forms - a common systemic sclerosis and limited systemic sclerosis. Systemic sclerosis implies a form of scleroderma with extensive skin lesions and also with the defeat of the various body systems, such as the esophagus, joints, intestines, lungs, heart and kidneys.
Course of the disease depends on the severity and / or progression of the disease. It can range from widespread skin tightening (diffuse form) to a more limited lesion of the skin (CREST - syndrome).
Diffuse form of scleroderma - a different term that describes systemic sclerosis, and skin changes in many parts of the body. Thick, glossy (shiny) skin, resembling ivory, may be on the trunk and upper extremities, as well, and on the face, chest and lower extremities.
The face masklike, amimichnoe, ears and nose thinned ("bird" nose), thin lips, difficult mouth opening, around which the wrinkles are formed ("purse-string" mouth), impaired coupling of age, impaired the growth of eyelashes and eyebrows. Very often, this condition leads to disability.
The specific lesions are joint changes in scleroderma. They are manifested by inflammation with a fairly rapid violation of the mobility of the joints and the formation of the so-called contractures, ie stiffness due to the irreversible proliferation of fibrous tissue in the joint and loss of elasticity of the capsule. There are complaints of morning stiffness, mild transient pain, a sense of "crunch" in the joints. Affects the joints in the area of cutaneous manifestations. Joint damage is also one of the causes of disability of patients.
The defeat of the internal organs (intestines, lungs, heart and kidney) shows a violation of their functions, gradually increasing failure of these organs, which in turn complicates the full life of patients.
CREST-syndromeCREST - an acronym formed from the first five letters of the most prominent manifestations of this form of scleroderma:
- Calcification: accumulation of calcium salts under the skin;
- Raynaud syndrome: affects the circulation of small blood vessels, especially in the legs and arms. It is a phenomenon of phase change in color of fingers and toes, and in some cases also the tip of the nose, lips, tongue tip, the ear. Initially, the attack appears whitening of the skin, accompanied by a feeling of numbness and coldness. This phase is followed by a phase of cyanosis blueness of the skin. At the end of the attack appear red with a sensation of heat, "crawling", stress and soreness;
- Dysfunction of the esophagus: violation of the motor function of the lower third of the esophagus in patients seen belching, swallowing;
- Sclerodactyly: the fingers are thin and inactive, with large nails and clubbing, and they are difficult to compress into a fist (sausage-shaped fingers);
- Telangiectasias: expansion of the capillaries and small vessels of the skin, resembling a star, on the face, the upper half of the torso and other areas.
Limited forms of sclerodermaThey include a ring-shaped scleroderma (morphea), limited scleroderma, which begins with an inflammatory stage, followed by the appearance of a small number of rounded lesions, and linear scleroderma, which is shown a group of seal skin, often limited to one area. Foci in its development through three stages: the spots, plaques, atrophy. Disease begins quietly, with the appearance of one or more of lilac-pink round or oval patches of varying size. Gradually the center of the spot turns white and begins to thicken. Lesion is gradually turning into a very dense plaque yellowish-white color with a smooth shiny surface. Hair fall out on the plaques, the figure is smoothed flow and excretion of fat terminated. Then gradually the affected area atrophies and sinks.
In the future, for the O-ring is usually characterized by scleroderma process activity subsided after several years of illness, with the softening of the skin and pigmentation (coloring), and sunken areas of the skin. For linear scleroderma is characterized by a longer period of time to improve the process, and damaged skin can accumulate deposits of calcium salts. Linear scleroderma usually affects babies.
In severe cases there may be loss of limb muscles (muscle atrophy). They become stiff, weak and painful, losing its function.
What do we know about the causes and treatment of scleroderma?No one knows what causes scleroderma, but it is prevalent in all countries and ethnic groups, from infants to the elderly. Scleroderma is four times more common in women than in men, and its frequency increased in both sexes with age. Perhaps, the disease has a genetic predisposition.
While scleroderma is a rare, this disease is actually more common than muscular dystrophy, multiple sclerosis and many other well-known diseases.
There is no specific treatment for scleroderma, there are many treatments that can manage fibrosis of the skin and internal organs and help control symptoms. As with other forms of arthritis, there are many things people can do to manage the symptoms of the disease.
Patients must comply with special care to protect your hands, face and ears from the cold or overly active cooling. Other measures include the food in the form of a balanced diet, avoiding alcohol and smoking, maintaining normal weight and avoidance of fatigue, stress, exposure to vibration.
It is also used anti-inflammatory and restorative tools, restore the lost functions of the locomotor apparatus, in the presence of focal infection with antibiotics.
What can you do?If you have yourself or your family notice any of the above symptoms, you should seek medical advice immediately. Undertake independent attempts to treat dangerous. You can aggravate the disease. The sooner you contact the person, the more likely to prevent the further development of the disease.
If someone is sick in the family, it is better to check all the next of kin of a specialist, because the recorded cases of the disease within one family.