The appearance is very variable and depends on the shape. A distinction is made between the circumscribed (= localized, circumscribed) form, which affects only the connective tissue of the skin and is also referred to as morphea, and the progressive systemic scleroderma, which, to varying degrees, also includes the connective tissue of internal organs.
In addition, one speaks of "scleroderma-like diseases" - forms that are similar to scleroderma, but have specific triggers. This includes related symptoms after stem cell transplantation for leukemia or as a result of certain substances, such as breast augmentation materials or dry cleaners.
Circumcripts Scleroderma (CS)
This form usually begins with small red spots, especially on the arms, legs or trunk. These enlarge very slowly, later hardened skin in the center, surrounded by a reddish ring. The skin can be banded or ring-shaped, oblong, nodular or ulcerated and of white, reddish or - later - brown color. In most cases, the spots do not exceed a certain size.
If severely affected, there may be considerable scarring and shrinkage of the skin and subcutaneous tissue which, especially in the area of joints, may impair mobility. Not infrequently, the lesions occur in places where pressure is exerted from the outside, for example by bra straps or too tight waistband.
Today it is assumed that the CS does not go into a progressive form; Some authors even think that the clinical pictures are similar only by the hardening, but otherwise have nothing to do with each other.
Progressive systemic scleroderma (PSS)
The course of the disease is extremely variable - if the changes in a patient are measurable only with technical equipment, they can restrict breathing, swallowing or digestion in another. The following areas can be affected:
- Fingers and hands
- Internal organs
Fingers and hands
Most PSS begins on the fingers, which are initially swollen and reddened by the inflammatory reactions. More than 90 percent of patients experience a so-called Raynaud's phenomenon as a result of vascular changes, often preceded by other symptoms up to several years: in cold weather or stress, the arteries of the fingers contract, causing the fingers to whiten, followed by painful redness. or blue discoloration (cyanosis).
On the fingertips, this can lead to ulceration and death of the tissue (rat bite necrosis) as well as thickening and pain on the cuticle. The thickening of the skin progressively leads to tissue loss. This causes the fingers to narrow and stiffen in flexion. Later, the changes spread to the entire hands and forearms. In severe cases, this can mean that, for example, fingers (limbs) have to be amputated.
Systemic scleroderma on the head
In the face, hardening causes the mouth opening to become smaller, surrounded by wrinkles ("tobacco pouch mouth") and can no longer be opened wide. This can be a problem for dental procedures, for example. The affected are increasingly limited in their facial expressions ("mask face"), in addition, it can lead to vascular dilation (telangiectasia), hair loss on the face, for lighter and sharpener nose and ears.
Also, the glandular connective tissue may be affected, which leads to the eye to impair the production of tears with increased risk of irritation and conjunctivitis, in the mouth to reduce salivation with dry mouth and dysphagia.
Often affected are esophagus and gastrointestinal tract. The first indication is the shortening of the tongue ribbon. It can cause dysphagia, heartburn and indigestion. The lungs are often affected by the increase in connective tissue, which in particular has shortness of breath.
In almost half of the patients also changes of kidney and / or heart tissue occur. Kidney weakness and high blood pressure are more common, less common are heart failure and cardiac arrhythmia. Musculature, skeletal and nervous systems can also be affected, resulting in joint and muscle pain, for example. Scleroderma is therefore also attributed to the diseases of the rheumatic type.
A relatively mild form is the CREST syndrome, an acronym of the symptoms that occur exclusively: calcinosis (limescale deposits in the skin and muscles), Raynaud's syndrome, esophagus (esophageal involvement), sclerodactyly (finger hardening) and telangiectasia (vascular dilations).