The appearance is highly variable and depends on the form of progression. A distinction is made between the circumscritic (= localized, circumscribed) form, which affects only the connective tissue of the skin and is also referred to as morphaea, and progressive systemic scleroderma, which – to a very different extent – also involves the connective tissue of internal organs. In addition, one also speaks of “scleroderma-like diseases” – forms of progression that resemble scleroderma but have specific triggers. These include corresponding symptoms after stem cell transplantation for leukemia or as a result of certain substances, for example, materials after breast reconstruction or in dry cleaning workers.
Circumscribed scleroderma (CS).
This form usually starts with small red patches, mainly on the arms, legs or trunk. These enlarge very slowly, and later the skin hardens in the center, surrounded by a reddish ring. The skin foci can be band-shaped or ring-shaped, elongated, nodular or ulcerated, and white, reddish or – later – brown in color. In most cases, the spots do not exceed a certain size. If severe, there may be considerable scarring and shrinkage of the skin and subcutaneous tissue, which can lead to limited mobility, especially in the area of joints. It is not uncommon for the skin changes to occur in areas where external pressure is exerted, for example by bra straps or a waistband that is too tight. Today, it is believed that CS does not progress to a progressive form; some authors even believe that the clinical pictures are similar only because of the indurations, but otherwise have nothing to do with each other.
Progressive systemic scleroderma (PSS).
The course of the disease is highly variable – if the changes are only measurable with technical equipment in one patient, they may limit breathing, swallowing, or digestion in another. The following areas may be affected:
- Fingers and hands
- Head
- Internal organs
Fingers and hands
Most often, PSS begins on the fingers, which are initially swollen and reddened due to the inflammatory reactions. In more than 90 percent of patients, a so-called Raynaud’s phenomenon is found as a result of vascular changes, which often precedes the other symptoms by up to several years: when exposed to cold or stress, the finger arteries contract, causing the fingers to first turn white, followed by painful red or blue discoloration (cyanosis). At the fingertips, this can lead to ulceration and tissue death (rat bite necrosis), as well as thickening and pain at the cuticle. The skin thickening progressively turns into tissue atrophy. This causes the fingers to narrow and stiffen in the flexed position. Later, the changes spread to the entire hands and forearms. In severe cases, this can lead to the need to amputate fingers (limbs), for example.
Systemic scleroderma of the head
On the face, the hardening causes the mouth opening to become smaller, surrounded by folds (“tobacco pouch mouth”) and unable to be opened wide. This can cause difficulties during dental procedures, for example. Affected persons are increasingly restricted in their facial expressions (“mask face”). In addition, vascular dilatation (telangiectasia), hair loss, and lighter and more pointed noses and ears may occur in the face. The glandular connective tissue may also be affected, leading to impaired tear production in the eye with increased risk of irritation and conjunctivitis, and to restricted saliva production in the mouth with dry mouth and dysphagia.
Internal organs
The esophagus and gastrointestinal tract are frequently affected. The first sign is shortening of the lingual frenulum. Dysphagia, heartburn, and indigestion may occur. It is also not uncommon for the lungs to be affected by the proliferation of connective tissue, resulting in shortness of breath in particular. In almost half of the patients, changes in kidney and/or heart tissue also occur. Kidney weakness and high blood pressure are more frequent consequences, heart weakness and cardiac arrhythmias are less frequent. Musculature, skeletal and nervous systems can also be affected, leading to joint and muscle pain, for example. Scleroderma is therefore also classified as a rheumatic disease.A relatively mild form is CREST syndrome, an acronym of symptoms exclusive to: Calcinosis (calcification of the skin and muscles), Raynaud’s syndrome, esophagus (esophageal involvement), sclerodactyly (finger induration), and telangiectasias (vascular dilatation).
