The word comes from ancient Greek and means “hard skin”. Scleroderma is a rare inflammatory rheumatic disease from the group of collagenoses, which can take on mild and severe, life-threatening forms. The disease affects the small blood vessels and connective tissue.
This is where collagen is deposited, which manifests itself as hardened skin foci. Scleroderma is an autoimmune (Greek autos = self) disease, which is why specific proteins (autoantibodies) are detectable in the blood. There are various forms of the disease, whereby either only the skin is affected (localized scleroderma), while in other forms the internal organs such as the gastrointestinal tract, lungs, kidneys or heart are also affected (systemic scleroderma).
Classification of scleroderma
Localized scleroderma occurs in three forms: Morphea: coarse foci with too little or too much pigment on the inside and surrounded by a redness on the outside (erythema), mainly on the trunk Generalized morphea: like morphea, but confluent and more widespread, the face is free Linear scleroderma: band- or channel-shaped foci, mainly located on the extremities and head Systemic scleroderma exists in two forms Diffuse scleroderma: distributed throughout the body, spreading rapidly, internal organs are affected early Limited scleroderma initially reduced blood circulation in individual fingers (initial Raynaud’s phenomenon), then infestation of the extremities and face, later of the internal organs, often in connection with the so-called CREST syndrome (C = calcinosis, a calcification in the skin; R = Raynaud’s phenomenon, see above; E = (o)esophageal motility disorder, a movement disorder of the esophagus; S = sclerodactyly, a hardening of the skin of the fingers with functional impairment of the fingers; T = telangiectasia, a local dilatation of the capillary vessels of the skin)
Causes of scleroderma
The exact cause of the disease is still unknown. A familial occurrence has been described sporadically. Also the increased incidence of systemic scleroderma in coal and gold miners has been reported.
At the molecular level, sometimes increased levels of so-called HLA antigens of the type DR1, DR2 or DR5 occur. There is also much evidence of a cell-mediated autoimmune reaction that leads to damage of the inner wall of the blood vessels (endothelial damage). Acquired genetic alterations are also common.
However, a causal connection with the above mentioned influences and scleroderma could not be established so far. The diagnosis can be made by laboratory chemistry. Antinuclear antibodies (ANA) are elevated in more than 95% of people with scleroderma.
These are proteins produced by the body itself that attack the body’s own cell nuclei. If one tests only for “ANA” in general, this is relatively unspecific. ANA can also be positive, for example, in rheumatoid arthritis.
Therefore one looks somewhat more exactly and selects completely certain ANAs, for example Anti-Scl70, which is increased with systemic Sklerodermie. In CREST syndrome, anti-centromere antibodies can be used to diagnose the syndrome, as these can be found in 70-90% of patients with the syndrome. The blood count may be anaemic, as iron deficiency can occur in the intestine. In the case of kidney infestation, elevated serum creatinine may be found, as well as blood or protein in the urine.
