In layman terms, scleroderma is hardening of the skin (almost as wood) or organs. It occurs in women and children more than men. The term is derived for the Greek term ‘skleros’, meaning indurate and ‘derma’, meaning hard. The existence of scleroderma has been known since the times of Hippocrates. From 18th century, this has been researched by Carlo Curzio, Giovambattista Fantonetti, and Robert H. Goetz who termed scleroderma as a systemic disease in 1945.
In scleroderma, the skin becomes very hard, ivory-coloured, diffused and stiff. It makes the person immobile in extreme cases of scleroderma. There are two types of scleroderma:
1. Localized Scleroderma– This contains Localized morphea, Morphea-lichen sclerosus et atrophicus overlap, Generalized morphea, Atrophoderma of Pasini and Pierini, Pansclerotic morphea, Morphea profunda and Linear scleroderma.
2. Systemic Scleroderma- This contains CREST (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) syndrome and Progressive systemic sclerosis.
The American College of Rheumatology (ACR) has provided us with a list of minor and major criteria’s for scleroderma.
The minor criterion are:
– Loss of substance, depression or scars visible on the finger pads. This is known as ischemia.
– There are symptoms of o Bibasilar pulmonary fibrosis which includes “a bilateral reticular pattern of linear or lineonodular densities most pronounced in basilar portions of the lungs on standard chest roentgenography.” These may take the appearance of “honeycomb lung” or “diffuse mottling”.
– Sometimes the tightening of the skin takes place only in fingers. There is thickening of the finger known as Sclerodactyly.
The major criterion are:
– Face, thorax and abdomen, neck are extremely effected.
– There are chances of proximal scleroderma wherein the skin near to metatarsophalangeal or metatarsophalangeal joints are effected by symmetric thickening.
Causes Of Scleroderma:
– Exposure to silica;
– Exposure to radiation;
– Cellular derangement;
– Endothelial cell injury;
– Fibroblast activation;
– Bleomycin and pentazocine drugs;
– Exposure to solvent liquids like benzene and carbon tetrachloride;
– Human herpes virus 5;
– Parvovirus B19