Scleroderma
| Scleroderma | |
|---|---|
 |
|
| Morphea | |
| Classification and external resources | |
| Specialty | Rheumatology |
| ICD-10 | L94.0-L94.1, M34 |
| ICD-9-CM | 701.0 710.1 |
| MedlinePlus | 000429 |
Scleroderma is a long term autoimmune disease that results in hardening of the skin. In the more severe form, it also affects internal organs. The cause is unknown. The underlying mechanism involves the body's immune system attacking healthy tissues. There is a strong association with certain mutations in HLA genes. Environmental factors have also been implicated.
There are two main types of the disease: the localized form (called localized scleroderma, limited scleroderma, or morphea) and the systemic form (called systemic scleroderma, diffuse scleroderma, generalized scleroderma, or systemic sclerosis). Limited scleroderma involves mainly the skin of the hands, arms and face. Diffuse scleroderma is rapidly progressing and affects a large area of the skin and one or more internal organs, frequently the kidneys, esophagus, heart and/or lungs. This form of scleroderma can be quite disabling. A limited cutaneous subtype of it is called CREST syndrome.
There are no treatments for scleroderma itself, but organ system–specific complications are treated. Outcomes are generally good for limited scleroderma of the skin when there are no lung complications. They are worse for those with the diffuse skin disease, particularly in older age and males. Death occurs most often from lung, heart and kidney complications. In diffuse cutaneous disease, five-year survival is 70% and 10-year survival is 55%. Scleroderma was first described in 1753 by Carlo Curzio of Ospedale degli Incurabili, Naples.
Potential signs and symptoms include:
Scleroderma is caused by genetic and environmental factors. Mutations in HLA genes seem to play a crucial role in the pathogenesis of some cases (but not all), likewise silica, aromatic and chlorinated solvents, ketones, trichloroethylene, welding fumes and white spirits exposure seems to contribute to the condition in a small proportion of affected persons.
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