What is "scleroderma"

Scleroderma \Scler`o*der"ma\, n. [NL.] (Med.) A disease of adults, characterized by a diffuse rigidity and hardness of the skin.

1866, from Modern Latin, from Greek skleros "hard" (see sclerosis) + derma "skin" (see derma). Related: Sclerodermatous; sclerodermatic.

n. (context medicine English) A chronic systemic autoimmune disease characterized by hardening the skin or other organs through excessive deposits of collagen.

1. n. an autoimmune disease that affects the blood vessels and connective tissue; fibrous connective tissue is deposited in the skin [syn: dermatosclerosis]

2. genus of poisonous fungi having hard-skinned fruiting bodies: false truffles [syn: genus Scleroderma]

Scleroderma is a genus of fungi, commonly known as earth balls, now known to belong to the Boletales order, in suborder Sclerodermatineae. The best known species are S. citrinum and S. verrucosum. They are found worldwide. Various members of this genus are used as inoculation symbionts to colonize and promote the growth of tree seedlings in nurseries.

The name comes from the Greek sclera meaning hard and derma meaning skin.

Scleroderma, also known as systemic sclerosis, is a chronic systemic autoimmune disease characterised by hardening (sclero) of the skin (derma). In the more severe form, it also affects internal organs.

Limited scleroderma involves cutaneous manifestations that mainly affect the hands, arms and face. It was previously called CREST syndrome an acronym of the following common manifestations: calcinosis (the deposition of calcium nodules in the skin), raynaud's phenomenon (exaggerated vasoconstriction in the hands, with fingers undergoing white-blue-red color transitions in the cold), esophageal dysfunction (leading to difficulty swallowing), sclerodactyly (skin thickening on the fingers), and telangiectasias (dilated capillaries on the face, hands and mucous membranes).

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. There are no treatments for scleroderma itself, but individual organ system complications are treated.

The prognosis is generally good for limited cutaneous scleroderma persons who escape lung complications, but is worse for those with the diffuse cutaneous disease, particularly in older age and for 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%.

The cause of scleroderma is unknown. It is an autoimmune condition, in which the body's immune system attacks healthy tissues. Strong associations with certain mutations in HLA genes have been identified. Strong environmental influences have also been implicated in the etiology of scleroderma. Scleroderma was described in 1753 by Carlo Curzio ( Ospedale degli Incurabili, Naples).