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sclerodermie (aandoening)
sclerodermie
Scleroderma
A rare autoimmune connective tissue disorder characterized by abnormal hardening of the skin and sometimes other organs. It is classified into two main forms: localized scleroderma and systemic sclerosis (SSc), the latter comprising three subsets; diffuse cutaneous SSc (dcSSc), limited cutaneous SSc (lcSSc) and limited SSc (lSSc). Localized scleroderma is the cutaneous form of scleroderma characterized by fibrosis of the skin causing cutaneous plaques (morphea) or strips (linear scleroderma). Systemic sclerosis (SSc) is a generalized disorder characterized by fibrosis and vascular obliteration in the skin and organs, particularly, lungs, heart, and digestive tract. The exact cause of scleroderma is unknown. The disease originates from an autoimmune reaction, which leads to localized overproduction of collagen. In some cases, the condition is associated with exposure to chemicals. Other suggested causes include genetic and infectious mechanisms.
Id267874003
StatusPrimitive
Finding sitestructuur van bindweefsel
Pathological processauto-immuunproces
SNOMED CT to Orphanet simple map801
SNOMED CT to ICD-10 extended map
TargetM34.9
RuleTRUE
AdviceALWAYS M34.9
CorrelationSNOMED CT source code to target map code correlation not specified
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