Scleroderma

Scleroderma
MercMorphea.JPG
A type of localized scleroderma known as morphea
SpecialtyRheumatology
Usual onsetMiddle age[1]
TypesLocalized, systemic scleroderma[2]
CausesUnknown[2]
Risk factorsFamily history, certain genetic factors, exposure to silica[3][4][5]
Diagnostic methodBased on symptoms, skin biopsy, blood tests[6]
Differential diagnosisMixed connective tissue disease, systemic lupus erythematosus, polymyositis, dermatomyositis[1]
TreatmentSupportive care[1]
MedicationCorticosteroids, methotrexate, non-steroidal anti-inflammatory drugs (NSAIDs)[2]
PrognosisLocalized: Normal life expectancy[7]
Systemic: Decreased life expectancy[3]
Frequency3 per 100,000 per year (systemic)[3]

Scleroderma is a group of autoimmune diseases that may result in changes to the skin, blood vessels, muscles, and internal organs.[2][6] The disease can be either localized to the skin or involve other organs in addition to the skin.[2] Symptoms may include areas of thickened skin, stiffness, feeling tired, and poor blood flow to the fingers or toes with cold exposure.[1] One form of the condition, known as CREST syndrome, classically results in calcium deposits, Raynaud's syndrome, esophageal problems, thickening of the skin of the fingers and toes, and areas of small dilated blood vessels.[1]

The cause is unknown; however, some suspect it may be due to an abnormal immune response.[2] Risk factors include family history, certain genetic factors, and exposure to silica.[3][4][5] The underlying mechanism involves the abnormal growth of connective tissue which is believed to occur as a result of the body's immune system attacking healthy tissues.[6] Diagnosis is typically based on a person's symptoms and may be supported by a skin biopsy or blood tests.[6]

While there is no cure, treatments may improve symptoms.[2] Medications used include corticosteroids, methotrexate, and non-steroidal anti-inflammatory drugs (NSAIDs).[2] Outcomes depend on the extent of disease.[3] Those with localized disease generally have a normal life expectancy.[7] In those with systemic disease typical life expectancy is about 11 years from onset.[3] Death is often due to lung, gastrointestinal, or heart complications.[3]

About 3 out of 100,000 people per year develop the systemic form.[3] The condition most often begins in middle age.[1] Women are more often affected than men.[1] Scleroderma was likely first described in 1753 by Carlo Curzio[8] and then well documented in 1842.[9] The term is from the Greek "sklerosis" meaning "hardness" and "derma" meaning "skin".[6]

Signs and symptoms

Arm of a person with scleroderma showing skin lesions

Potential signs and symptoms include:[10][11][12]

Other Languages
Alemannisch: Sklerodermie
العربية: تصلب الجلد
български: Склеродерма
Deutsch: Sklerodermie
Ελληνικά: Σκληρόδερμα
español: Esclerodermia
euskara: Esklerodermia
français: Sclérodermie
한국어: 피부경화증
Bahasa Indonesia: Skleroderma
italiano: Sclerodermia
עברית: סקלרודרמה
Кыргызча: Склеродермия
magyar: Szkleroderma
Nederlands: Sclerodermie
日本語: 強皮症
polski: Twardzina
português: Esclerodermia
slovenčina: Sklerodermia
svenska: Sklerodermi
中文: 硬皮病
Lingua Franca Nova: Scleroderma