[Systemic sclerosis in childhood].
Systemic sclerosis is a multisystem disease of unknown cause, characterized by inflammation, vascular and fibrotic changes with excessive accumulation of connective tissue. The lesions may involve the skin and various internal organs (kidney, lung, gastrointestinal tract and heart). The first symptom is usually Raynaud's phenomenon, followed by skin changes; at the beginning the skin is swollen and oedematous, and then becomes thick, taut, shiny and atrophic. The prognosis of SS depends mainly on the severity of visceral involvement. The treatment of SS consists of drugs that improve the microcirculation and reduce collagen proliferation, such as calcium-antagonists (nicardipine) and D-Penicillamine.
Falcini
F
F
Dipartimento di Pediatria, Università degli Studi, Firenze, Italia.
Trapani
S
S
Taccetti
G
G
Tafi
L
L
D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D000367:Age Factors; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000627:therapeutic use; D002648:Child; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D008297:Male; D010396:Penicillamine / Q000627:therapeutic use; D011379:Prognosis; D012595:Scleroderma, Systemic / Q000175:diagnosis / Q000188:drug therapy
D000305:Adrenal Cortex Hormones; D000894:Anti-Inflammatory Agents, Non-Steroidal; D010396:Penicillamine
Sclerosi sistemica in età pediatrica.