[When thinking to scleroderma?].
Scleroderma encompasses an heterogeneous group of autoimmune disorders characterized by an hidebound thickened skin involvement. When the changes are limited to the skin, localized scleroderma is suspected. Although the latter is most often a benign disease, it may be exceptionally associated with involvement of multiple organs, mainly the neurological system. At the opposite, systemic sclerosis is a serious disorder associated with high morbidity and even mortality and defined by an extended skin sclerosis, multiple organ involvement and general symptoms. Raynaud phenomena is nearly always present at the beginning of the disease. Identifying initial manifestations of the disease (Raynaud phenomena, diffuse non pitting edema, symmetrical polyarthritis with tendon friction rubs, dysphagia associated with mucosal telangiectasia) may allow the clinician to rapidly transfer the patient to a specialized reference center in order to organize a multidisciplinary approach and to prompt optimum therapy.
Cogan
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Service de Médecine Interne, Hôpital Erasme, Bruxelles.
D001168:Arthritis / Q000209:etiology; D001327:Autoimmune Diseases / Q000175:diagnosis; D003937:Diagnosis, Differential; D006801:Humans; D008171:Lung Diseases / Q000209:etiology; D011928:Raynaud Disease / Q000175:diagnosis / Q000503:physiopathology; D012594:Scleroderma, Localized / Q000175:diagnosis; D012595:Scleroderma, Systemic / Q000145:classification / Q000175:diagnosis* / Q000401:mortality / Q000503:physiopathology