[How to recognize scleroderma].
Systemic sclerosis is a multisystem disorder characterized by the presence of connective tissue sclerosis, vascular abnormalities concerning small-sized arteries and the microcirculation, and autoimmune changes. This disease is usually preceded by Raynaud's phenomenon. Cutaneous changes are useful for diagnosis. Sclerosis is initially proeminent on hands with frequently pitted scars. Telangiectases and calcium deposits are mainly observed in a good prognosis subset of the disease (CREST) characterized by the association of limited skin involvement and anti-centromere antibodies with possible late onset of pulmonary hypertension. The bad prognosis subset is associated with diffuse cutaneous sclerosis, pulmonary fibrosis, gastrointestinal disease, myocardial involvement and anti-topo-isomerase I antibodies. Histological skin changes are similar to those observed in morphea. In morphea, there is only localized sclerosis of the skin with highly variable aesthetic disability. The condition may be subdivided clinically into the following types: circumscribed plaques, linear morphea and frontoparietal lesions. The last two forms may be associated with atrophy of the underlying anatomic structures. Many other diseases may have a scleroderma-like appearance. In these diseases, nail folds abnormalities, visceral involvement and immunological changes are usually different from those observed in systemic scleroderma.
Francès
Camille
C
Service de médecine interne Groupe hospitalier La Pitié-La Salpêtrière 75651 Paris. camille.frances@psl.ap-hop-paris.fr
Ayoub
Nakhle
N
Barete
Stéphane
S
D003937:Diagnosis, Differential; D006801:Humans; D011379:Prognosis; D011928:Raynaud Disease; D012595:Scleroderma, Systemic / Q000150:complications / Q000175:diagnosis* / Q000473:pathology; D012598:Sclerosis; D012867:Skin / Q000473:pathology*