• K Gensch, S Gudowius, T Niehues, and A Kuhn.
• Hautklinik, Heinrich-Heine-Universität, Düsseldorf.
• Hautarzt. 2005 Oct 1;56(10):925-36.

AbstractConnective tissue diseases are a heterogeneous group of chronic multisystem inflammatory disorders including systemic lupus erythematosus (SLE), progressive systemic sclerosis (PSS), dermato- (DM) and polymyositis (PM), mixed connective tissue disease (MCTD), and Sjögren's syndrome (SS). Patients can present with similar clinical features, particularly during the first onset of symptoms, which frequently makes the diagnosis of a specific disease difficult. The incidence of connective tissue diseases is much lower in children than adults; however, the clinical picture is more variable. Clinical signs, such as fatigue, fever, or weight loss, may precede any systemic organ involvement and in children, mucocutaneous manifestations develop most frequently during the varying disease course. This review summarizes recent information on epidemiology, clinical manifestations, diagnostic procedures, and treatment strategies of the different connective tissue diseases, concentrating on specific problems in childhood.

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