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Mixed connective tissue disease

Case contributed by Roberto Schubert
Diagnosis certain

Presentation

Clinical diagnosis of Sharp syndrome. Progressive deformities and functional impairment of both hands.

Patient Data

Age: 70 years
Gender: Female
x-ray

Advanced osteoarthritis with joint space narrowing, erosions and deformities of the PIP and DIP joints. Subcutaneous calcifications at dig. III left and dig III and IV right.

Case Discussion

The occurrence of subcutaneous calcifications (calcinosis cutis) in an autoimmune disorder (scleroderma) has been first described by the French dermatologists Georges Thiebierge and Raymond Weissenbach in 1911. Hence, it is sometimes referred to as Thiebierge-Weissenbach syndrome.

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