Scleredema

Excerpt

Scleredema of Buschke is a rare pathological condition of connective tissue. Its pathogenesis is poorly understood. First described by Curizo in 1752, the disease was well defined afterward by Buschke in 1902. It belongs to the spectrum of scleroderma-like disorders. Clinically, it is responsible for a fibro-mucinous progressive induration of the skin, involving the neck, shoulders and proximal upper members and eventually the face. It occurs most commonly following an infectious episode. It has also been linked to diabetes mellitus and more rarely to hematological disorders. Histological features include dermal fibrosis with thickened collagen bundles and variable amounts of mucin deposits.