Scleredema

Scleredema, also known as scleredema of Buschke, is a rare connective tissue disorder characterized by symmetric, nonpitting thickening and induration of the skin. It most often affects the upper back, neck, and shoulders, but may involve the face, trunk, and limbs and can limit range of motion, particularly of the neck and shoulders.

The condition is typically categorized into three clinical types. Type 1 is postinfectious, occurring after an upper respiratory or streptococcal infection and often resolving over months to years. Type 2 is associated with hematologic disorders, such as monoclonal gammopathy or multiple myeloma, and can be more persistent. Type 3, known as diabetic scleredema, occurs in people with diabetes mellitus, especially with poor glycemic control, and tends to involve the neck and upper trunk.

Pathology shows thickened dermis with increased mucin deposition between collagen bundles, while the epidermis remains relatively normal. The clinical hallmark is nonpitting edema and firm, woody induration of the skin that can restrict movement.

Diagnosis is usually clinical, with biopsy performed to confirm if needed to distinguish from other scleroderma-like conditions. Laboratory tests help evaluate diabetes and screen for paraproteins in Type 2.

Management focuses on treating the underlying condition and maintaining mobility. Glycemic control in diabetes, treatment of any infection, and physical or occupational therapy are central. In refractory cases, adjunctive therapies such as ultraviolet light therapy, systemic medications (corticosteroids, immunosuppressants, retinoids), plasmapheresis, or intravenous immunoglobulin have been reported, but evidence is limited. Prognosis varies by type, with Type 1 often improving or resolving over time.