The appearance of severe ulceration of the skin in patients with rheumatoid arthritis is often associated with a tendency to progression of the underlying disease, involvement of internal organs and increased mortality. In the pathogenesis of such ulceration there are multiple causes for their development, persistence and tendency to poor healing. They include localized or generalized immune complex vasculitis, treatment with anti-inflammatory drugs and their side effects following the treatment, arterial and venous insufficiency, and mechanical factors. The management of severe ulceration requires stabilization of the underlying autoimmune disease, e.g. with high doses of glucocorticosteroids or other immunosuppressive drugs or plasmapheresis. Adjuvant treatment of pain with analgesics, improvement of blood perfusion and anti-inflammatory drugs should accompany the topical therapy of ulcers. After suppression of the local inflammatory reaction surgical intervention becomes necessary in most of the patients, and vascularized muscle flaps should be used in preference to meshgrafts or split skin grafts for extensive ulceration in rheumatoid arthritis. A hopeful perspective in the treatment of severe rheumatoid arthritis might be opened up with immunotherapy using monoclonal antibodies.