RA is an inflammatory and erosive disease that typically presents as a polyarthritis. It may result in destruction of cartilage and bone erosions, leading to joint destruction. When diagnosed early and treated intensively with appropriate medication, RA is usually well controlled. When poorly controlled, as in this patient, extra-articular manifestations are more likely to occur and may be found in as many as 40% of patients. Rheumatoid nodules and secondary Sjögren syndrome are the most common systemic features. In poorly treated or untreated patients, systemic features may multiply and can include Felty syndrome, pneumonitis, coronary artery disease, scleritis, and small-to-medium vessel vasculitis (ie, rheumatoid vasculitis). Rheumatoid vasculitis is associated with a poor prognosis and high mortality.
The biopsy in this patient revealed evidence of diabetic dermopathy and venous insufficiency, as well as leukocytoclastic vasculitis with associated thrombosis and necrosis. This description is classic for the microscopic changes of small vessel vasculitis in RA, which was the diagnosis in this patient. Some studies suggest that morbidity and mortality associated with rheumatoid vasculitis can be as high as 30%-50% over a 5-year period. Advanced rheumatoid vasculitis, as in the patient discussed here, is a serious manifestation of RA that, once present, can be difficult to control. However, with advances in treatment of RA, including the use of biologic DMARDs, current mortality may be lower.
Rheumatoid vasculitis typically occurs years to decades after the initial presenting symptoms of RA. The vasculitis is associated with autoantibodies and immune complex binding against endothelial cells of small- to medium-sized vessels. This type III hypersensitivity reaction is due to the endothelial cells having receptors to which the autoantibodies bind; once activated, this causes a series of events that result in an inflammatory response, including plasma extravasation, leukocyte recruitment, and release of degradative enzymes and activation of the coagulation cascade.
The cytotoxic substances released by leukocytes damage the endothelial cells of the lumen of vessels as they migrate across the activated vascular walls. This process results in the visible vasculitic lesions and rash as seen in this patient.
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Cite this: Victoria M.F. Mank, Jefferson R. Roberts. A 59-Year-Old Woman With Painful Rash on Her Elbows - Medscape - Aug 03, 2023.