Presented by: Jaryd Freedman, MD and Carrie Ann Cusack, MD
Drexel University College of Medicine
May 16, 2016
Approximately 80% of patients with systemic lupus erythematosus (SLE) have cutaneous manifestations. In addition to common cutaneous manifestations of SLE such as malar rash, discoid lesions and mucosal ulcerations, chronic cutaneous ulcerations (CCU) are another skin lesion that has frequently been reported in patients with SLE. Wysenbeek et al reported that the prevalence of CCU in the SLE populations is 8%. In addition to the common causes of chronic cutaneous ulceration (ie: venous stasis, atherosclerosis, neuropathy), the SLE population is at increased risk for rare causes of CCU like atypical infections, vasculitis, antiphospholipid antibody syndrome, and other autoimmune conditions which can cause ulceration.
Patients with SLE have an increased risk of infection. Approximately 15% of hospitalizations and 25% of deaths in patients with SLE are due to infection. This increased risk of infection is thought to be due to multiple factors including an intrinsic immune dysregulation, higher incidence of renal disease, and the use of immunosuppressive agents in this patient population. In addition to organisms that commonly cause cutaneous infections one must consider unusual and atypical causes of cutaneous infection in patients with SLE. If cutaneous infection is suspected, aerobic and anaerobic bacterial, fungal, and mycobacterial cultures should also be considered. As nontuberculous mycobacteria can be fastidious, 16S RNA PCR can be a useful adjunctive test if suspicion for atypical mycobacterial infection is high but cultures are negative.
In addition to infectious causes, CCU in SLE patients can be a result of associated autoimmune phenomenon. Patients with SLE are at increased risk of thrombosis which can rarely present as CCU. The risk of thrombosis is increased by having positive titers for antiphospholipid antibodies which are present in approximately 40% of the SLE population. Even SLE patients without antiphospholipid antibody syndrome are at increased risk of vaso-occlusive disease likely due to their inflammatory disease state and certain treatment regimens. In addition to vaso-occlusive disease, SLE patients are at increased risk of vasculitis with reported rates of 11-36%. These patients can present with CCU, but will often have other clinical findings. Pyoderma gangrenosum has also been associated with SLE, though much more rarely than other autoimmune diseases like rheumatoid arthritis or inflammatory bowel disease. Finally, patients with SLE must be thoroughly worked up for more common causes of CCU including venous stasis, arterial disease, and neuropathic disease.
The delineation of infectious versus noninfectious causes of atypical CCU in patients with SLE is of vital importance. While many noninfectious etiologies of CCU in SLE patients may benefit from increased immunosuppression, infectious etiologies may be exacerbated. Despite extensive workup, the etiology of CCU in SLE patients may elude precise diagnosis.
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