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CLINICAL IMAGES
3639
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DOI 10.1002/art.30602
Clinical images: Cutaneous necrotizing vasculitis in a patient with
lepromatous leprosy
The patient, a 32-year-old woman from Paraguay who had a history of polyarthritis involving small joints, was seen for evaluation
of extensive skin ulcers on the arms and legs, buttocks, and the lower back, and for low-grade fevers. Physical examination showed
livedo reticularis and punched-out and serpiginous ulcers on the upper and lower extremities (A). Complete blood cell count and
levels of creatinine were normal, and findings on tests for antinuclear antibodies, antineutrophil cytoplasmic antibodies, lupus
anticoagulant, IgG anticardiolipin antibodies, and syphilis were negative. IgM anticardiolipin antibodies were positive (74 IgM
phospholipid units/ml), the erythrocyte sedimentation rate was 56 mm/hour, and rheumatoid factor was positive (45 units/ml). A skin
biopsy was performed, and revealed a necrotizing vasculitis of the small vessels with multiple necrotic areas (B). Treatment with
prednisone (1 mg/kg/day) was initiated. The patient returned 1 month later with more extensive skin lesions, which were treated with
intravenous cyclophosphamide. The atypical evolution made the diagnosis of primary vasculitis questionable, and a new biopsy
showed necrotizing vasculitis and aggregations of acid-fast bacilli in the endothelium. These were identified by Fite-Faraco
technique as Hansen bacilli; typical Virchow’s cells were also identified (C). These findings led to a diagnosis of necrotizing
vasculitis, secondary to the Lucio’s phenomenon associated with lepromatous leprosy. After antileprosy treatment and a tapered
dosage of glucocorticoids, no new lesions appeared and the residual lesions cicatrized. Lucio’s phenomenon is an immune response
to Mycobacterium leprae, leading to sudden changes in the clinical manifestations of untreated lepromatous leprosy in some patients;
the diagnosis may thus be quite challenging. Due to immigration from countries with a high prevalence of leprosy, it is necessary
to consider this diagnosis in specific clinical situations, even in regions where leprosy is uncommon.
Teresa Camps-Garcı́a, MD
Iván Pérez-de Pedro, MD
Ignacio Márquez Gómez, MD
Dariusz Narankiewicz, MD
Mar Ayala-Gutierrez, MD
Andres Sanz-Trelles, MD
Hospital Carlos Haya
Málaga, Spain
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