Case Presentation: A 47-year-old African American woman with poly-substance abuse presented with a facial rash, bilateral hand joint pain and painful ulcers to the lower extremities for one week. Physical examination revealed violaceous tender skin lesions on the bilateral helices and right naris. There was mild erythema to the malar area involving the naso-labial folds. Well-demarcated necrotic ulcers with overlying eschar were seen on both knees and the right ankle. Scars from healed ulcers were noted on both lower extremities. The remainder of physical examination was unremarkable.Laboratory tests revealed leukopenia (WBC 2.95 K/uL), neutropenia (1.37 K/uL), elevated inflammatory markers, and mild hypocomplementemia. Positive perinuclear anti-neutrophil cytoplasmic antibody to myeloperoxidase (p-ANCA), (210 AU/mL) and antinuclear antibody (ANA). Other rheumatologic markers including lupus anticoagulant (LA), anti-dsDNA, anti-Smith, anti-Jo-1, anti-SSA (Ro), anti-SSB (LA), anti-histones and cytoplasmic anti-neutrophil cytoplasmic antibody (c-ANCA) were all negative. Urine toxicology showed cocaine and opiates.
A diagnosis of Levamisole-Induced Vasculitis (LIV) was made based on the consistency of lesions with Levamisole-induced retiform purpura and positive serology. The patient declined biopsy. She was treated with prednisone and subsequently improved.
Discussion: Levamisole, originally marketed as an anthelmintic and chemotherapeutic adjuvant, is known for its immunomodulatory properties. It was withdrawn from the human market 18 years ago, after serious adverse effects including leukopenia, agranulocytosis, and skin vasculitis were recognized. These effects now characterize the syndrome known as Levamisole-Induced Vasculitis (LIV). The pathogenesis is poorly understood. It has been reported that 69% of cocaine in the United States is tainted with Levamisole which adds bulk while potentiating the stimulant effects of cocaine.
LIV should be considered in patients with cocaine use presenting with the constellation of clinical findings including retiform purpura with or without skin necrosis, associated leukopenia, neutropenia and positive serological markers (ANA, LA, anti-phospholipid antibodies, high titer p-ANCA and/or c-ANCA). Typically, no other organ is involved. Histopathology can be nonspecific and detection of Levamisole is not required for diagnosis.
In the majority of cases reported, spontaneous resolution was seen with cessation of cocaine use making this the most important aspect of management along with supportive care. Systemic corticosteroids have been used, however, there is no evidence that they alter the clinical course and their potential adverse effects may limit their use.
Conclusions: With almost three quarters of the population of cocaine users in the United States exposed to Levamisole – adulterated cocaine, it is imperative that clinicians be able to identify the clinical and laboratory features of LIV and distinguish it from other vasculitides