Cocaine-Levamisole-Induced Vasculitis/Vasculopathy Syndrome

Abstract

New insights into immunological mechanisms involved in the pathogenesis of the disease. There are still many unknown aspects in the pathogenesis of this disease, such as the immune system interaction with p-ANCAs and the release of inflammatory NETs (neutrophil extracellular traps), which are the origin of auto-antigens and tissue damage, manifesting as vasculitic purpura on the skin. The clinical presentation constitutes a challenge for the clinician to be able to distinguish it from small-vessel vasculitides. This paper intends to improve the understanding of this condition, exhibiting the broad clinical spectrum of local and systemic manifestations of cocaine-levamisole-induced vasculitis, to facilitate a timely diagnosis, in order to take corrective measures and avoid sequelae, along with tissue damage and the consequent deformities and permanent scars.

Multiple cutaneous and mucosal lesions in a patient with cocaine-levamisole–induced vasculopathy syndrome

“…3,4 CLIVS is identified by its cutaneous manifestations, neutropenia, and ANCA positivity. 5,6 A conspicuous feature of CLIVS in early reports has been the paucity of organ involvement apart from dermatologic lesions and granulocytosis. 7 Typical cutaneous manifestations include palpable purpura or petechiae with predilection for the external pinna.…”

“…6,7 Cocaine and levamisole also lead to the formation of neutrophil extracellular traps, which induce an adaptive immune response leading to production of ANCA. 5,7 This leads to immune complex deposition with resultant vascular necrosis, inflammation, hemorrhage, and fibrin deposition. 1,6,11 Diagnosis requires the appropriate clinical history, classic cutaneous exam findings, leukopenia, ANCA positivity, and exclusion of infectious, idiopathic, or vasculitic entities.…”

Antineutrophil cytoplasmic antibodies negative levamisole‐induced leukocytoclastic vasculitis: a presumed case and literature review

“…The role of laboratory evaluation in suspected cases remains unclear. High p‐ANCA titers are almost always found (86–100%), and approximately 50% of cases also have cytoplasmic antineutrophil antibodies . Serologic findings of LIV are difficult to distinguish from that of other vasculitides, including granulomatosis with polyangiitis, which is characterized by antinuclear antibodies, lupus anticoagulant, and cytoplasmic antineutrophil cytoplasmic antibodies (c‐ANCA) staining pattern.…”

“…There are no established criteria for levamisole‐induced vasculitis/vasculopathy, and the diagnosis is based on clinical suspicion and that of exclusion . Hence, a judicious history and physical exam remain a clinician's strongest diagnostic tool.…”

“…21,22 A study by Lood and Hughes 22 14,20,21,[28][29][30] There are no established criteria for levamisole-induced vasculitis/vasculopathy, and the diagnosis is based on clinical suspicion and that of exclusion. 14,27,31 Hence, a judicious history and physical exam remain a clinician's strongest diagnostic tool. Since patients do not always readily admit to cocaine use, toxicology may be helpful to determine cocaine exposure in select cases.…”