Cocaine‐related retiform purpura: evidence to incriminate the adulterant, levamisole

Abstract

The term ‘cocaine‐induced pseudovasculitis' was coined to encompass a constellation of clinical and laboratory findings which mimics a systemic vasculitis but lacks confirmatory evidence of vasculitis on biopsy. Antineutrophil cytoplasmic antibodies reacting with human neutrophil elastase (HNE) have been reported to distinguish the cocaine‐related syndrome from a true autoimmune vasculitis. Published cases of retiform purpura related to cocaine use are rare and an etiologic role for levamisole, a common adulterant of cocaine, has been postulated. We describe two female patients aged 39 and 49 years with cocaine‐related retiform purpura, mainly affecting the legs. The initial clinical and serological profile in case 1 led to a suspicion of anti‐phospholipid syndrome and in case 2 to Wegener's granulomatosis with an unexplained associated neutropenia. Skin biopsies revealed a mixed pattern of leukocytoclastic vasculitis and microvascular thrombosis in case 1 and pure microvascular thrombosis in case 2. Identification of anti‐HNE antibodies in both patients linked their disease to cocaine. The mixed vasculopathic pattern in case 1 and the associated neutropenia in case 2, both known adverse effects of levamisole, point to this as the true etiologic agent. Urine toxicology shortly after a binge of cocaine use in each case was positive for levamisole. Walsh NMG, Green PJ, Burlingame RW, Pasternak S, Hanly JG. Cocaine‐related retiform purpura: evidence to incriminate the adulterant, levamisole.

Contaminated Cocaine and Antineutrophil Cytoplasmic Antibody-Associated Disease

“…Over the same period, there were a series of reports in the literature linking levamisole with agranulocytosis and vasculitic skin lesions in cocaine users (4,11,12,18 -23). Many clinical and laboratory features of these cases are in keeping with previously described levamisole-related autoimmune disease (12,18,23). However, ANCA has not been commonly associated with autoimmune phenomena related to cocaine or levamisole ingestion (23).…”

Breaking-Out Bad: A Case of Levamisole-Induced Vasculitis

“…3,7 Other autoantibodies such as antiphospholipid antibody; antihuman neutrophil antibody; and anti-nuclear antibodies (ANAs), including anti-double-stranded DNA and lupus anticoagulant, have also been shown to be associated. 9,10 The pathogenesis of LIV is not fully known, although some hypothesize that levamisole may be directly cytotoxic to neutrophils or endothelial cells. 11 Other theories suggest it may be a nonspecific immune adjuvant in certain individuals predisposed to autoimmunity, or may induce loss of tolerance to specific autoantigens in a manner that initiates or perpetuates autoimmunity.…”

Cutaneous Vasculopathy Associated with Levamisole-Adulterated Cocaine

“…Recurrent skin lesions do occur when patients are re-challenged with levamisoleadulterated cocaine. 17 Recurrent lesions on re-challenge was noted in our patient; purpuric lesions on the earlobes and knees recurred after using cocaine again (figure 1).…”

“…Low titer ANA and antiphospholipid-antibodies have been reported in several cases. 7,14,16,17 Recently, anti-human neutrophil elastase (HNE), a subset of ANCA, has been reported to distinguish the cocaine-levamisole vasculopathy syndrome from true autoimmune vasculitis. 18 ANCA-associated vasculitis has been linked to various drugs, such as hydralazine, propylthiouracil, and minocycline.…”