ANCA-positive necrotizing vasculitis and thrombotic vasculopathy induced by levamisole-adulterated cocaine: A distinctive clinicopathologic presentation

Jenkins, Jennifer; Babu, Kavita M.; Hsu-Hung, Ellen; Robinson‐Bostom, Leslie; Kroumpouzos, George

doi:10.1016/j.jaad.2010.09.778

Cited by 38 publications

(32 citation statements)

References 4 publications

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“…In users of cocaine contaminated with levamisole, a cutaneous vasculopathy syndrome has recently emerged and is characterized by a distinctive purpuric rash with a predilection for the ears, ANCA positivity, and leukopenia. [1][2][3] Levamisole, an antihelminthic with immunomodulatory properties, was previously used for the treatment of autoimmune disorders, pediatric nephritic syndrome, and cancer. It was withdrawn from the market in 2000 due to its adverse side effect profile, specifically agranulocytosis.…”

Section: Discussionmentioning

confidence: 99%

“…1,10 One of the most unique features of this syndrome is that the rash has a predilection for the ears, which has been documented in many case reports and case series. 2,3,8,11,12 It is interesting to speculate on the reason why the ears are preferentially involved. It may be due to the fact that the lower temperature of the ear may facilitate the deposition of immune complexes.…”

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confidence: 99%

“…1 Biopsy features may show necrosis of small and medium-sized vessels of the dermis and subcutis or an occlusive vasculopathy with intravascular fibrin thrombi and epidermal necrosis without vasculitis. 2,12 Detection of levamisole in both serum and urine must be performed using GC-MS or liquid chromatography-tandem mass spectrometry (LC-MS), as it is not detectable by routine toxicologic testing. The half-life of levamisole is approximately 5.6 hours, and limited amounts are excreted in the urine, which can make detection difficult.…”

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confidence: 99%

“…1,14 Cocaine-levamisole cutaneous vasculopathy syndrome has unique immunologic abnormalities, the most important of which is ANCA positivity. 1,2,7,8,[14][15][16] This can present a significant diagnostic challenge and renders it difficult to distinguish from true idiopathic systemic vasculitis. In cocaine-levamisole vasculopathy patients, high titer ANCAs against multiple antigens, higher incidence of antiphospholipid antibodies, and low titer ANA, are characteristic.…”

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confidence: 99%

“…14 Specifically, these patients may have high titer perinuclear-ANCA (p-ANCA) positivity alone, 2,8 or characteristically, both p-ANCA and cytoplasmic-ANCA (c-ANCA), with anti-MPO and anti-PR3 positivity. 1,7,14,16 In systemic vasculitis, reactivity to anti-PR3 alone is sensitive and specific to Wegener granulomatosis, whereas reactivity to anti-MPO alone is typically associated with microscopic polyangiitis and Churg-Strauss syndrome.…”

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confidence: 99%

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Cutaneous Vasculopathy Associated with Levamisole-Adulterated Cocaine

Tran

Tan

Marnejon

2012

Clinical Medicine & Research

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We report a case of cutaneous vasculopathy associated with the use of levamisole-adulterated cocaine. This recently described clinical entity is characterized by a purpuric rash with a predilection for the ears, leukopenia, and anti-neutrophilic cytoplasmic antibody (ANCA) positivity. It is estimated that more than 70% of the current United States cocaine supply is contaminated with levamisole. Levamisole is a widely available, inexpensive, white powder used as a "cutting agent" in cocaine to expand volume and increase profits. It may also increase the euphoric and stimulatory effects of cocaine by increasing brain dopamine levels and producing amphetamine-like metabolites. Our patient exhibited a characteristic rash with involvement of the ears, leukopenia, and cocaine metabolites were detected in serum and urine. The presence of levamisole was confirmed in the urine utilizing gas chromatography-mass spectrometry. ANCA positivity was also present. Punch biopsy of the skin demonstrated vascular thrombosis and necrosis without true vasculitis. We review the literature for reported cases of cocainelevamisole cutaneous vasculopathy syndrome, highlight the salient immunologic abnormalities, and contrast the features of this entity with idiopathic systemic vasculitis.

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Cutaneous Vasculopathy Associated with Levamisole-Adulterated Cocaine

Tran

Tan

Marnejon

2012

Clinical Medicine & Research

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Sepsis and persisting neutropenia in a drug addict

2017

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A 38-year-old woman was brought in to the emergency room (ER) because of altered level of consciousness. She had a known history of seizure disorder but was not on any prescription medications. She also had a history of intravenous (IV) drug abuse, including cocaine, methamphetamine, and heroin. She recently described herself as being "pill-sick" which according to her partner meant that she felt unwell after a recent administration of an illicit drug.In the ER, she was hypoxemic and was intubated. She was febrile, her blood pressure was 90 systolic, heart rate 120/min, and oxygen saturation undetectable by the digital probe. Admission hemoglobin was 11.6 g/dL (mean corpuscular volume [MCV] 86), white blood count 0.8 3 10 9 /L (absolute neutrophil count [ANC], 0.2), and platelet count 97 3 10 9 /L. Rare nucleated red blood cells were seen, but no myeloblasts or other primitive cells. Coagulation studies showed INR 3.1, activated partial thromboplastin time (aPTT) 81 s, and fibrinogen 1.9 g/L. The serum creatinine was 320 mmol/L (reference range, 50-98); serum lactate measured 13.0 mmol/L (reference range, 0.5-2.2). A chest X-ray showed multiple pulmonary infiltrates and right-sided pleural effusion. A CT head scan was negative for any acute abnormality. CT chest was suspicious for a right-sided empyema, but no vegetations were seen on the heart valves. The most striking finding on her admission blood work was leukopenia with near-absence of neutrophils in the peripheral blood. The clinical picture of hypotension, tachycardia, lactic acidosis, renal failure, pulmonary infiltrates, and pleural effusion in the setting of IV drug abuse, together with thrombocytopenia, coagulopathy, and normoblastemia, suggests septicemia in the setting of pneumonia or right-sided infective endocarditis, most likely complicated by disseminated intravascular coagulation (DIC)-although fibrin D-dimer levels would be helpful in supporting the last diagnosis. Severe sepsis can result in a transient leukopenia/neutropenia, 1 although complete absence of circulating neutrophils is unusual and points to a possible de novo neutropenic disorder such as drug-induced agranulocytosis 2,3 (although the patient was not receiving any prescription medications). Current guidelines do not recommend starting this patient on G-CSF on admission due to lack of proven mortality benefit. 4,5 Circulating nucleated red blood cells (normoblastemia) portend a poor prognosis in a critically ill patient. 6 She had a central venous catheter inserted into her right internal jugular vein and was transferred to the intensive care unit (ICU) on vasopressin and norepinephrine. Blood and urine cultures were sent and the patient was started on piperacillin-tazobactam and vancomycin. Repeat blood work showed progressive thrombocytopenia, with the platelet count measuring 9 3 10 9 /L 20 h later, and with the leukocytes and ANC remaining profoundly reduced at 0.1 and 0, respectively. Repeat coagulation studies showed INR 3.9, aPTT >150, fibrinogen 2.0, and D-dimer > 20,00...

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Nomenclature of Cutaneous Vasculitis

Sunderkötter

Zelger

Chen³

et al. 2018

Arthritis & Rheumatology

227

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Standardized names and definitions are a prerequisite for developing validated classification and diagnostic criteria for cutaneous vasculitis. Accurate identification of specifically defined variants of systemic and skin-limited vasculitides requires knowledgeable integration of data from clinical, laboratory, and pathologic studies. This proposed nomenclature of vasculitides affecting the skin, the D-CHCC, provides a standard framework both for clinicians and for investigators.

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ANCA-positive necrotizing vasculitis and thrombotic vasculopathy induced by levamisole-adulterated cocaine: A distinctive clinicopathologic presentation

Cited by 38 publications

References 4 publications

Cutaneous Vasculopathy Associated with Levamisole-Adulterated Cocaine

Cutaneous Vasculopathy Associated with Levamisole-Adulterated Cocaine

Sepsis and persisting neutropenia in a drug addict

Nomenclature of Cutaneous Vasculitis

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