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Fellow’s Forum Case Report: True Vasculitis or Vasculitis Mimic?

Reeti K. Joshi, MD, Richa Gupta, MD, Hyon Ju Park, MD, Christine Pham, MD, and Leslie Kahl, MD  |  Issue: October 2012  |  October 1, 2012

There are no set recommendations for the treatment of levamisole-associated vasculopathy. In many reported cases, the skin disease was self-limited and resolved upon cessation of cocaine use.2 Several of our patients received pulses or short courses of oral prednisone. Although the skin lesions are thrombotic and not inflammatory, we found that our patients responded rapidly to steroid treatment. However, there appears to be no role for chronic immunosuppressive therapy.

We suspect the frequency of levamisole-induced vasculopathy will continue to increase as clinicians become aware of the clinical presentation. The differential diagnosis of levamisole-induced vasculopathy should be entertained when faced with necrotic lesions suggestive of vasculitis. A careful history, urine drug screening, and serological testing should point one toward the correct diagnosis. The importance of recognizing levamisole-induced vasculopathy lies in the fact that cessation of use of the levamisole-contaminated drug is the key treatment, rather than immunosuppression and/or anticoagulation therapy.

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At the time of writing this article, Drs. Joshi, Gupta, and Park were fellows in the division of rheumatology at Washington University/Barnes-Jewish Hospital in St. Louis. Dr. Joshi is now in clinical practice in Beaumont, Texas. Dr. Gupta is in practice in Gulfport, Miss. Dr. Pham is an associate professor at Washington University/Barnes-Jewish Hospital. Dr. Kahl is professor in the division of rheumatology at Oregon Health Science Center in Portland. Dr. Park is pursuing a third year fellowship.

References

  1. Peikert T, Finkielman J, Hummel A, et al. Functional characterization of antineutrophil cytoplasmic antibodies in cocaine-induced midline destructive lesions. Semin Arth Rheum. 2008;58:1546-1551.
  2. Specks U. The growing complexity of the pathology associated with cocaine use [comment]. J Clin Rheumatol. 2011;17:167-168.
  3. Brewer J, Meves A, Bostwick J, et al. Cocaine abuse: Dermatologic manifestations and therapeutic approaches. J Am Acad Dermatol. 2008;59:483-487.
  4. Walsh N, Green P, Burlingame RW, Pasternak S, Hanly J. Cocaine-related retiform purpura: Evidence to incriminate the adulterant, levamisole. J Cutan Pathol. 2010;37:1212-1219.
  5. Substance Abuse and Mental Health Services Administration. Nationwide public health alert issued concerning life-threatening risk posed by cocaine laced with veterinary anti-parasite drug. Published September 21, 2009. Available at www.samhsa.gov/newsroom/advisories/090921vet5101.aspx. Accessed July 8, 2012.
  6. Centers for Disease Control and Prevention. Agranulocytosis associated with cocaine use—four states, March 2008-November 2009. MMWR Morb Mortal Wkly Rep. 2009;58:1381-1385.
  7. Chang A, Osterloh J, Thomas J. Levamisole: A dangerous new cocaine adulterant. 2010;88:408-411.
  8. Khan T, Cuchacovich R, Espinoza L et al. Vasculopathy, hematological and immune abnormalities associated with levamisole-contaminated cocaine use. Semin Arthritis Rheum. 2011;41:445- 454.
  9. Ullrich K, Koval R, Koval E, et al. Five consecutive cases of a cutaneous vasculopathy in users of levamisole-adulterated cocaine. J Clin Rheumatol. 2011;17:167-168.
  10. Zhang W, Du X, Zhao G, et al. Levamisole is a potential facilitator for the activation of Th1 responses of the subunit HBV vaccination. Vaccine. 2009;27:4938-4946.
  11. Spector S, Munjal I, Schmidt DE. Effects of the immunostimulant, levamisole, on opiate withdrawal and levels of endogenous opiate alkaloids and monoamine neurotransmitters in rat brain. Neuropsychopharmacology. 1998;19:417-427.
  12. Rongioletti F, Ghio L, Ginevri F, Bleidl D, Rinaldi S, Edefonti A, et al. Purpura of the ears: A distinctive vasculopathy with circulating autoantibodies complicating long-term treatment with levamisole in children. Br J Dermatol. 1999;140:948-951.
  13. Walsh NM, Noreeen MG. Cocaine-related retiform purpura: Evidence to incriminate the adulterant, levamisole. J Cutan Pathol. 2010;37:1212-1219.
  14. Jacob RS, Silva CY, Powers JG, et al. Levamisole-induced vasculopathy: A report of two cases and a novel histopathologic finding. Am J Dermatopathol. 2012;34:208-213.
  15. Davin J, Merkus M. Levamisole in steroid sensitive nephritic syndrome of childhood: The lost paradise? Pediatr Nephrol. 2005. 20:10-14. Neuropsychopharmacology. 1998;19:417-427.
  16. Weisner O, Russell K, Lee A, et al. Antineutrophil cytoplasmic antibodies (ANCA) reacting with human neutrophil elastase as a diagnostic marker for cocaine induced destructive lesions but not for autoimmune vasculitis. Arthritis Rheum. 2004;50:2954-2965.
  17. Kouassi E, Caille G, Lery L, et al. Novel assay and pharmacokinetics of levamisole and p-hydroxylevamisole in human plasma and urine. Biopharm Drug Dispos. 1986;7:71-89.

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