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Diagnosing pyoderma gangrenosum? Consider cocaine/levamisole as a potential culprit!

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By Warren R. Heymann, MD
May 1, 2016

The authors presented 8 patients with PG following exposure to levamisole-contaminated cocaine. All patients had lower extremity involvement, and 6 also had PG on the upper extremities. Retiform purpura was only observed in 3 patients. Most had elevated titers for p-ANCA and antiphospholipid antibodies. Histologic examination was predominantly neutrophilic, consistent with PG. Lesions improved or remained stable with conservative management or short coursed of steroids. Recurrence was only noted on re-exposure to adulterated cocaine.

Upon seeing a patient with retiform purpura involving the ears, the diagnosis is levamisole-induced vasculitis until proven otherwise. The appearance is so characteristic, it has become a proverbial “doorway diagnosis”. This is an important case series highlighting the association of levamisole contamination of cocaine with pyoderma gangrenosum. Keith et al (Pyoderma gangrenosum: A possible cutaneous complication of levamisole-tainted cocaine abuse. Int J Dermatol 2015; 54: 1075-7) presented the case of a 51 year-old woman with PG of her forehead, torso, and fingers. Her serologies were positive for the following: ANA, p-ANCA, lupus anticoagulant, anti-cardiolipin, and cryoglobulins (trace). SPEP, HIV and hepatitis studies were negative. The biopsy demonstrated neutrophilic infiltration without vasculitis. Cultures were negative, as was the “standard” work-up for other disorders characteristically associated with PG (inflammatory bowel disease, hematologic malignancy, or other autoimmune disorders). The patient responded to prednisone, however, she relapsed after subsequent exposure to cocaine.

We recently have seen several patients with pyoderma gangrenosum, two being considered as “post-operative” PG. The thought of levamisole-induced lesions never crossed my mind. These are extraordinarily important reports. Although the presentation may be atypical (facial and upper extremity involvement); the fact is that many lesions are characteristic PG, without associated retiform purpura, meaning that we must inquire about cocaine/levamisole exposure in such patients. Once considered, it is clear that the real challenge may not be in the diagnosis, but rather, addressing the addiction to cocaine in order to prevent recurrence.

Jeong HS, et al. Pyoderma gangrenosum associated with levamisole-adulterated cocaine. Clinical, serologic, and histopathological findings in a cohort of patients. J Am Acad Dermatol 2016; 74: 892-8.

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