To characterize levamisole-induced vasculopathy.
We performed a systematic review searching MEDLINE for articles published from 1972 to 2016.
We retrieved 357 references and abstracts and selected 111 articles. Levamisole-induced vasculopathy was reported in 192 patients, with a female predominance (n = 122, 63.5%). Median [interquartile range] age was 44 [38-50]. Skin was the most frequently involved organ (n = 182, 94.8%). Cutaneous lesions were mostly on the face (n = 136, 70.8%), especially the ears. Purpura (n = 131, 68.2%) was the most reported cutaneous lesion. Organ involvement included acute renal failure (n = 24, 12.5%), and pulmonary involvement (n = 20, 10.4%). Anti-neutrophil cytoplasmic antibodies (ANCAs) were found in 167/178 patients (93.8%), with both anti-myeloperoxydase and anti-proteinase 3 specificity reported in 51/118 patients (43.2%). Anti-phospholipid (APL) antibodies were found in 93/137 patients (67.9%). Leukopenia was detected in 69/138 patients (50%). Skin biopsies identified vasculitis and thrombotic vasculopathy in 73/148 (49.3%) and 62/148 (41.9%) patients, respectively. The outcome was favourable in 116/134 patients (86.6%), but relapses were reported in 33 (28.4%), mainly on levamisole re-exposure.
Levamisole-induced vasculopathy is characterized by a female predominance, skin involvement, ANCA and/or APL antibody positivity, leukopenia, vasculitis or vascular thrombotic histological lesions, and despite possible systemic involvement, a favourable outcome with levamisole interruption.