FM is a 44 yo black female with a history of mixed connective tissue disorder who presented to the emergency room with worsening pain in her left wrist, facial swelling and worsening ulcers and rash on her face. The rash on her ears and face started 2 days prior. She had been off her prednisone for 5 months prior but recently was started back on it in clinic. She was compliant with her plaquenil. She has a history of MRSA infections as well as levamisole toxicity from cocaine but has been cocaine free for one year. She was nontoxic with stable vital signs but had a bruised rash, aphous ulcers and multiple wounds on her body as the images below show. Her ESR was 88 and CRP was 147 and a had her creatinine peak at 1.34 from a normal baseline. The patient was admitted and had rheumatology, infectious disease, dermatology and orthopedics consult for the wounds and concern for septic joint versus autoimmune flare.
The patient initially had an arthrocentesis which revealed a dry tap and despite IV antibiotics her pain and range of motion in the wrist decreased. An MRI was ordered which showed a fluid collection and possible synovitis of the wrist. She was taken to the operating room by orthopedics and had a debridement which showed extensive synovitis but no pus. Infectious diseases consulted and given sensitives recommended four weeks of Bactrim solution. Dermatology performed and punch biopsy on the calf and ear which showed thrombotic vasculopathy with epidermal hyperkeratosis and acanthosis with dermal fibrosis and lymphplasmacytic infiltrate consistent with a relapsing levamisole vasculopathy. Given this finding and concern that this could be worsening her renal function steroids were concurrently given with antibiotics with the plan of close outpatient follow up and weaning of steroids.
Discussion:
Levamisole is a substance that is present in cocaine as cutting agent. It is present in up to 69 percent of cocaine samples in some studies. It has been used for chemotherapy agents and is routinely used as an anti-helminthic agent in veterinary medicine. It is used with cocaine to add bulk but it can potentiate the euphoria associated with cocaine as its metabolite aminorex inhibits dopamine and norepinephrine reuptake and increases the release of serotonin and thus enhances synaptic catecholamine activity. Its side effects include agranulocytosis. It has been shown to cause purpuric skin lesions in users in the cheeks and pathognomonic rash on the ears, leukopenia, as well as ANCA positivity. Although rare it can develop within one year of cocaine use or relapse and show a similar presentation compared to the original one. The metabolite can be seen in the urine using gas chromatography mass spectrometry. In patients who present with the classic rash and ear findings as well as an exposure history one needs to have a high index of suspicion. Patients with debilitating arthropathy, biopsy proven vasculitis, and elevated CRP can be prescribed systemic steroids but evidence is not conclusive that it changes the natural course.
Conclusions:
Levamisole induced vasculopathy is an uncommon condition that causes a near pathognomonic rash pattern including the ears. By quick identification and testing one can recognize this rash and provide the counselling and supportive treatment that your patient needs. It can relapse without further exposure to cocaine.