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An unusual case of distal necrosis. Looking for the truth

Published:August 29, 2017DOI:https://doi.org/10.1016/j.ejim.2017.08.014

      1. Indication

      A 47 year-old man presented with a history of pain and necrosis on the distal phalange of the third finger on the right hand. He also presented subungual splinter haemorrhages and eritroviolaceous prenecrotic lesions in finger pads of both hands but not in other areas of the body (Fig. 1A–C ). The rest of the physical examination was normal. He denied consuming illicit drugs or herbalism products. There was no history of Raynaud syndrome, ophthalmologic symptoms, oral or genital aphthosis, photosensitivity, dyspnoea, constitutional syndrome or fever. The tibial, radial and cubital pulses were conserved. Laboratory findings revealed neutropenia and elevated C Reactive Protein (CRP). High-titre of antinuclear antibodies (ANAs), anti-double-stranded DNA antibodies, anti-histone antibodies and antineutrophil antibodies with both cytoplasmic (c-ANCA) and perinuclear pattern (p-ANCA) with a positive serology against PR-3 and MPO were detected. The target of p-ANCA were atypical antigens including the human neutrophil elastase (HNE).
      Fig. 1
      Fig. 1A. Skin manifestation of levamisole-adulterated cocaine. Necrotic lesion and subungual haemorrhages in splinter in both hands.
      B. Purpuric plaque with advanced necrotic area on third finger of the right hand.
      C. Retiform purpura on distal phalanges of the left hand.

      2. What is the diagnosis?

      2.1 Diagnosis

      Answer: Thrombotic vasculitis and Lupus-like Syndrome induced by cocaine/levamisole.
      The differential diagnosis included infection, cardiac embolisms, autoimmune conditions and drugs, all of which were discarded.
      The urine analysis revealed cocaine, which he subsequently admitted consuming. Nowadays, there is a high prevalence of adulterated cocaine with levamisole, used to increase its euphoric effect. We could not demonstrate its presence because it must be determined before 72 h after initial exposure [
      • Imbernón-Moya A.
      • Chico R.
      • Aguilar-Martínez A.
      Manifestaciones cutáneas y mucosas asociadas al consumo de cocaína.
      ] However, the clinical and analytic findings were consistent with the toxic effects of levamisole. It causes a distinctive syndrome consisting in neutropenia, retiform purpura with necrosis and high-titre of ANCA with atypical patterns (HNE among others) which has high specificity [
      • Imbernón-Moya A.
      • Chico R.
      • Aguilar-Martínez A.
      Manifestaciones cutáneas y mucosas asociadas al consumo de cocaína.
      ,
      • Graf J.
      Rheumatic manifestations of cocaine use.
      ,
      • Muirhead T.T.
      • Eide M.J.
      Toxic effects of levamisole in a cocaine user.
      ]. There are also frequent positive tests for one or more antiphospholipid, antinuclear and anti-double-stranded DNA antibodies, as in our case.
      He was treated with intravenous prostaglandins and opioids for the pain. Necrosis was solved with the spontaneous amputation of the phalange and the rest of the skin lesions disappeared.
      After four months the patient developed serositis, neutropenia and wrist arthritis, being this compatible with a lupus-like syndrome after a new exposure of cocaine, that was solved with a short cycle of corticoids.
      Therefore, the evaluation of a patient with suspected cocaine abuse requires a high index of suspicion because it is usually not admitted. As it is shown in this case, cocaine adulterated with levamisole can induce a clinical syndrome of inflammation, vasculitis and antibody production that resemble a primary idiopathic vasculitis.

      Conflicts of interests

      None.

      References

        • Imbernón-Moya A.
        • Chico R.
        • Aguilar-Martínez A.
        Manifestaciones cutáneas y mucosas asociadas al consumo de cocaína.
        Med Clin. 2016; 146: 544-549
        • Graf J.
        Rheumatic manifestations of cocaine use.
        Curr Opin Rheumatol. 2013; 25: 50-55
        • Muirhead T.T.
        • Eide M.J.
        Toxic effects of levamisole in a cocaine user.
        N Engl J Med. 2011; 364e52