A 30-year-old woman presented with sudden onset of large irregular shaped confluent hemorrhagic necroses, ecchymoses and bullae affecting both legs (Fig. 1A). Physical examination was otherwise unremarkable apart from a malar rash. Laboratory evaluation disclosed a high C-reactive protein level, thrombocytopenia, prolonged thrombin time and International Normalized Ratio, normal fibrinogen, elevated D-dimer levels and hypocomplementemia. Four years earlier, SLE had been diagnosed in the presence of malar rash, autoimmune hemolytic anaemia, hypocomplementemia and high-titer antinuclear and anti-dsDNA antibodies [1]. Antiphospholipid-antibodies and lupus anticoagulant have not been detected. The patient had been in remission under hydroxychloroquine treatment (cumulative dose 590 g) until her current admission.
Manifestation of Sanarelli Shwartzman phenomenon in a patient with SLE
(A) Right and left leg: ecchymoses and evolving hemorrhagic necrosis and bullae. (B) Microthrombus in small vessels (arrow).
Ruling out other causes for disseminated intravascular coagulation such as septicaemia, trauma, malignancies, immune thrombocytopenia, mixed cryoglobulinemia and catastrophic antiphospholipid syndrome, the patient was diagnosed with SLE and Sanarelli Shwartzman phenomenon, a rare complication of SLE, resulting from inflammatory complement-mediated disseminated intravascular coagulation with cutaneous hemorrhagic necrosis [2]. Accordingly, dermal biopsy revealed disseminated microthrombi in cutaneous vessels without signs of vasculitis (Fig. 1B).
Disease progression was stopped with high-dose glucocorticoids, cyclophosphamide and rituximab. Debridement of necroses and skin grafting followed. The patient has remained in remission under maintenance therapy with ciclosporin, mycophenolate-mofetil and hydroxychloroquine for 12 months now.
Funding: No specific funding was received from any funding bodies in the public, commercial or not-for-profit sectors to carry out the work described in this manuscript.
Disclosure statement: The authors have declared no conflicts of interest.
Data availability statement
Data are available upon reasonable request by any qualified researchers who engage in rigorous, independent scientific research, and will be provided following review and approval of a research proposal and Statistical Analysis Plan (SAP) and execution of a Data Sharing Agreement (DSA). All data relevant to the study are included in the article.
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