Fatal thrombotic thrombocytopenic purpura in discoid lupus

Manuscript type : Brief Report | Article Date : 2015/07/24

  • Autors

    1. Dai Shengchuan
    2. Lin Yan
    3. Sabzwari Syed Rafay Ali
    4. Satiya Jinendra
    5. Abusaada Khalid
  • Abstract

    The association between TTP and systemic lupus erythematosus (SLE) has been infrequently reported, however an association with discoid lupus was not reported in the literature. Usually, patients with TTP have increased SLE activity and frequent renal involvement. Here we present a case of a 65 year old man who presented to the emergency department with malaise, fever and purpura of several days duration. His past medical history was significant for discoid lupus diagnosed by biopsy 34 years ago as well as hypertension. On laboratory investigation, he had low complement levels and ADAMTS13 level of < 5 %. A peripheral blood smear showed the presence of schistocytes. On the basis of clinical and biochemical findings, he was diagnosed with TTP.
  • Description

    BRIEF REPORT

    Thrombotic thrombocytopenic purpura (TTP) is an acute and uncommon disorder resulting from oc-clusion of small arterioles and capillaries by micro-thrombi. It is defined as a pentad consisting of mi-croangiopathic hemolytic anemia, thrombocytopenia, nonfocal neurologic abnormalities, renal function impairment and fever. Discoid lupus is a variant of cutaneous lupus characterized by well-defined in-flammatory plaques that may occur independently or as manifestation of systemic lupus erythematosus. There are few reports about the association between TTP and SLE but the association of TTP with discoid lupus was not reported in the literature.1 A 65-year-old man who presented to the Emergency Department (ED) due to lightheadedness , purpuric rash, subjective fever, and dark urine for 4 days, he also had one episode of confusion with difficulty ex-pressing his thoughts. The patient was diagnosed with discoid lupus by biopsy in 1980 and was not on any immunosuppressants as he showed no clinical signs of lupus in the last 34 years. Physical examination re-vealed pale conjunctivae, petechiae on his extremities and lower trunk area (Fig. 1A), and discoid discolor-ation on scalp (Fig. 1B). The blood tests on admission showed: hemoglobin 11.6 g/dl, platelets 11,000 mm3, LDH 1641 U/L, haptoglobin uld involve a detailed examination of the heart tissue.7 Knowledge of this syndrome and the high risk of sud-den death justifies the need for quick diagnosis and treatment.6 Patients with SLE who develop TTP, do significantly worse than those with idiopathic TTP, with mortality of 34%-62.5%. The case we presented is noteworthy because it shows for the first time that low activity discoid lupus not only can cause TTP but also can be fatal and that such cases need more aggressive treatment with im-munosuppressants such as rituximab and cyclophos-phamide. Physicians should be alert of the possibility of TTP when treating a patient with discoid lupus. Even in cases of discoid lupus with low disease activity, TTP can be life-threatening. Hence patients with history of discoid lupus who present with TTP are recommended to undergo aggressive treatment with plasma exchange and immunosuppressants.
  • Reference

    1. 1.Garcia Boyero R, Mas Esteve E, Mas Esteve M, et al. Systemic lupus erythematosus and thrombotic thrombocytopenia purpura: a refractory case without lupus activity. Reumatol Clin 2013; 9(6): 373 -5
    2. 2.Lansigan F, Isufi I, Tagoe CE. Microangiopathic haemolytic anaemia resembling thrombotic thrombo-cytopenic purpura in systemic lupus erythematosus: the role of ADAMTS13. Rheumatology (Oxford) 2011; 50 (5):824-9.
    3. 3.Cesar A. Perez, Nabil Abdo, Anuj Shrestha, et al. “Systemic Lupus Erythematosus Presenting as Thrombotic Thrombocytopenia Purpura: How Close Is Close Enough?” Case Reports in Medicine, 2011, Article ID 267508
    4. 4.Hamasaki K, Mimura T, Kanda H, et al. Systemic lupus erythematosus and thrombotic thrombocytopenic purpura: a case report and literature review. Clin Rheumatol 2003; 22:355-8.
    5. 5.D A Fox, J D Faix, J Coblyn, et al. Thrombotic thrombocytopenic purpura and systemic lupus erythematosus. Ann Rheum Dis 1986 45: 319 -322
    6. 6.Wajima T, Johnson EH. Sudden cardiac death from thrombotic thrombocytopenic purpura. Clin Appl Thromb Hemost. 2000; 6(2):108-10.
    7. 7.Tasaki T, Yamada S, Nabeshima A, et al. An autopsy case of myocardial infarction due to idiopathic thrombotic thrombocytopenic purpura. Diagn Pathol. 2015 May 29; 10 (1):52.