Article, Dermatology

Idiopathic purpura fulminans

Case Report

Idiopathic Purpura fulminansB Abstract

A 45-year-old man presented to the ED with a history of bruising rush on both his breast areas initiated 2 days ago and which increased rapidly. The vital signs and physical examination result of the patient were normal except for blue-black hemorrhagic and bruised lesions with a surround- ing erythematous border on both his breast areas. Prothrom- bin and activated prothrombin time and international normalized ratio (1.2 [upper level is 1.2]), protein C, protein S, and antithrombine III levels and other laboratory parameters were also normal. He was admitted to the hospital for treatment. We were presented with a case of idiopathic purpura fulminans which is rarely diagnosed in the ED.

A 45-year-old man presented to the ED with a history of bruising rush on both his breast areas initiated 2 days ago and which increased rapidly. He had no past medical history and medication. The vital signs of the patient were as follows: blood pressure was 137/74 mm Hg, pulse rate was 92 beats per minute, respiratory rate was 17 per minute, oral body temperature was 36.6?C, and oxygen saturation was 96% using a pulse oxymeter in room air. Physical examination was normal except for blue-black hemorrhagic and bruised lesions with a surrounding erythematous border on both his breast areas (Fig. 1). No signs of infection were found on physical examination. Prothrombin time and activated prothrombin time and international normalized ratio (1.2 [upper level is 1.2]), protein C, protein S, and antithrombine III levels and other laboratory parameters were also normal. He was admitted to the hospital for treatment. The only fresh frozen plasma was given for treatment. In 72 hours, symptoms and signs resolved almost completely and the patient showed no signs of disseminated intravascular coagulation and hemodynamic instability at any point in time. He was discharged from the hospital fully recovered 5 days later.

Purpura fulminans (PF) is one of the dermatological emergencies and requires rapid diagnosis and management. It is characterized by the Rapid progression of symmetrical ecchymotic skin lesions, especially of the extremities, that

? This case report was supported by the Akdeniz University Research Foundation.

may progress to gangrene, ultimately resulting in amputation caused by DIC and dermal vascular thrombosis. The purpura varied from petechiae to purpuric patches to Hemorrhagic bullae and occurred anywhere on the body. Patients are acutely ill with fever, have hemorrhage from multiple sites that is hypotensive, and manifest typical laboratory signs of DIC [1]. Three distinct categories of PF are identified: First, acute sepsis associated or secondary PF. Second, idiopathic PF, which can manifest within 10 days of an antecedent illness, most commonly infections with meningococcus, varicella, Staphylococcus aureus, Streptococcus, and Hae- mophilus influenzae in children. Most skin lesions are localized to the breasts or to the lower half of the body. Third, hereditary PF, which can be seen in association with protein C, protein S, or antithrombine III deficiency in affected neonates [2,3]. Differential diagnosis includes thrombotic

Fig. 1 Symmetrical blue-black hemorrhagic and bruised lesions with a surrounding erythematous border on both his breast areas.

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thrombocytopenic purpura, Henoch-Schonlein purpura, and postinfectious thrombocytopenic purpura, most of which are not serious. Management of these patients in the ED should include aggressive fluid resuscitation, vasopressors to alleviate shock, and appropriate antibiotics. Hydrocortisone is used for Adrenal insufficiency. Fresh frozen plasma and platelet transfusions may be necessary for coagulopathy [4].

Firat Bektas MD Secgin Soyuncu MD

Department of Emergency Medicine Akdeniz University Faculty of Medicine

07059 Antalya, Turkey E-mail address: [email protected]

doi:10.1016/j.ajem.2010.04.022

References

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  3. Francis Jr RB. Acquired purpura fulminans. Semin Thromb Hemost 1990;16(4):310-25.
  4. Smith OP, White B, Vaughan D, Rafferty M, Claffey L, Lyons B, et al. Use of protein-C concentrate, heparin, and haemodiafiltration in meningococcus-induced purpura fulminans. Lancet 1997;350(9091): 1590-3.