Purpura Fulminans in Pneumococcal Sepsis: Case Report and Review

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  • ORIGINAL ARTICLE Scand J Infect Dis 29: 479-483, 1997

    Purpura Fulminans in Pneumococcal Sepsis: Case Report and Review CHACE T. CARPENTER and ALLEN B. KAISER From the Depurtineni of Medicine, Vanderbilt Uttiuersity School of Medicine, ~ushuille, TN, USA

    Purpura fulminans is classically defined by ecchymotic skin lesions, fever, and hypotension. The majority of cases occur in association with bacterial sepsis, and disseminated intravascular coagulation (DIC) is usually present. Prompted by our experience with a patient with pneumococcal sepsis and purpura fulminans in whom hypotension was never observed, we evaluated the important parameters of sepsis in reports of this syndrome. 42 additional cases of pneumococcal bacteremia and purpura fulminans were identified. Hypotension was present in only 51%. Although DIC was present in 85% of patients, hypofibrinogenemia was documented in only 26%. By contrast, both hypotension and hypofibrinogenemia are present in the vast majority of patients described with purpura fulminans in association with meningococcal sepsis. These data confirm that hypotension is not a necessary feature of the syndrome of purpura fulminans associated with pneumococcal sepsis and suggest further that qualitative or quantitative differences exist in the DIC cascade of pneumococcal vs meningococcal sepsis.

    A . B. Kaiser, MD, Department of Medicine, 0-3100 MCN. Vandevbilt University School of Medicine, Nashville, TN 37232-2358, USA

    20 years have passed since the classic monograph on pur- pura fulminans by Spicer and Rau was published (1). In that review, 3 primary clinical features of the syndrome were noted: (i) large purpuric lesions of the skin; (ii) fever; and (iii) hypotension. Hematologic and/or pathologic evi- dence of disseminated intravascular coagulation (DIC) was characteristically present as well. We present here a case report and review of the literature of purpura fulminans occurring in pneumococcal sepsis. The purpose of this review is to summarize what is known about the patho- genesis of pneumococcal purpura fulminans and to deter- mine the necessity of hypotension as a feature of the syndrome.

    CASE REPORT A 46-year-old previously healthy white male presented to his local hospital approximately 18 h after the sudden onset of fever, chills, and malaise. 17 years earlier he had undergone a splenectomy following a gunshot wound.

    The patient was alert but appeared acutely ill with complaints of generalized malaise and severe pain in his legs and feet. His blood pressure was 110/60 mmHg; pulse, 104/minute and regular; respiratory rate, I8jminute; and oral temperature, 3823C. The extremities were cool to the touch, and mottled cyanosis of his nose, lips, ears, and hands was present. He had scattered, non-blanching petechial lesions over his trunk. There were confluent purpuric lesions on his legs and feet. Arterial pulses were intact. The remainder of the physical exam was unremarkable, including clear lungs and a supple neck. Blood cultures were obtained and he was treated with ceftriaxone and gentamicin for presumed sepsis. Methylene blue was given intravenously for concern of possible methemoglobinemia. He was transferred to our facility 24 h later because of increasing cyanosis, hypoxemia, and rapidly rising serum creatinine.

    Physical examination at our facility was unchanged. Laboratory examination revealed: white blood cell count, 26.9 x 109/l; hematocrit, 0.46; platelet count, 68 x 109/l; serum creatinine, 433 pmol/l; fibrin split products, 154 (normal range = 0- 1:4);

    fibrinogen, 510 mg/dl (normal range = 190-400); prothrombin time, 15 s (normal range 10-13); and activated partial thromboplastin time, 36 s (normal range 25-40). Serum TNF-a level was elevated at 56 pg/ml.

    The pulmonary capillary wedge pressure was 10 mmHg. Urine output improved with hydration. The patient was treated with ceftriaxone and penicillin. On the second hospital day, blood cultures from his local hospital were reported as positive for S. pneumoniae, and ceftriaxone was discontinued. Skin biopsy of one of the petechial lesions on his trunk revealed dermal thrombosis and hemorrhage, as typically seen in purpura fulminans. His serum creatinine rose to a maximum of 645 prnol/i, but over the course of his hospitalization. his renal function slowly returned to normal. The purpuric lesions and cyanosis resolved on his ears, nose, lips, and upper extremities. He had necrosis of the tissue on several of his toes and subsequently had autoamputation of the distal portion of eight toes. He was discharged after a 21-day hospital course with plastic surgery follow-up. Pneumococcal vaccine was administered prior to discharge.

    METHODS The literature of purpura fulminans associated with pneumococcal sepsis was reviewed. Case reports were included if the patients had documented pneumococcal bacteremia, temperature greater than 38C (100.4 F), and a clinical diagnosis of purpura fulminans. Purpura fulminans was considered present if so stated by the authors, or if gangrene or purpura were used to describe the skin and soft tissue abnormalities. While hypotension was not considered a necessary feature of the syndrome, the presence or absence of hypotension at the time of onset of the skin lesions was noted. Hypotension was considered present if the systolic blood pressure was < 90 mmHg, the patient was described as being in shock, or vasoconstrictor agents were employed to support the blood pressure. Coagulation studies were also reviewed. DIC was considered to be present if two of the following abnormalities were present: platelets were described as low or were < 100 x 109/l; fibrin degradation products were elevated; fibrinogen was described as low or was < 2g/l; or prothrombin time was prolonged, > 1 s from control, or > 14 s. An absent spleen was assumed if specifically mentioned, if the patient had sickle cell anemia, or if asplenia was documented at autopsy.

    0 1997 Scandinavian University Press. ISSN 0036-5548

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  • 480 C. T. Carpenter and A . B. Kaiser Scand J Infect Dis 29

    Table I. Clinical and laborutory findings in patients presenting with pneumococcemia, fever, and purpura fulminans

    DIC Values Case No. Spleen Clinical (Ref) Age/Sex Hypotension absent DIC"? Abnormal Normal gangrene Outcome

    1 (2) 2 (3) 3 (4) 4 (4) 5 ( 5 ) 6 (6) 7 (7) 8 (8) 9 (8)

    10 (9) 11 (10) 12 (11) 13 (11) 14 (12) 15 (13) 16 (14) 17 (15) 18 (16) 19 (17) 20 (17) 21 (18) 22 (19) 23 (20) 24 (21) 25 (22) 26 (23) 27 (24)

    37 (26) 38 (27) 39-41 (28)*** 42 (29) 43

    28-36 (25)**

    9 1 ~ 60/M 61/F 37/M 32/F

    19/M 16/M 70/M 34/F 22/M 22/F 70/F 31/M 63/F 65/M 80/M 62/F 2/F

    8 mo/M 34/M 33/F 40/F 31/M 34/M 23/M

    1 IF Adults 43/F 67/F 1 5 y*** 60/F 46/M

    5 1 ~

    No No No Yes* N o Yes Yes Yes Yes Yes No Yes Yes Yes Yes Yes Yes Yes Yes Yes Yes Yes Yes Yes No Yes No Yes Yes Yes Yes Yes No No No No ID Yes ID Yes No Yes No Yes Yes Yes No No No Yes No Yes Yes No 3/9** 4/9** Yes No Yes No 3/3*** 0/3*** Yes No No Yes

    ID IDb ID ID Yes Yes No Yes No Yes Yes ID Yes Yes Yes Yes Yes ID Yes ID Yes Yes Yes No Yes Yes Yes 7/9** Yes Yes ID Yes Yes

    PT, F

    P, F

    p, F

    F

    F F F P F PT, F

    F

    p, F F

    F

    F

    Yes Yes No No Yes Yes Yes No ID Yes Yes Yes Yes Yes Yes Yes Yes Yes No No Yes Yes No No Yes Yes No 9/9** Yes Yes ID No Yes

    Survived' Survived'.d Expired Expired Survivedc Survived Survived Expiredd Expiredd Expired Expired Survived' SurvivedC Survived Unknown Expired Expired Expired Expiredd Survivedd SurvivedC Survived Survivedd Expired" Survivede Survived" Expired" 9/9** Survived Expiredd Survived" 2/3*** Survived Expired Survived"

    a Disseminated intravascular coagulation defined as present if so stated by the authors or if abnormalities occurred in at least two of the following laboratory tests: platelets (P), defined as low (JP), if so stated or if reported as < 100 x 109/1; fibrin degradation products (FDP), defined as elevated (TFDP), if so stated; prothrombin time (PT), defined as prolonged (TPT), if so stated or if z 1 s from control or > 14 s; fibrinogen (F), defined as low (JF), if so stated or

  • Scdnd J Infect Dis 29 Purpura Fulminans 48 1

    hypotensive had adrenal hemorrhage (1 documented by autopsy and the other by ultrasound). A trend of higher mortality among hypotensive patients was observed; 50% of patients presenting with hypotension and purpura died compared to only 20% of those presenting with purpura alone (p = 0.097, x2 with Yates correction). Coagulation studies were reported for 33 patients, 28 (85%) of whom had a profile consistent with DIC. Seven patients had skin biopsies, 5 of which revealed the classically described lesion of purpura fulminans, i.e. dermal thrombosis and hemorrhage. Of the 19 patients for whom plasma fibrinogen levels were reported, 14 (74%) had normal or elevated levels. However, fibrin degradation products were elevated in all 12 patients for whom these values were reported. Survival had no apparent correlation with any of the parameters of DIC.

    DISCUSSION

    This review reveals several interesting features of purpura fulminans in pneumococcal sepsis, the first being that hy- potension is not a necessary feature of the syndrome, occurring in only 51% of reported cases. In comparison, literature reviews of meningococcal sepsis indicat