Purpura fulminans complicating pneumococcal sepsis.

Abstract

Sir: Purpura fulminans is an unusual, often fatal syndrome, complicating bacterial, viral and fungal illnesses, mainly meningococcaemia, varicella, and scarlet fever [1±4]. Cases secondary to pneumococcal infection are extremely rare, occurring in individuals with predisposing factors such as hypogammaglobulinaemia, HIV infection, splenectomy, sickle cell disease, alcoholism, or diabetes mellitus [1±3]. A previously healthy 6-month-old girl was transferred to the University Hospital with a 24-h history of fever up to 40°C, purpuric rash and rapid deterioration. On admission the infant appeared gravely ill; capillary re®ll was prolonged, respiratory rate 47/min, pulse rate 160/min and blood pressure 100/60 mm Hg. Di€use purpuric patches were noted, more prominent over the extremities, and the head and the soles were markedly cyanotic. Laboratory ®ndings were: haematocrit, 25.3%; white blood cell count 32.7 10/L; neutrophils 22.9 10/L; platelets 106 10/L; international normalised ratio(INR) 3.9; activated partial thromboplastin time 93 s(normal 30 s); ®brinogen 141 mg/dL (normal 150±400 mg/dL); urea nitrogen 99 mg/dL; creatinine 1.4 mg/dL; bicarbonate 11.9 mmol/L; alanine aminotransferase 592 U/L; calcium 5.3 mg/dL; erythrocyte sedimentation rate 4 mm/h; and C-reactive protein 94 mg/dL. A chest roentgenogram showed cardiomegaly and bilateral lung in®ltrates. Lumbar puncture revealed 13 cells, protein 55 mg/dL and glucose 63 mg/dL. CSF, blood and skin lesion cultures grew Streptococcus pneumoniae, sensitive to penicillin but resistant to chloramphenicol. Acute and convalescent levels of immunoglobulins lgA, lgE, IgG, IgM, as well as complement C3 and C4 levels were normal. Levels of protein C, protein S and antithrombin III were not measured. Intensive uid resuscitation with fresh frozen plasma and packed red cells was started; parenteral penicillin, ceftriaxone, dexamethasone, and cimetidine were administered. The infant remained in critical condition over the following 3 days, presenting a series of complications including left-sided pneumonia, cardiac failure and atrioventricular block, gastroplegia, melena, microscopic haematuria, hypertonia, generalized seizures, schistocytosis, positive direct Coomb's reaction and elevation of serum creatine kinase to 21,950 U/L and of lactate dehydrogenase to 6,400 U/L. The skin lesions turned to sharply demarcated ecchymoses with dry gangrene on the distal part of feet; the toes were insensitive to pin prick but the distal pulses remained palpable. Fresh frozen plasma on a daily basis and aspirin in low doses (5 mg/kg body weight) were administered for 2 weeks. After 3 weeks, the girl was discharged. Her skin lesions healed gradually; the single residual lesion consisted of nail loss in the left small toe. The 33 month follow up period was uneventful. This case was characterised by overwhelming pneumococcal sepsis and multiple organ damage with distal extremity ischaemia leading to symmetrical dry cutaneous gangrene. This is the typical scenario described in the rare cases of purpura fulminans complicating pneumococcal infection [1±3]. Contrary to analogous cases, however, where a striking history of immunode®ciency was present, in this infant no such de®ciency was traced. Management remains controversial. Auto-amputations or amputations are the rule and mortality rates up to 30% have been reported [1±4]. In this infant, fresh frozen plasma for replacing the consumed coagulation factors and aspirin for decreasing the platelet aggregation were preferred, with satisfying outcome. In conclusion, purpura fulminans may complicate pneumococcal sepsis in immunocompetent patients. Judicious haemodynamic support, management of the numerous complications, and avoidance of early operative intervention may be helpful resulting in ultimate tissue loss less severe than initially anticipated.