Purpura fulminans complicating puerperal sepsis
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A 30-year-old P3L3 woman presented eight days postpartum, following an uneventful vaginal delivery, with high-grade fever, loose stools and cough for three days, and bluish discoloration of the right ankle which rapidly progressed to involve the limbs, abdomen, and perineum. Her present pregnancy, past pregnancies, and family history were insignificant. Upon examination, the patient was febrile and hemodynamically unstable. The extremities showed extensive purpurae and bullae on both feet extending just above the ankle and the dorsum of the left hand with gangrenous changes in the left ring finger. These lesions were tender and intensely pruritic. Postpartum, the patient’s uterus was mildly tender, the lochia exhibited a foul smell, and internal examination revealed forniceal tenderness.

Blood tests revealed mild anemia, leukocytosis, and marked thrombocytopenia. Hepatic and renal functions were deranged. Coagulation profile was abnormal, fibrin degradation product (FDP) and D-dimer were markedly elevated, consistent with DIC. Escherichia coli was isolated from vaginal swab. Chest radiograph showed pneumonia and bilateral pleural effusion. Ultrasound of the abdomen–pelvis and Doppler of bilateral lower limb vessels were normal. The patient began treatment with intravenous piperacillin-tazobactam (4 g/0.5 g TDS) and teicoplanin (400 mg QID) which was later changed to tigecycline as per culture-sensitivity results. Eight units of platelets, 10 of fresh frozen plasma, and 13 units of cryoprecipitate were administered along with supportive treatment for end-organ damage. Therapeutic low molecular weight heparin was initiated at 60 mg subcutaneously for 6 weeks, followed by warfarin (5 mg OD) and Pentoxifylline (TDS). The patient showed remarkable improvement within five days of treatment. She recovered but developed dry gangrene on the toes of both feet and the left ring finger for which she opted for auto-amputation. The patient was discharged 49 days post-admission and advised to attend regular follow-up.

There are no established guidelines on management of PF as knowledge of the disease is limited. Broad spectrum antibiotics, blood products, and supportive management form the mainstay of treatment. Heparin is recommended to stabilize coagulation cascade and arrest disease progression, while carefully balancing the risk of life-threatening bleeding in the presence of DIC. The role of antithrombin III, activated protein-C, and therapeutic plasma exchange remains controversial. The potential of corticosteroids is untapped despite evidence of promising results in severe sepsis. PF is a life-threatening condition requiring a high degree of suspicion and prompt treatment due to its potentially devastating consequences. A multidisciplinary approach can enhance therapeutic outcomes

Source: https://obgyn.onlinelibrary.wiley.com/doi/abs/10.1002/ijgo.13342
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