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CN: Bilateral Symmetrical Gangrene (Purpura Fulminans) -
Opinion for management
Posting on Nephrol From Malvinder Parmar

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A 31-year old woman with unremarkable past medical history (about 1-year postpartum), presented initially in September 2001 with acute Pumonary-renal syndorme (only two weeks history of flu-like illness, fatigue and shortness of breath) with bilateral pulmonary infilterates and renal failure with serum creatinine of 400 umol/L and active urine sediment, elevated ESR of 110 and marked anemia with hemoglobin of 64 G/L.

Wondered about Vasculitis, Wegener's, Goodpasteur's. Lupus etc.  Performed renal biopsy that showed Immune-complex necrotizing glomerulonephritis with advanced glomerulosclerosis.

Serology: ANCA: Negative, anti-GBM: Negative, slightly low C3. ANA weakly positive with titre of 1:80.  Anti-DNA was not done, as initially the likelihood of lupus was thought to be low and possibility of vasculitis was high on the list.

Started on Immunosuppression - Bolus Prednisolone followed by Oral Prednisone and oral Cytoxan and Septra.

Diagnosis in the end was felt to be "Immune complex nephritis, likely secondary to Lupus" although patient had no previous symptomatology of Lupus or any other disorder.  Because of advanced glomerulosclerosis, it was felt and discussed with the patient that less likley kidneys will respond but a trial of immunosuppressive therapy was justified.

In a month, the pulmonary infilterates completely resolved and Chest X-ray was normal but renal function initially responded slightly but continue to progress and then started on hemodialysis with continuing immunosuppression with weekly monitoring of WBC.

On Decemebr 8, she presented to ER with febrile neutropenia (WBC of 0.4, a  week before her WBC were normal at 7.8) with BP of 70 mmHg - Febrile Neutropenia with Septic shock and new pulomary infilterate.  She was managed with Fluids, Inotropic support (required Dopamine and Levophed for 72-hours) daily dialysis, antibiotics (Ceftizadime, Piperacillin and iNtermittent Gentamycin) and ventilatory support.  Blood cultures were positive for Pseudomonas.  We also initially planned to give activated Protein C (although not available in Canada but after reviewing the NEJM paper - renal failure patients on dialysis were not included in the study and by the time we would have received the drug it might have been beyond 72-hours after initial presentation, hence not given).

She developed overwhelming DIC (disseminated intravascular coagulation) with D-dimer up to 12, thromobocytopenia and required supportive therapy.  I also gave small dose of heparin but then developed bleeding hence need to stop.  But then by day 4th developed the untoward complication and her both lower extremities about 8 cm below the knees developed bluish discoloration and were cold to touch (see aabove photograph).  Asked vascular surgeon to see if any thing could be done, told me that she has developed 'rigor mortis' of both legs and will require bilateral below knee amputations and by Doppler he could not find popliteal pulse beyond about 3-4 cm below the knee joints.

Continued with ventilatory support, antibiotics and dialy dialysis and Transparenetral nutrition. About 8th day or so, saw some improvement in color of legs and then did some literature search to see if anything could be done to save her legs.  Came across a nice review on "bilateral symmetrical gangrene - Purpura fulminans - also known as "Dopamine gangrene" a rare complication of severe sepsis, septic shock especially with meningococcemia with upto 80% mortality.  There appear to be only about 70-80 cases reported in literature. As few of the cultures were later groing candida in sputum, I also added I/V Fluconazole.

Then, treated her with transdermal nitrates (on lower extremities), intraarterial chlorpromazine and intravenous infusion of Prostaglandin E2 for 72-hours.  Successfully extubated her on post X-mas day.  Legs remains discolored with clear demarcation line at soles and tips of her toes but lower 2/3rd of the legs remain discolored with almost no sensations, decreased muscle activity in this part of legs.

Asked Orthopedic surgeon, who felt that she may require above knee amputations and vascular surgeon again and he also feels that although there is improvement in flow but the muscles in lower 2/3rd of legs are atrophied, the ankle is mainly plantiflexed (though she has some movement at both ankles) that she likely would require bilateral BKA's.   The literature (review article suggest that usually there is autoamputation and surgical amputation may not be required unless there is active infection but debridement might be required).

I need help in the non-nephrological management of this patient's legs - that are still discolored but appears to have some perfusion and are not stiff as were initially (when surgeon described the legs having rigor mortis).  Do you have experience in management of such a case or know of ICU or surgical staff that may be of help.

I could not find anything on the long-term outcome of the extremities, in such patients who survived.

Appreciate any thoughts!

Thank you.

M. Parmar, MD, FRCPC, FACP
Timmins, ON. Canada

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