Severe inflammatory response and vasculitis leading to quadruple limb\namputations
A previously fit 33-year-old Somali woman returned from holiday in Canada with an\nurticarial rash on her upper arms accompanied by fever, dyspnoea, polyarthralgia, fatigue\nand headache. The rash resolved but the other symptoms continued, and after four weeks she\nreceived Clarithromycin for pharyngitis. A week later, she suffered a miscarriage at six\nweeks’ gestation (G4 P3) and was given Trimethoprim for a urinary tract infection. After a\nfurther week of persistent symptoms, she was admitted for treatment of pneumonia with IV\nMeropenem and Clarithromycin but deteriorated and was admitted to the intensive treatment\nunit (ITU) with type I respiratory failure. She was tachycardic with cool peripheries but\nnormotensive, and all peripheral pulses were palpable. She was pyrexial (38.9℃) and fully\nalert with no focal neurology. The differential diagnosis on ITU admission was systemic\ninflammatory response syndrome (SIRS) secondary to sepsis or pulmonary embolism (PE). She\ncontinued on IV antibiotics and was commenced on therapeutic low-molecular weight heparin\n(Clexane) until CT pulmonary angiogram excluded PE. Echocardiogram excluded endocarditis and\nevacuation of the uterus showed no retained products. All cultures were negative and blood\nresults revealed a white cell count of 25.1 × 109/L, CRP of 197 nmol/L and\nFerritin >40,000 ng/mL. All other investigations were unremarkable (). She soon required\nintubation and ventilation and the initiation of two vasopressor infusions (dopamine and\nnoradrenaline). After 48 h, she remained pyrexial and active cooling was commenced with\nhaemofiltration; this necessitated anticoagulation with IV unfractionated heparin. Within\n24 h, she developed a non-blanching, purpuric rash over her breasts, abdomen and all four\nlimbs which rapidly developed into large, tense bullae and full-thickness necrosis at the\nfingertips and toes. An abdominal skin biopsy revealed necrotizing small vessel vasculitis.\nShe received a three-day course of 1 g methylprednisolone; however, after 48 h the necrosis\nhad covered the limbs, breasts and abdomen, and peripheral pulses were no longer palpable.\nAfter a further week with continued skin loss and minimal response to steroids, it was\nconsidered that her condition may be due to adult Still’s disease. Other causes had been\nexcluded; she had a raised Ferritin level and met the Yamaguchi criteria. For this reason,\nshe was commenced on Anakinra (100 mg daily), which had shown some benefit in previous case\nreports of adult Still’s disease. She responded rapidly and within a week was extubated with\ndecreasing vasopressor requirements. On further investigation, an angiogram showed\nasymmetric iliac arteries with no flow beyond the popliteal arteries, and a muscle biopsy\nrevealed non-viable tissue below the knees. She underwent bilateral above-knee and\nbelow-elbow amputations and debridement of skin over her abdomen and breasts. Five months\nlater, she has undergone extensive skin grafting and is now receiving rehabilitation with\nlimb prostheses.