TEN-like presentation of purpura fulminans secondary to subgroup Y meningococcaemia.

A 63-year-old woman presented to ED with acute onset vomiting, sore throat and altered mental status, following several days of intractable headache and malaise. She was hypotensive and developed rapidly evolving purpura. She was treated with IV ceftriaxone, penicillin and dexamethasone for presumed invasive meningococcal disease and transferred to ICU. Blood cultures grew Neisseria meningitidis subgroup-Y. Over 24 h, the patient progressed to purpura fulminans and multi-organ failure. Skin desquamation was noted on day-2 and frank blistering on day-3. Upon Dermatology review (day-4), there were widespread bullae (40% BSA) with positive Nikolsky sign and oral mucosal involvement. Skin biopsies showed a shallow, cell-poor subepidermal vesicle and full thickness epidermal necrosis. No dermal microvascular thrombosis or vasculitis, features commonly seen in infectious purpura fulminans (1), were seen. It was postulated this was either a TEN-like presentation of purpura fulminans, where epidermolysis is a well-known phenomenon (2), or concomitant TEN secondary to antibiotic therapy. Antibiotics were changed to moxifloxacin and she received three consecutive daily doses of IVIG 1 g/kg. There was improvement in other organ systems but the involved skin progressed to 70% BSA. She was transferred to the statewide burns service, however the skin changes were ultimately deemed too extensive for grafting/artificial skin and she passed away some days later. This case presents multiple learning opportunities, including the challenge in differentiating between purpura fulminans and TEN clinically and histologically, the importance of early recognition of both conditions to direct treatment, and changing incidence of N.meningitidis subgroups in Australia.