WaterhouseFriderichsen Syndrome is adrenal gland failure due to adrenal haemorrhage. It is an uncommon but usually life-threatening condition, which can be one of the serious complications of severe sepsis. Clinical manifestations of adrenal haemorrhage can vary widely depending on the degree and rate of haemorrhage. Adrenal haemorrhage may result from trauma, sepsis, anticoagulation medication, coagulopathy, autoimmune conditions, underlying tumour or idiopathic disease. We present a case of WaterhouseFriderichsen syndrome in a 45-year-old female with no significant past medical history apart from hypertension, who was admitted for sepsis with pyrexia, extreme lethargy, recent UTI and diarrhoea and digital finger ischaemia, complicated by sudden onset refractory shock and disseminated intravascular coagulopathy. There was a note of digital splinter haemorrhages with no generalised rash. There was no evidence of infective endocarditis on echocardiogram. CT abdomen showed inflammatory colitis with mesenteric lymphadenopathy and bilateral non traumatic adrenal haematomas. All microbiological cultures and viral serology were negative. She was immediately treated with intravenous antibiotics and steroids and subsequently made good recovery. Patient was discharged with replacement hydrocortisone and fludrocortisone and followed up in the endocrine clinic. In view of digital ischaemia, left calf skin ulcer and mononeuritis multiplex, she was reviewed by Rheumatology and Dermatology and managed as antiphospholipid syndrome with vasculitis. Her skin biopsy result of left calf ulcer showed dermal ulcer with occlusive thrombi and she was initiated on warfarin and prednisolone.
Conclusion: In the medical emergency setting, it is important to consider the diagnosis of WaterhouseFriderichsen Syndrome, based on the clinical features of adrenal insufficiency (refractory shock) in a septic patient. This case highlights the value of prompt recognition and rapid steroid replacement, which are lifesaving. Finally, investigations for the underlying aetiology of adrenal haemorrhage, such as sepsis and autoimmune condition, should be undertaken.