Addisonian crisis in autoimmune polyglandular syndrome type II

A 16-year-old female patient presented to the pediatric surgical outpatient clinic with abdominal pain, a local wound infection, fever, and vomiting 14 days after an appendectomy. After a second presentation in the same night the patient was admitted to hospital due to dehydration and significantly reduced general condition. Due to arterial hypotension and tachycardia despite infusion therapy, the patient was transferred to the intensive care unit with suspected sepsis. In the absence of improvement despite escalation of antibiotic therapy, a treatment-resistant hypotension and a conspicuous hyperpigmentation raised the suspicion of an Addisonian crisis. After the administration of hydrocortisone there was a rapid improvement in the clinical symptoms. Hormone and autoantibody testing confirmed the diagnosis of Addison's disease. Combined with the recently diagnosed Hashimoto's thyroiditis, an autoimmune polyglandular syndrome (APS) type II was identified.