A 34-year-old male was brought to emergency services due to loss of consciousness. His capillary blood glucose was 37 mg/dL in the ambulance, so 20% dextrose was infused intravenously. At emergency services, his physical examination indicated that he was confused and had a blood pressure of 70/50 mmHg and pulse of 95/min, and temperature was 37 C. His skin and oral mucosa had widespread hyperpigmentation and scars were on both legs (Figure 1). Other laboratory examination results were 1 mg/dL creatinine, 135 mmol/L sodium, 6 mmol/L potassium, 11.7 g/dL hemoglobin, 7.14 K/muL leukocyte count, 83 K/muL thrombocyte count, 5.79 muIU/mL thyroid stimulating hormone, and 1.36 ng/dL free T4. Cortisol and adrenocorticotropic hormone levels (1.91 mug/dL and 550 pg/mL, resp.) were evaluated with the initial diagnosis of adrenal insufficiency due to widespread hyperpigmentation, hypotension, hypoglycemia, and hyperkalemia. Acute adrenal crisis treatment was started and the patient was hospitalized to evaluate the causes of PAF.
He had a history of nausea, vomiting, weakness, fatigue, and skin hyperpigmentation for 1.5 years. He had two presyncope attacks. In addition, he had a medical history of depression and his medications included sertraline (50 mg/day) and olanzapine (5 mg/day).
The patient was evaluated for possible causes of PAF. The magnetic resonance imaging of the adrenal glands was normal. The adrenal hormone measurements (and normal ranges) were 69.8 ng/L (5.3-99.1) direct renin, 40.2 pg/mL (38.1-313.3) aldosterone, 9.27 mug/24 hours (88-444) metanephrine, and 55.03 mug/24 hours (52-341) normetanephrine. He was evaluated for human immunodeficiency virus, syphilis, disseminated fungal infections, and tuberculosis, but no infectious disease was detected. He had no history of drugs causing adrenal insufficiency. He was also evaluated for autoimmune polyglandular syndrome, but the parathormone, C-peptide, calcium, and testosterone levels were normal. No other autoimmune disease accompanied the primary adrenal failure.
A bilateral low extremity arterial and venous Doppler ultrasound was performed because of the varicose veins, scars, and stasis ulcers in his legs. The Doppler ultrasound revealed bilateral thromboses in the main femoral vein, right deep femoral vein, superficial femoral vein, popliteal vein, and right vena saphena magna (Figure 2). No thrombus was detected with echocardiography. For thrombosis etiology, blood samples were taken for protein C, protein S, activated partial thromboplastin time (aPTT), and prothrombin time. The results and normal ranges were 85.2% (70-140), 82.4% (60-130), 77.7 seconds (20-35), and 15 seconds (11-16), respectively. Antiphospholipid syndrome was suspected due to the increased aPTT levels and thrombosis. The laboratory test results for antiphospholipid antibodies were 191.2 U/mL (0-15) anticardiolipin IgG, 33.7 U/mL (0-12) anticardiolipin IgM, 14.2 U/mL (0-15) anti-beta-2 glycoprotein IgM, 229.6 U/mL (0-15) anti-beta-2 glycoprotein IgG, and 0.6 U/mL (0-0.8) antinuclear antibody. After 12 weeks, the repeated tests for antiphospholipid antibodies were also high. Based on the revised Sapporo criteria, the patient was diagnosed with primary antiphospholipid syndrome because no disease related to antiphospholipid syndrome was seen. Anticoagulation treatment was started for APS. The patient is now following up with hydrocortisone, fludrocortisone, and warfarin sodium.