- Etiology/Pathophysiology
- Clinical Features
- Onset is sudden and fulminant. The condition is a medical emergency.
- Presents as a complication of severe sepsis in a patient, often a child or young adult.
- Septic shock: Profound hypotension (often refractory to fluids/pressors), tachycardia, fever.
- Adrenal crisis: Weakness, vomiting, confusion, or coma due to lack of cortisol and aldosterone.
- Rash: A characteristic widespread petechial or purpuric rash that can progress to ecchymoses is a major clue, indicating underlying DIC.
- Diagnostics
- Diagnosis is primarily clinical, based on the rapid deterioration in a septic patient.
- Labs:
- Hypoglycemia: Due to cortisol deficiency.
- Electrolytes: Hyponatremia and hyperkalemia due to aldosterone deficiency.
- CBC: Thrombocytopenia. Leukopenia can be a poor prognostic sign.
- Coagulation studies: ↑ PT, ↑ PTT, ↓ fibrinogen, ↑ D-dimer, consistent with DIC.
- Hormonal studies: ↓ Cortisol, ↑ ACTH. An ACTH stimulation test would show no cortisol response, but treatment should not be delayed for testing.
- Imaging: Abdominal CT can confirm bilateral adrenal hemorrhage but is often not performed due to patient instability.
- Treatment
- Immediate emergent management is critical.
- High-dose corticosteroids: IV hydrocortisone or dexamethasone to treat the adrenal insufficiency. This is life-saving.
- Antibiotics: Broad-spectrum IV antibiotics immediately after blood cultures are drawn (e.g., ceftriaxone for suspected meningococcemia).
- Supportive care: Aggressive IV fluid resuscitation and vasopressors to manage septic shock.
- Complications
- High mortality rate if not recognized and treated immediately.
- Multi-organ failure from septic shock.
- Skin necrosis and gangrene of digits or limbs may occur.
- Survivors may require lifelong glucocorticoid and mineralocorticoid replacement.