Waterhouse-Friderichsen syndrome

Acute adrenal insufficiency due to massive haemorrhage into the adrenal gland, more often bilateral, caused by malignant form of meningitis. It occurs in fulminant bacterial infections, due principally to the meningococcus, influenza or colon bacillus. The onset of the syndrome is dramatically sudden. The patient exhibits a cyanotic pallor, a pechial or purpuric rash, and sometimes large purpuric cutaneous haemorrhages often followed by necrosis and sloughing. In the preshock stage, patients are alert but pale with coldness and cyanosis of the extremities due to generalized vasoconstriction. Fever initially moderate, then high.

Evidence of acute adrenal haemorrhage is manifested by circulatory collapse, which is characterized by clammy skin, high fever, a rapid thready pulse, laboured respiration, an alarming drop in blood pressure, and coma. Other features may include dehydration, vomiting, diarrhoea, oliguria, neck stiffness, and occasionally anuria. Occur usually in infants or children, occasionally in adults. Death usually after a few hours, adrenal insufficiency being the immediate cause. patients who recover may suffer from extensive sloughing of the skin and loss of digits due to gangrene.

First described in 1894 by Arthur Francis Voelcker (1861-1946) and in 1901 by the British dermatologist Ernest Gordon Graham Little (1867-1950). It was first reported as an entity by Waterhouse in 1911, and the subject was comprehensively reviewed in 1918 by the Danish paediatrician Carl Friderichsen.

• F. Marchand:
  Über eine eigentümliche Erkrankung des Sympathicus, der Nebennieren der peripherischen Nerven (ohne Broncehaut).
  [Virchow’s] Archiv für pathologische Anatomie und Physiologie und für die klinische Medizin, 1880, 81: 477-502.
• A. F. Voelcker:
  Middlesex Hospital Rep med Surg path registrars (1894), 1895, 278.
  Abstract of post mortem report, no title.
• E. G. G. Little:
  Cases of purpura, ending fatally, associated with hemorrage into the suprarenal capsules.
  The British Journal of Dermatology, 1901, 13: 445.
• R. Waterhouse:
  A case of suprarenal apoplexy. Lancet, 1911, I: 577-578.
• C. Friderichsen:
  Nebennierenapoplexie bei kleinen Kindern.
  Jahrbuch für Kinderhilkunde, 1918, 87: 109-125.