The Waterhouse-Friederichsen syndrome

Abstract

VOLCKER first described fulminating purpura with bilateral adrenal hmmorrhage in 189.4. BLAHER and BAILEY suggested in 1901, that it was blood poisoning. LITTLE in the same year could find no bacteriological evidence of infection in his series of cases. WATERHOUSE gave detailed description of the disease enti ty in 1911, under the title "Supra-renal apoplexy " and reported one case in a boy aged 8 months. He stated "The case related above appears to form one of a distinct group, examples of which have been described by VOLCKER, STILL, GARROD and DRYSDALE, BATTEN, ANDREWS, TALBOT, BLATTER and BAILY, GRAHAM LITTLE, LONGMEAD and DUDGEON, omitting the case of STILL, in which miliary tuberculosis also was found at the necropsy and that of BATTEN in which hzemorrhage occurred into one supra-renal capsule only in a boy aged 2.', years ; there remains, including that just described, 15 cases, all infants between the ages of 2 and 15 months, and all of whom died after an illness lasting in the majority less than 24 and in none more than 48 hours. In all but three a hmmorrhagic rash of greater or less extent was present. Vomiting occurred in 6, diarrhoea in 1, convulsions in 5, and symptoms suggestive of abdominal pain in 3. In several cases sign of bronchitis was present. The temperature in most between 100 ~ and 103~ Volcker's case 105 ~ , and in one of GRAHAM LITTLE reached 108 ~ before death. Apart from the lesions of the adrenals and the hmmorrhagic rash, the most constant post-mortem findings have been intense engorgement of the lungs. Cultures from various organs have in most cases proved negatiw~ but in one of TALBOT'S streptococcus was obtained by DRYSDADE from the supra-renal cal~sule after death. (Pp. 578)." In 1918, FRIEDERICHSEN reported 12 cases of his own and summarised the literature. In 1933, the clinical pathological entity was first designated as "The Waterhouse-Friederichsen Syndrome" (GLANZMAN). So far there had been 125 reported cases in the world literature.