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Annals of the Rheumatic Diseases logoLink to Annals of the Rheumatic Diseases
. 2001 Jan;60(1):69–71. doi: 10.1136/ard.60.1.69

Prolonged prodrome, systemic vasculitis, and deafness in Cogan's syndrome

S Van Doornum 1, G McColl 1, M Walter 1, I Jennens 1, P Bhathal 1, I Wicks 1
PMCID: PMC1753354  PMID: 11114287

Abstract

Cogan's syndrome is a rare, multisystem disease which occurs predominantly in children and young adults. It was originally described as the combination of interstitial keratitis and audiovestibular disturbance, but other forms of ocular disease, as well as systemic vasculitis, have since been recognised as part of the syndrome. Diagnosis can be difficult if the various manifestations occur separately, but early recognition is important because prompt treatment may prevent deafness. Two cases are presented here illustrating the features of this disease, and providing histological evidence of systemic vasculitis in both.



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Figure 1  .

Figure 1  

Computed tomography of the abdomen of patient 1: 4 cm hypodense lesion in the liver.

Figure 2  .

Figure 2  

(A) Liver biopsy patient 1. Portal tract granuloma (arrow) and inflammatory cell infiltrate. (Haematoxylin and eosin stain, original magnification ×400.) (B) Skin biopsy patient 1. Occluded artery in subcutaneous fat with adventitial inflammation. (Haematoxylin and eosin stain, original magnification ×400.)


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