Abstract:
Cronkhite–Canada syndrome is a rare gastro-enterocolopathy
of uncertain aetiology first described
almost 60 years ago. It is characterised by diffuse
gastrointestinal polyposis sparing only the oesophagus,
ectodermal abnormalities and an unpredictable but often
fatal clinical course. The disease may demonstrate
extremely diverse clinical and endoscopic features, which
often leads to a delay in diagnosis. A high index of
suspicion and recognition of the characteristic
histological findings frequently facilitate a correct
diagnosis, but the distribution of the gastrointestinal
pathology and its microscopic features may be atypical.
The pathologist thus requires a thorough knowledge of
both the typical and many atypical faces of this disease,
for which various documented therapies often still prove
ineffective. Close correlation with clinical findings,
including any pertinent ectodermal abnormalities, and
careful examination of biopsies derived from polypoid
and endoscopically spared mucosa will ensure a timely
and correct diagnosis in patients with this enigmatic
syndrome.
