common causes of haematochezia in adults are diverticular disease , vascular ectasia , colitis ( ischaemic , inflammatory or infectious ) , colonic neoplasia and anorectal conditions ( 1 ) .
haematochezia is not reported as a common mode of presentation among patients with cronkhite - canada syndrome ( ccs ) in which diarrhea and dysgeusia are the predominant initial presentations ( 2 ) .
surgery in patients with ccs has been carried out for associated gastrointestinal tract malignancies , intestinal obstruction and severe protein loosing enteropathy ( 3 ) .
we present a case of persistent severe haematochezia in a patient with ccs , successfully managed with proctocolectomy .
a 50-year - old sri lankan man presented with a 3-month history of diarrhoea , haematochezia and weight loss of 20 kg .
one month prior to these symptoms he has noticed dark pigmentation of the skin and hair loss . physical examination revealed a cachectic man with alopecia , generalized skin hyperpigmentation involving the palms and onychodystrophy of fingernails ( fig 14 ) .
diffuse skin hyperpigmentation involving the palms biochemical investigations revealed a haemoglobin value of 9g / dl and marked hypoalbuminaemia with serum albumin of 12g / l ( normal- 36 - 44g / l ) .
upper gastrointestinal endoscopy and colonoscopy revealed multiple , sessile polyps of varying sizes distributed throughout the stomach , duodenum , colon and rectum without any polyps in the oesophagus .
video capsule endoscopy revealed numerous similar polyps throughout the small intestine ( fig 4 ) .
onychodystrophy of finger nails biopsy of colonic polyps revealed cystic dilatation of crypts with oedema of the lamina propria suggesting hamartomatous polyps .
thus , a diagnosis of cronkhite - canada syndrome was made , considering the characteristic ectodermal abnormalities on physical examination , endoscopic findings and histology .
haematochezia worsened despite medical treatment for 2 months and proctocolectomy with a permanent ileostomy was performed .
histology of the specimen revealed multiple polyps with a diverse spectrum of morphologic features including juvenile - type polyps , adenomatous polyps , serrated adenomas , hamartomatous polyps and mixed polyps .
first described in 1955 , cronkhite - canada syndrome ( ccs ) is a rare , nonhereditary polyposis syndrome with an uncertain aetiopathogenesis . approximately about 400 cases have been reported in the literature with the majority being reported from japan .
it is characterized by the presence of diffuse gastrointestinal polyposis ( sparing the oesophagus ) associated with characteristic ectodermal abnormalities such as alopecia , nail dystrophy and cutaneous hyperpigmentation ( 4,5 ) .
associations with raised ana and igg4 levels , hypothyroidism and various autoimmune diseases such as systemic lupus erythematous , rheumatoid arthritis and scleroderma have been published in the literature ( 6 ) .
most of these patients present with diarrhea and dysgeusia and the dermatological manifestations are known to appear later ( 2 ) .
the presentation in our patient is different because he had not developed dysgeusia , his cutaneous symptoms have preceded the onset of diarrhoea and haematochezia was one of his key presenting features .
, different treatment modalities have been attempted with varying degrees of success ( 7 ) .
these include hyperalimentation , corticosteroids , h2-receptor antagonists , antibiotics , acid suppression , cromolyn sodium , anabolic steroids , surgery , and combinations of these therapies ( 7 ) . among the various complications
this can be as high as 15% and both gastric and colorectal malignancies are known to occur in these patients ( 8,9 ) .
some studies have shown overall mortality rates as high as 55% among their patients ( 2 ) . to our knowledge , this is the first case of total proctocolectomy for refractory haematochezia in a patient with ccs .
this also highlights the importance of considering cronkhite - canada syndrome as a rare possibility in a middle aged male presenting with haematochezia , specially if the patient is of asian origin and if there are any cutaneous abnormalities suggestive of this condition .
 cronkhite - canada syndrome is an extremely rare condition of gastrointestinal polyposis in which the main presenting features are diarrhoea and dysgeusia . 
 the polyps in this condition are characteristically distributed throughout the entire gastrointestinal tract except the oesophagus , and these patients exhibit unique ectodermal abnormalities . 
 herein , we report a 50-year - old male who had recurrent episodes of severe haematochezia from the polyps in the colon . further examination and investigations revealed a diagnosis of cronkhite - canada syndrome . 
 proctocolectomy was carried out for medically refractory haematochezia and the patient is asymptomatic at present . 