Polyposis, Skin Pigmentation, Alopecia, And Fingernail Changes

Description

Cronkhite-Canada syndrome is characterized by gastrointestinal hamartomatous polyposis, alopecia, onychodystrophy, skin hyperpigmentation, and diarrhea. It is associated with high morbidity (summary by Sweetser et al., 2012).

Clinical Features

Cronkhite and Canada (1955) described 2 unrelated patients with a syndrome of gastrointestinal polyposis, skin hyperpigmentation, alopecia, onychodystrophy, and diarrhea. Another patient with similar features was reported by Jarnum and Jensen (1966). Manousos and Webster (1966) reported the fourth patient. All 4 cases were sporadic and all patients were adults. The prognosis is poor. The pigmentation is diffuse rather than spotted as in the Peutz-Jeghers syndrome (175200).

Sweetser et al. (2012) described 14 consecutive cases of Cronkhite-Canada syndrome seen at the Mayo Clinic on whom tissue and follow-up were available. Diffuse polyposis throughout the gastrointestinal tract with esophageal sparing and ectodermal abnormalities (alopecia, onychodystrophy, and skin hyperpigmentation) were seen in most patients. Laboratory findings suggested that malabsorption with hypoalbuminemia was nearly universal. A significant level of IgG4 in steroid-responsive polypoid tissue in patients suggested an autoimmune inflammatory process.

Inheritance

Sweetser et al. (2012) stated that 'Cronkhite-Canada syndrome...is a noninherited condition.'