19. Cronkhite-Canada Syndrome
Definition
Cronkhite-Canada syndrome is a very rare, sporadically occurring, uninherited disorder. It is characterized by gastrointestinal polyps, cutaneous pigmentation, alopecia, and onychodystrophy.
Incidence
Cronkhite-Canada syndrome is an extremely rare disease. Since first described in 1955, 467 cases have been reported through the year 2002. The vast majority of cases have been documented in Japan, but cases have been reported worldwide. There is a very slight male to female difference in rate of incidence: 1.3:1 to 1.5:1.
The disease may manifest itself at virtually any age; however, fewer than 10 cases in infants have been reported since 1955. Cases have been reported in adults from 31 to 86 years of age.
Etiology
The etiology of Cronkhite-Canada syndrome is not known at present. Currently available evidence and data do not support a familial predisposition to developing the disease. Reports have linked incidences of the disease to arsenic poisoning.
Signs and Symptoms
• Cataracts
• Change in taste sensation
• Cheilosis papillary atrophy of the tongue
• Chronic or recurrent watery diarrhea, sometimes melena
• Difficulty swallowing
• Edema (mild and peripheral to massive anasarca)
• Episodic or constant abdominal pain (upper or lower)