Cronkhite-canada syndrome Q87.82

Very rare, non-familial, juvenile polyposis syndrome (see below hereditary polyposis syndromes), which often occurs in combination with gastrointestinal carcinomas, skin changes, eye malformations, spasticity and severe developmental delay.

After the age of 50.

Generalized intestinal polyposis: malabsorption, therapy-resistant diarrhea with electrolyte and protein losses. Universal, non-scarring alopecia of all terminal hairs, onychodystrophy and skin pigmentation in the form of pinhead-sized, roundish, brownish lentigines on the palms of the hands and soles of the feet. Also hypopigmentations. Mental retardation with cramps, spasticity, scoliosis, microphthalmia, secondary ectropion, amaurosis, cryptorchidism, growth retardation.

Unfavourable: lethal outcome in 50% of cases, on average 18 months after the onset of refractory diarrhoea. Risk of colorectal cancer is not increased.

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