Síndrome de Cronkhite-Canada: una poliposis adquirida y potencialmente reversible

Cronkhite-Canada syndrome: a new case report of this enigmatic and infrequent disease

“…At microscopy show lamina propria edema and cystic glandular enlargement with accumulation of mucinous material, polymorphonuclear aggregates and lymphoplasmacytic and eosinophilic inflammatory infiltrates (1,2,5-10). In some cases it has been described the existence of an outstanding mast cells infiltrate (1,2,6). Skin pigmentation shaped like brown macular lesions because of the accumulation of melanin in basal layer, use to be demonstrated in limbs, palms, plants, face and neck (1)(2)(3)(4)(5)(6)11).…”

“…In some cases it has been described the existence of an outstanding mast cells infiltrate (1,2,6). Skin pigmentation shaped like brown macular lesions because of the accumulation of melanin in basal layer, use to be demonstrated in limbs, palms, plants, face and neck (1)(2)(3)(4)(5)(6)11). Nails show dystrophic changes and, in advanced cases, easily detached themselves from nail bed.…”

“…Nails show dystrophic changes and, in advanced cases, easily detached themselves from nail bed. CCS is included in the differential diagnosis of the gastrointestinal polyposis, non adenomatous and non inherited, as lym- phoid nodular hyperplasia and hyperplasic, inflammatory, lipomatous or lymphomatous polyposis (1)(2)(3)8,(12)(13)(14)(15)(16). It is a very infrequent disease, at least in occidental countries, leading most of published cases to Asiatic countries.…”

“…It is a very infrequent disease, at least in occidental countries, leading most of published cases to Asiatic countries. In Spain we have just two reference of reported cases (2,3), while in Japan there have been published reviews with over one hundred cases. Etiopathology of CCS is still unknown (2,4,14).…”

“…The Cronkhite-Canada syndrome (CCS) is an infrequent disease, non-inherited, characterized by a gastrointestinal non-adenomatous polyposis, chronic diarrhea, low blood proteins, malnutrition and skin and nail alterations (1)(2)(3)(4)(5)(6). Etiopathogeny is unknown, there is no effective treatment with a scientific base and, although cases with response to empiric treatments have been described, prognosis is usually poor.…”