Long-lasting remission in a case of Cronkhite-Canada syndrome

Cronkhite-Canada syndrome is a rare disease characterised by diffuse gastrointestinal polyposis, diarrhoea, weight loss, skin hyperpigmentation, onychodystrophy and alopecia. More than 500 cases have been described in the medical literature so far. The disease is probably caused by immune-mediated inflammatory mechanisms, and the diagnosis is based on clinical history, physical examination, endoscopic findings and histology. Differentiating this condition from other clinical entities presenting with similar signs and symptoms may be challenging. We present here the case of a 70-year-old Sardinian man where apparently the syndrome was induced by a mental strain triggered by a work-related accident. Continuous treatment with low-dose of antidepressant, anti-inflammatory and immunosuppressive medications in addition to nutritional support was remarkably effective and resulted in sustained (longer than 8 years) disappearance of clinical manifestations as well as the persistence of physical and psychological well-being. This case argues against the poor prognosis previously ascribed to patients with this syndrome.