Recombinant Thrombomodulin Used to Successfully Treat Cronkhite-Canada Syndrome with Disseminated Intravascular Coagulation Due to Sepsis in a Compromised Patient

A patient with Cronkhite-Canada syndrome (CCS), complicated by severe sepsis and disseminated intravascular coagulation, was successfully treated using a combination of therapies, including recombinant human soluble thrombomodulin. CCS is a rare disease marked by gastrointestinal polyposis, chronic diarrhea, ectodermal dysplasia, skin hyperpigmentation, hair loss, and nail atrophy. Despite the demonstrated efficacy of corticosteroids and immunomodulatory agents, no standard treatment regimen existed, and the prognosis remained poor due to complications. This case highlighted the potential of recombinant thrombomodulin in managing severe CCS complications.