Cronkhite-Canada Syndrome: A Rare Disease Presenting with Dermatological and Gastrointestinal Manifestations
June 2015
in “
Australasian Journal of Dermatology
”
TLDR A patient with Cronkhite-Canada syndrome improved with nutritional support and steroids, but early diagnosis is key due to high mortality.
The document reports a case of Cronkhite-Canada syndrome (CCS), a rare disease characterized by dermatological symptoms such as alopecia, hyperpigmentation, and onychodystrophy, along with gastrointestinal symptoms and hamartomatous polyps. A 66-year-old Caucasian woman presented with scalp pruritis, hair thinning, onycholysis, and gastrointestinal issues. A scalp biopsy indicated telogen effluvium, and she was treated with oral prednisolone, which led to hair regrowth. However, her gastrointestinal symptoms worsened, leading to hospitalization and a diagnosis of CCS after endoscopy. Treatment with multivitamins, vitamin D, and nutritional support resulted in recovery, with improvement in gastrointestinal symptoms, weight gain, hair regrowth, and nail formation. CCS has a high morbidity and mortality rate, with a 5-year mortality of up to 55%. The disease is thought to be immune-mediated, and diagnosis is based on clinical and pathological findings. Treatment typically involves nutritional support and immunosuppression, with corticosteroids showing effectiveness. Early recognition of CCS is crucial for reducing morbidity and mortality.