Alopecia in Cronkhite-Canada Syndrome: A Case Report
August 2017
in “
British Journal of Dermatology
”
alopecia areata Cronkhite-Canada syndrome gastrointestinal polyposis hyperpigmentation ectodermal abnormalities telogen hair follicles hair miniaturization anagen-telogen conversion autoimmune diseases IgG4 plasma cells immunosuppressants hair loss CCS polyps skin discoloration hair thinning immune system diseases
TLDR Hair loss in Cronkhite-Canada syndrome may be due to an autoimmune response.
The document discusses a case report on alopecia areata in Cronkhite-Canada syndrome (CCS), a rare disease characterized by gastrointestinal polyposis, hyperpigmentation, and ectodermal abnormalities including alopecia. The syndrome was first described in 1955, with most cases reported from Japan. A large series of 110 patients by Goto in 1995 categorized the disease into five types based on symptoms. The case report by Ong et al. provided histological evidence of a 'shift out of anagen' in CCS, with an increased number of telogen hair follicles and diffuse hair miniaturization, suggesting alopecia areata. This contrasts with previous findings of a diffuse anagen-telogen conversion. The autoimmune nature of CCS is supported by the frequent occurrence of other autoimmune diseases in patients with CCS, the presence of IgG4 plasma cells in polyps, and the response to immunosuppressants. The rarity of CCS necessitates further research, and case reports like Ong et al.'s contribute to understanding the syndrome's clinical and pathogenetic aspects.