Clinical features of taste disorders in Cronkhite-Canada syndrome: a report of 10 cases

Cronkhite-Canada syndrome (CCS) is a very rare gastrointestinal disorder with ectodermal abnormalities. Taste abnormalities appear in more than 80% of cases. Our objective was to investigate the characteristics of CCS. Ten patients with taste abnormalities who were diagnosed with CCS were included. A medical interview, examination of the tongue findings, and blood tests were performed, and taste functions were assessed using an electrogustometry (EGM) and a filter paper disc (FPD) before and after treatment. There was nail atrophy in all cases, weight loss in 8 cases, hair loss in 6 cases, skin hyperpigmentation in 5 cases, gastrointestinal symptoms in 4 cases, and atrophy of the lingual papillae in at least 8 cases. Zinc therapy for taste disorders by the previous physicians was ineffective in all cases. The results of the FPD at the first examination showed a severe decrease in taste function of the anterior tongue, whereas taste function tended to be preserved in the posterior tongue (P < 0.01, Wilcoxon). In all cases, subjective symptoms improved within 3 mo after treatment of CCS. Taste function improved significantly after treatment (FPD in anterior tongue, P < 0.05, EGM in posterior tongue, P < 0.01, Wilcoxon). Taste disorder in CCS tended to be severe in the anterior tongue with findings of tongue papillary atrophy, which appears to be an ectodermal abnormality. Their taste function improved along with symptoms after treatment. The taste tests were useful for determining the effect of treatment for CCS.