A CASE OF COLE-ENGMAN SYNDROME

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  • NAKATA MITSUO
    Devision of Otolaryngology, Institute of Clinical Medicine, The University of Tsukuba
  • EGUCHI SANETOMI
    Devision of Otolaryngology, Institute of Clinical Medicine, The University of Tsukuba
  • YOSHIE NOBUO
    Devision of Otolaryngology, Institute of Clinical Medicine, The University of Tsukuba

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Other Title
  • Ｃｏｌｅ‐Ｅｎｇｍａｎ症候群
  • Cole Engman ショウコウグン

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Abstract

A case of Cole-Engman syndrome which is a rare and fatal genodermatosis, characterised by the triad of reticulated hyperpigmentation of the skin, nail dystrophy, and leukoplakia of the mucous membrane was reported.<br>The patient was 16-year-old who had shown reticulated skin pigmentation on the bilateral ear, the neck, and the anterior chest, and atrophy and deformation of the nails for 5 years. Last year, folds and ulcers of the tongue emerged associated with pancytopenia, splenomegaly and hyperhidrosis in the regions of palms and soles.<br>Unfortunately, there was no effective treatments for this disease.

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