Zinsser-Cole-Engman症候群

・Zinsser-Cole-Engman症候群，別名の先天性角化不全症（dyskeratosis congenita：DKC）は合併症により若年で死亡する可能性があるため，定期的な血液検査および画像検査，悪性腫瘍の検索を継続していく必要がある

・皮膚科医の役割としては，とくに皮膚有棘細胞癌の発生に注意して経過観察することである．

・比較的生存期間の長いDKCの1例を経験した．

（「症例のポイント」より）

　

A case of Zinsser-Cole-Engman syndrome

　

Maeda, Tamaki1）Kobayashi, Tsukane1）Iwata, Yohei1）Yamakita, Takashi2）Okui, Tarou3）Sugiura, Kazumitsu1） 1）Department of Dermatology, Fujita University School of Medicine 2）Department of Dermatology, Kariya Toyota General Hospital 3）Department of Dental and Oral Surgery, Fujita University School of Medicine

　

A patient is 37-year-old man. White macula appeared on the back of the tongue 15 years ago from his first visit to the department of Dermatology and his taste became dull, so he visited to the department of Dental and Oral Surgery at his age of 32. He was diagnosed with mild heteromorphic leukoplakia by biopsy, and he took Etretinate. However, he was referred to the department of Dermatology for consultation to the leukoplakia because his symptoms improved little. The triad symptoms of Zinsser-Cole-Engman syndrome, also known as dyskeratosis congenita (DKC), reticular pigmentation, nail dystrophy, and mucosal leukoplakia, were identified in the patient. Thus the patient was diagnosed as Zinsser-Cole-Engman syndrome. At present, no complications have appeared in the patient, and the tongue lesions are also routinely biopsied at the department of Dental and Oral Surgery, but no malignant transformation has been observed. Since DKC may die at a young age due to complications such as bone marrow failure, malignant tumor, and interstitial pneumonia, we need to conduct periodic blood and imaging tests and search for malignant tumors in cooperation with other departments.