{"id":4619,"date":"2022-05-10T06:35:39","date_gmt":"2022-05-10T06:35:39","guid":{"rendered":"https:\/\/www.kauveryhospital.com\/ima-journal\/?p=4619"},"modified":"2025-04-11T04:47:18","modified_gmt":"2025-04-11T04:47:18","slug":"classic-triad-of-dyskeratosis-congenita-or-zinsser-cole-engman","status":"publish","type":"post","link":"https:\/\/www.kauveryhospital.com\/ima-journal\/ima-journal-may-2022\/classic-triad-of-dyskeratosis-congenita-or-zinsser-cole-engman\/","title":{"rendered":"Classic Triad of Dyskeratosis Congenita or Zinsser Cole Engman"},"content":{"rendered":"<p class=\"caps\">[vc_row][vc_column][vc_column_text]<\/p>\n<h2><strong>Syndrome<\/strong><\/h2>\n<ol>\n<li>Dystrophic nails<\/li>\n<li>Lacy reticular skin pigmentation<\/li>\n<li>Oral Leukoplakia<\/li>\n<\/ol>\n<p style=\"text-align: justify;\">Dyskeratosis congenita is one of the inherited bone marrow failure syndrome caused due to mutations of genes involved in the maintenance of telomere length.<\/p>\n<p style=\"text-align: justify;\">The other findings include short stature, microcephaly, hypogonadism, developmental delay, urethral stricture, idiopathic pulmonary fibrosis, idiopathic cirrhosis, and a strong predisposition to other cancers such as head and neck squamous cell carcinoma, Ca stomach\/Ca esophagus, Anorectal carcinoma, Myelodysplastic syndrome, and Acute Myeloid Leukemia<\/p>\n<p style=\"text-align: justify;\">Diagnosis is by clinical examination and bone marrow evaluation, telomere length analysis, and next generation sequencing to identify the genes involved in telomere length. The only curative treatment is Hematopoietic stem cell transplantation.<\/p>\n<p><img loading=\"lazy\" decoding=\"async\" class=\"alignnone wp-image-4620 size-full\" src=\"https:\/\/www.kauveryhospital.com\/ima-journal\/wp-content\/uploads\/2022\/05\/ima-22-2.jpg\" alt=\"\" width=\"536\" height=\"870\" srcset=\"https:\/\/www.kauveryhospital.com\/ima-journal\/wp-content\/uploads\/2022\/05\/ima-22-2.jpg 536w, https:\/\/www.kauveryhospital.com\/ima-journal\/wp-content\/uploads\/2022\/05\/ima-22-2-185x300.jpg 185w\" sizes=\"auto, (max-width: 536px) 100vw, 536px\" \/><\/p>\n<p><strong>Fig 1<\/strong>. Dystrophic nails<\/p>\n<p><img loading=\"lazy\" decoding=\"async\" class=\"alignnone wp-image-4621 size-full\" src=\"https:\/\/www.kauveryhospital.com\/ima-journal\/wp-content\/uploads\/2022\/05\/ima-22-3.jpg\" alt=\"\" width=\"732\" height=\"595\" srcset=\"https:\/\/www.kauveryhospital.com\/ima-journal\/wp-content\/uploads\/2022\/05\/ima-22-3.jpg 732w, https:\/\/www.kauveryhospital.com\/ima-journal\/wp-content\/uploads\/2022\/05\/ima-22-3-300x244.jpg 300w\" sizes=\"auto, (max-width: 732px) 100vw, 732px\" \/><\/p>\n<p><img loading=\"lazy\" decoding=\"async\" class=\"alignnone wp-image-4622 size-full\" src=\"https:\/\/www.kauveryhospital.com\/ima-journal\/wp-content\/uploads\/2022\/05\/ima-22-4.jpg\" alt=\"\" width=\"572\" height=\"760\" srcset=\"https:\/\/www.kauveryhospital.com\/ima-journal\/wp-content\/uploads\/2022\/05\/ima-22-4.jpg 572w, https:\/\/www.kauveryhospital.com\/ima-journal\/wp-content\/uploads\/2022\/05\/ima-22-4-226x300.jpg 226w\" sizes=\"auto, (max-width: 572px) 100vw, 572px\" \/><\/p>\n<p><strong>Fig 2<\/strong>. Lacy reticular skin pigmentation<\/p>\n<p><img loading=\"lazy\" decoding=\"async\" class=\"alignnone wp-image-4623 size-full\" src=\"https:\/\/www.kauveryhospital.com\/ima-journal\/wp-content\/uploads\/2022\/05\/ima-22-5.jpg\" alt=\"\" width=\"653\" height=\"869\" srcset=\"https:\/\/www.kauveryhospital.com\/ima-journal\/wp-content\/uploads\/2022\/05\/ima-22-5.jpg 653w, https:\/\/www.kauveryhospital.com\/ima-journal\/wp-content\/uploads\/2022\/05\/ima-22-5-225x300.jpg 225w\" sizes=\"auto, (max-width: 653px) 100vw, 653px\" \/><\/p>\n<p><strong>Fig 3<\/strong>. Oral Leukoplakia<\/p>\n<p>&nbsp;<\/p>\n<p>&nbsp;<\/p>\n<p><img loading=\"lazy\" decoding=\"async\" class=\"alignleft wp-image-4627\" src=\"https:\/\/www.kauveryhospital.com\/ima-journal\/wp-content\/uploads\/2022\/05\/Dr-Arshad-Raja.jpg\" alt=\"\" width=\"160\" height=\"187\" srcset=\"https:\/\/www.kauveryhospital.com\/ima-journal\/wp-content\/uploads\/2022\/05\/Dr-Arshad-Raja.jpg 600w, https:\/\/www.kauveryhospital.com\/ima-journal\/wp-content\/uploads\/2022\/05\/Dr-Arshad-Raja-256x300.jpg 256w\" sizes=\"auto, (max-width: 160px) 100vw, 160px\" \/><strong>Dr. Arshad Raja<\/strong><br \/>\n<em>Associate Consultant &#8211; Haematology and Haemato Oncology<\/em><br \/>\nKauvery Hospital Chennai[\/vc_column_text][\/vc_column][\/vc_row]<\/p>\n","protected":false},"excerpt":{"rendered":"<p>[vc_row][vc_column][vc_column_text] Syndrome Dystrophic nails Lacy reticular skin pigmentation Oral Leukoplakia Dyskeratosis congenita is one of the inherited bone marrow failure syndrome caused due to mutations of genes involved in the<\/p>\n","protected":false},"author":2,"featured_media":4630,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"footnotes":""},"categories":[41],"tags":[],"class_list":["post-4619","post","type-post","status-publish","format-standard","has-post-thumbnail","hentry","category-ima-journal-may-2022"],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v24.0 - https:\/\/yoast.com\/wordpress\/plugins\/seo\/ -->\n<title>Classic Triad of Dyskeratosis Congenita or Zinsser Cole Engman<\/title>\n<meta name=\"description\" content=\"Dyskeratosis congenita is one of the inherited bone marrow failure syndrome caused due to mutations of genes involved in the maintenance of telomere length.\" \/>\n<meta name=\"robots\" content=\"index, follow, max-snippet:-1, max-image-preview:large, max-video-preview:-1\" \/>\n<link rel=\"canonical\" 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