Late-onset adenosine deaminase deficiency presenting with Heck's disease

被引:15
作者
Artac, Hasibe [1 ]
Gokturk, Bahar [1 ]
Bozdemir, Sefika Elmas [1 ]
Toy, Hatice [4 ]
van der Burg, Mirjam [2 ]
Santisteban, Ines [3 ]
Hershfield, Michael [3 ]
Reisli, Ismail [1 ]
机构
[1] Selcuk Univ, Meram Med Fac, Dept Pediat Immunol & Allergy, TR-42080 Beysehir Yolu, Konya, Turkey
[2] Univ Med Ctr, Dept Immunol, Erasmus MC, Rotterdam, Netherlands
[3] Duke Univ, Med Ctr, Dept Med & Biochem, Durham, NC 27710 USA
[4] Selcuk Univ, Meram Med Fac, Dept Pathol, TR-42080 Beysehir Yolu, Konya, Turkey
关键词
Late-onset adenosine deaminase deficiency; Focal epithelial hyperplasia; Human papilloma virus; FOCAL EPITHELIAL HYPERPLASIA; IMMUNODEFICIENCY; THERAPY; ADULTS; HPV;
D O I
10.1007/s00431-009-1131-9
中图分类号
R72 [儿科学];
学科分类号
100202 ;
摘要
Focal epithelial hyperplasia, also known as Heck's disease, is a rare but distinctive entity of viral etiology with characteristic clinical and histopathological features. It is a benign, asymptomatic disease of the oral mucosa caused by human papilloma viruses (HPV). Previous studies postulated an association between these lesions and immunodeficiency. Genetic deficiency of adenosine deaminase (ADA) results in varying degrees of immunodeficiency, including neonatal onset severe combined immunodeficiency (ADA-SCID), and milder, later onset immunodeficiency. We report a 12-year-old girl with the late onset-ADA deficiency presenting with Heck's disease. Our case report should draw attention to the possibility of immunodeficiency in patients with HPV-induced focal epithelial hyperplasia.
引用
收藏
页码:1033 / 1036
页数:4
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