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��ժҪ�� DOCK8ȱ���ۺ��������ڱ�����ʷ��ѵ���8�Ļ���ͻ�����£����ۺ����ж��������Ե�Ƥ�����֣��������ص�Ƥ��������Ⱦ����Ӧ��Ƥ����Ƥ�����Ƥ��������ϵͳ�����������Ⱦ���緢Ƥ��������������������ϵͳ�ı��֡�ʵ���Ҽ�����Ϊ����ѪT�ܰ�ϸ�����٣���������ϸ�����ࡣ����Job�ۺ�����Wiskott-Aldrich�ۺ���������ϡ�ͨ��ϵͳ����Ƥ����Ⱦ������Ƥ����飬���ڷ���Ƥ���������Ը��ƻ��������������ӳ����������ڡ�

Liu Jiawei, Ma Donglai. Department of Dermatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100730, China Corresponding author: Ma Donglai, Email: mdonglai@sohu.com ��Abstract�� DOCK8 deficiency syndrome, a recently defined autosomal recessive genodermatosis caused by mutations in the dedicator of cytokinesis 8 ��DOCK8�� gene, has many characteristic cutaneous presentations such as severe viral infection, atopic dermatitis-like rashes, and recurrent cutaneous and respiratory staphylococcal infection. It is also associated with early-cutaneous malignancy and multiple organ abnormalities. Laboratory examination usually shows peripheral T lymphopenia and eosinophilia. Differential diagnosis should include Job′s syndrome and Wiskott-Aldrich syndrome. Systematic management of skin infection, regular skin examination, and timely detection of skin cancer may benefit the improvement of life quality and extend life span of patients.