Primary immunodeficiency diseases (PID), encompass a heterogeneous group of diseases, with increased susceptibility to recurrent, severe infections. Invasive fungal infections raise a serious concern related to their morbidity and mortality. Herein, we describe various fungal infections among different PID patients. Twenty-eight PID patients diagnosed with fungal infections were included; fourteen patients with chronic granulomatous disease, two with Hyper Immunoglobulin E syndrome, one with LRBA deficiency and one with MHC class II defect, one with unclassified immune dysregulation, one with CD4 lymphopenia and one patient with Immune dysregulation Polyendocrinopathy Enteropathy X-linked syndrome. Aspergillus species were the most common isolated causative organisms in 78% of patients, Candida species were the causative organisms in 32%, Pneumocystis jirovecii caused infections in 7% followed by Malassezia furfur, Fusarium spp., Mucormycosis, and Penicillium chrysogenium 3.5% for each. The mortality rate among our patients was 10/28 (35.7%). PID patients are at high risk of developing fungal infections.

原发性免疫缺陷疾病（PID）包括一组异质性疾病，对反复发作的严重感染的敏感性增加。侵袭性真菌感染引起与其发病率和死亡率有关的严重问题。在本文中，我们描述了不同PID患者之间的各种真菌感染。包括28名诊断为真菌感染的PID患者; 14例慢性肉芽肿病患者，2例高免疫球蛋白E综合征，1例LRBA缺乏和1例MHC II类缺陷，1例免疫功能失调，1例CD4淋巴细胞减少和1例免疫失调的多发性内分泌病性肠病X连锁综合征。在78％的患者中，曲霉菌是最常见的致病菌，念珠菌病原微生物占32％，吉罗肺孢子虫引起的感染占7％，其次为糠asse马拉色菌，镰刀菌，毛霉菌病和致青霉菌，分别为3.5％。我们患者的死亡率为10/28（35.7％）。PID患者极易发生真菌感染。