Carvalho Sandrina, Machado Susana, Sampaio Rita, Guedes Margarida, Vasconcelos Júlia, Semedo Diogo, Selores Manuela
Department of Dermatology, Centro Hospitalar do Porto, Oporto, Portugal.
Dermatol Online J. 2017 Mar 15;23(3):13030/qt2j6819c9.
Chronic granulomatous disease (CGD) is a primaryimmunodeficiency disorder that affects the phagocyticcells of the innate immune system. It is characterizedby recurrent or persistent infections with granulomaformation. Lupus-like lesions have been reported incarriers of CGD and less frequently, in patients withCGD. Immunological study in these patients areusually negative. We describe the case of an 8-yearoldboy with CGD who developed chronic and acutecutaneous lupus erythematous with angular cheilitis,oral ulcers, Raynaud phenomenon, and positiveserologies for antinuclear, anticentromere, and anti-Saccharomyces cerevisiae antibodies.
慢性肉芽肿病（CGD）是一种原发性免疫缺陷疾病，影响先天性免疫系统的吞噬细胞。其特征为反复或持续感染并形成肉芽肿。CGD携带者中曾有狼疮样病变的报道，CGD患者中则较少见。这些患者的免疫学检查通常为阴性。我们描述了一名8岁患CGD的男孩的病例，他出现了慢性和急性皮肤红斑狼疮，伴有口角炎、口腔溃疡、雷诺现象，且抗核抗体、抗着丝点抗体和抗酿酒酵母抗体的血清学检查呈阳性。
