Chronic granulomatous disease as a risk factor for cutaneous lupus in childhood
- Author(s): Carvalho, Sandrina
- Machado, Susana
- Sampaio, Rita
- Guedes, Margarida
- Vasconcelos, Júlia
- Semedo, Diogo
- Selores, Manuela
- et al.
Published Web Locationhttps://doi.org/10.5070/D3233034298
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.