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Dermatology Online Journal

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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.

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.

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