Chronic granulomatous disease as a risk factor for cutaneous lupus in childhood.
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.
Department of Dermatology, Centro Hospitalar do Porto, Oporto, Portugal. carvalhosandrine@gmail.com.
Sandrina
S
Carvalho
Susana
S
Machado
Rita
R
Sampaio
Margarida
M
Guedes
Júlia
J
Vasconcelos
Diogo
D
Semedo
Manuela
M
Selores
D000974:Antibodies, Antinuclear / Q000276:immunology; D000908:Antibodies, Fungal / Q000276:immunology; D002613:Cheilitis / Q000150:complications / Q000175:diagnosis / Q000276:immunology; D002648:Child; D005148:Facial Dermatoses / Q000150:complications / Q000175:diagnosis* / Q000276:immunology / Q000473:pathology; D005533:Foot Dermatoses / Q000150:complications / Q000175:diagnosis* / Q000276:immunology / Q000473:pathology; D006105:Granulomatous Disease, Chronic / Q000150:complications / Q000276:immunology*; D006801:Humans; D008178:Lupus Erythematosus, Cutaneous / Q000150:complications / Q000175:diagnosis* / Q000276:immunology / Q000473:pathology; D008297:Male; D019226:Oral Ulcer / Q000150:complications / Q000175:diagnosis / Q000276:immunology; D011928:Raynaud Disease / Q000150:complications / Q000175:diagnosis / Q000276:immunology; D012307:Risk Factors; D012441:Saccharomyces cerevisiae / Q000276:immunology
D000974:Antibodies, Antinuclear; D000908:Antibodies, Fungal; C538950:anticentromere antibody