We present a clinically atypical case of discoid lupus erythematosus in the mother of a boy with chronic granulomatous disease. In this disorder, the phagocytes are unable to produce superoxide anion to degrade incorporated microorganisms. In addition to a discoid lupus erythematosus-like skin disease, recurrent stomatitis aphthosa, hidradenitis suppurativa and Raynaud phenomenon are markedly associated with heterozygote carriers of chronic granulomatous disease. Based on this conspicuous association, diverse models concerning the pathogenesis of lupus erythematosus are discussed.