We present a clinically atypical case of discoid lupus erythematosus i
n the mother of a boy with chronic granulomatous disease. In this diso
rder, the phagocytes are unable to produce superoxide anion to degrade
incorporated microorganisms. In addition to a discoid lupus erythemat
osus-like skin disease, recurrent stomatitis aphthosa, hidradenitis su
ppurativa and Raynaud phenomenon are markedly associated with heterozy
gote carriers of chronic granulomatous disease. Based on this conspicu
ous association, diverse models concerning the pathogenesis of lupus e
rythematosus are discussed.