A 44-year-old male patient with lupusery-thematosus profundus (lupus p
anniculitis) (LEP) of the right cheek/infraorbital region presented as
a recurrent swelling. He had only anti-neutrophil-cytoplasmic antibod
ies (ANCA),which have not been previously described in association wit
h LEP. There was no indication of systemic lupus erythematosus. While
chloroquine produced no benefits, the skin lesions promptly cleared wi
th dapsone. Step by step we reduced dapsone to a final dose of 50-75 m
g per week. The ANCA titer decreased in parallel to the clinical impro
vement.