We describe a patient with a distinct verrucous variant of chronic discoid lupus erythematosus manifested by skin lesions resembling keratoacanthomas. The diagnosis of keratotic lupus was confirmed by characteristic immunofluorescence and ultrastructural findings and by an initial response to antimalarial therapy. Combination therapy with isotretinoin and hydroxychloroquine resulted in control of her previously refractory skin lesions, and the isotretinoin apparently played a key role in this improvement.