Lupus erythematosus profundus: a cutaneous marker for a distinct clinical subset?

Sixteen patients with histologically confirmed lupus erythematosus profundus were followed for a decade (on average). At the initial examination, two of 16 (12%) patients fulfilled the 1982 American Rheumatism Association (ARA) criteria for systemic lupus erythematosus (SLE). During the period of observation, SLE developed in another two (12%) patients, both of whom developed a malar rash. The remaining 12 (75%) patients never met the criteria for SLE. Four of the 16 (25%) had no extracutaneous manifestation. In conclusion, most patients with lupus erythematosus profundus have a relatively mild disease course, although a few develop systemic abnormalities and have abnormal laboratory findings.