PubMed1990
We encountered 4 patients (3 women, 1 man) with cutaneous vasculitis: three have a delayed pressure urticaria, two a vascular purpura with which a mixt cryoglobulinemia. Histology show a leukocytoclastic vasculitis. Initially all studies for lupus erythematosus were negative. However, after 3 to 10 years of follow-up, the 4 patients developed clinical, serological and histological features of systemic lupus erythematosus meeting four or more criteria of the American Rheumatism Association for the diagnosis of SLE. In one case there is a moderate renal disease. In three others cases there are a severe visceral injury: one with aseptic valvula's injury treated by surgery, another who died from a septicemic incident. The last who died from a neurological complication of systemic lupus. During the isolated cutaneous vasculitis phasis antinuclear antibodies and antibodies to double stranded DNA were all negative. At the time of SLE's diagnosis anti-DNA antibodies were present with or without ANA. In all four cases hypocomplementaemia was not initially seen which distinguish these cases from others previously reported in the literature. The syndrome recognized in these patients may constitute an "ante" serological and clinical phasis of SLE.