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Dan M. Lipsker, MD; Catherine Schreckenberg-Gilliot, MD; Béatrice Uring-Lambert, MD, PhD; Alice Meyer; Dominique Hartmann; Edouard M. Grosshans, MD; Georges Hauptmann, MD, PhD

Arch Dermatol. 2000;136:1508-1514.

Background  The gene deletion responsible for the type I human complement C2 deficiency was reported in 1992. The purpose of our study is to evaluate clinical and immunological characteristics of 11 patients with lupus erythematosus and type I C2 deficiency.

Observations  We observed 5 patients with a homozygous C2 deficiency and 6 with a heterozygous C2 deficiency. Eight patients had systemic lupus erythematosus, 2 had subacute cutaneous lupus erythematosus, and 1 had chronic lupus erythematosus. Photosensitivity was present in 73% of the patients, and 64% tested positive for anti-Ro (SSA) antibodies. Renal involvement that required immunosuppressive therapy was present in 54% of the patients. Ninety percent of the patients tested positive for antinuclear antibodies, and 54% tested positive for anti–double-stranded DNA antibodies. Phenotyping of the fourth component of the complement was performed in 82% of the patients and showed a C4A4B2 phenotype, which is suggestive for the type I C2 deficiency.

Conclusions  Most patients with lupus erythematosus associated with C2 type I deficiency are photosensitive, and this is probably related to the presence of anti-Ro (SSA) autoantibodies. The prognosis for those patients is not better than that for patients with lupus erythematosus in general.

From the Clinique Dermatologique (Drs Lipsker, Schreckenberg-Gilliot, and Grosshans) and Laboratoire d'Immuno-Hématologie des Hôpitaux (Drs Uring-Lambert and Hauptmann and Mss Meyer and Hartmann), Universitaires de Strasbourg, Strasbourg, France.

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