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March 1977

Discoid Lupus Erythematosus and the Variant Form of Hereditary Angioedema

Arch Dermatol. 1977;113(3):374-375. doi:10.1001/archderm.1977.01640030120024

To the Editor.—  There are a number of reports in the literature of patients who, in association with connective tissue disease, have deficiencies of one or another component of the complement system. Recently Kohler et al1 reported identical male twins with longstanding discoid lupus erythematosus (LE) and hereditary angioedema with Cl inhibitor (C1 INH) deficiency and a marked secondary decrease in C4.Two genetic forms of hereditary angioedema have been described, the common and variant forms.2 About 85% of patients have the common form. Immunochemical and functional concentrations of C1 INH in these patients range from undetectable to about 50% of normal. The remaining 15% of patients have the variant form of the disease; that is, normal or increased levels of an antigenically identical, but functionally deficient protein.3 We recently have had the opportunity to study a patient with classical discoid LE and the variant form of