Lupus-Erythematosus-Like Disease in Three Unrelated Women with Hereditary Angioneurotic Edema
Authors: VIRGINIA H. DONALDSON
Journal: Annals of Internal Medicine (1977)

Letters1 March 1977Lupus-Erythematosus-Like Disease in Three Unrelated Women with Hereditary Angioneurotic EdemaVIRGINIA H. DONALDSON, M.D., EVELYN V. HESS, M.D., F.A.C.P., A. JAMES MCADAMS, M.D.VIRGINIA H. DONALDSON, M.D.Search for more papers by this author, EVELYN V. HESS, M.D., F.A.C.P.Search for more papers by this author, A. JAMES MCADAMS, M.D.Search for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-86-3-312 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptInherited deficiencies of components of complement have been reported in association with connective tissue disorders, including lupus erythematosus. In hereditary angioneurotic edema serum the fourth (C4) and sometimes the second (C2) components of complement are readily depleted largely because of the activation of C1 that occurs in this hereditary deficiency of C1 inhibitor (1). Three patients with hereditary angioneurotic edema and lupus-erythematosus-like disease are described below.Patient 1. A clinical diagnosis of hereditary angioneurotic edema made at age 23 was supported by virtual absence of serum C1 inhibitor function and antigens. She developed a "butterfly rash" on her face and...References1. DONALDSONROSEN VF: Action of complement in hereditary angioneurotic edema: the role of C11-esterase. J Clin Invest 43:2204-2213, 1964 CrossrefMedlineGoogle Scholar2. ROSENFELDPARTRIDGEBARTHOLOMEW GRW: Hereditary angioneurotic edema (HANE) and systemic lupus erythematosus (SLE) in one of identical twin girls (abstract). J Allergy Clin Immunol 47:68-69, 1974 Google Scholar3. KOHLERPERCYCAMPION PJW: Hereditary angioedema and "familial" lupus erythematosus in identical twin boys. Am J Med 56:406-411, 1974 CrossrefMedlineGoogle Scholar4. SEIGELLEE MS: The epidemiology of systemic lupus erythematosus. Semin Arthritis Rheum 3:1-54, 1973 CrossrefMedlineGoogle Scholar5. ROWELL N: Lupus erythematosus, scleroderma, dermatomyositis, polyarteritis, in Textbook of Dermatology, vol. 1, edited by ROOK A, WILKINSON DS. Philadelphia, F. A. Davis Co., 1968, p. 516 Google Scholar6. CZOPNUSSENZWEIG JV: Studies of the mechanism of solubilization of immune precipitates by serum. J Exp Med 143:615-630, 1976 CrossrefMedlineGoogle Scholar This content is PDF only. To continue reading please click on the PDF icon. 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