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August 1982, Vol 118, No. 8 >
Article | August 1982

The Complement System and the Skin: Title and subTitle BreakAn Overview

Robert E. Jordon, MD
Arch Dermatol. 1982;118(8):539-541. doi:10.1001/archderm.1982.01650200007006
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At the turn of the century, Ehrlich1 concluded that lysis of bacteria by immune serum samples required the presence of two soluble factors—a specific recognition unit we now refer to as "antibody" and a nonspecific labile factor that was later to be called "complement" by Bordet. Thus, for several decades, complement was considered to be a single serum factor. Subsequently, complement has proved to be multiple factors that react in a cascade-like fashion. As of now, at least 19 known distinct proteins constitute this system. An explosive increase in our understanding of the various proteins involved, in their reactive sequences, and in the role complement plays in a variety of disease states has occurred during the past decade. Skin diseases have been no exception. Complement may be subdivided into the following three segments: (1) the classic pathway, (2) the alternative pathway, and (3) the terminal sequence (Figure). These segments

REFERENCES

1 +
Ehrlich P: Studies in Immunity , ed 2. New York, John Wiley & Sons Inc, 1910;, p 1.
2 +
Müller-Eberhard JH:  Complement . Annu Rev Biochem 1975;;44:697-724.
3 +
Gigli I:  The complement system: Mechanisms of action, biology, and participation in dermatologic diseases , in Safai B, Good RA (eds): Comprehensive Immunology: Immunodermatology . New York, Plenum Press Inc, 1981;, pp 65-100.
4 +
Mayer MM:  Membrane damage by complement . Johns Hopkins Med J 1981;;148:243-251.
5 +
Fearon DT, Austen KF:  Activation of the alternative complement pathway due to resistance of zymosan-bound amplification convertase to endogenous regulatory mechanisms . Proc Natl Acad Sci USA 1977;;74:1683-1687.
6 +
Schrieber RD, Medicus RG, Götze O, et al:  Properdin and nephritic factor-dependent C3 convertases: Requirement of native C3 for enzyme formation and the function of bound C3b as properdin receptor . J Exp Med 1975;;142:760-772.
7 +
Fearon DT, Austen KF:  Properdin binding to C3b and stabilization of C3b-dependent C3 convertase . J Exp Med 1975;;142:856-863.
8 +
Kolb WP, Müller-Eberhard HJ:  The membrane attack mechanism of complement: Isolation and subunit composition of the C5b-9 complex . J Exp Med 1975;;141:724-735.
9 +
Rommel FA, Mayer MM:  Studies of guinea pig complement component C9: Reaction kinetics and evidence that lysis of EAC1-8 results from a single membrane lesion caused by one molecule of C9 . J Immunol 1973;;110:637-647.
10 +
Ziccardi RJ, Cooper NR:  Modulation of the antigenicity of C1r and C1s by Cl inactivator . J Immunol 1978;;121:2148-2152.
11 +
Whaley K, Ruddy S:  Modulation of the alternative complement pathways by β1H-globulin . J Exp Med 1976;;144:1147-1163.
12 +
Weiler JM, Daha MR, Austen KF, et al:  Control of the amplification convertase of complement by the plasma protein β1H . Proc Natl Acad Sci USA 1976;;73:3268-3272.
13 +
Fujita T, Gigli I, Nussenzweig V:  Human C4-binding protein: II. Role in proteolysis of C4b by C3b inactivator . J Exp Med 1978;;148:1044-1051.
14 +
Conradie JD, Volanakis JE, Stround RM:  Evidence for a serum inhibitor of C1q . Immunochemistry 1975;;12:967-971.
15 +
Bokish VA, Müller-Eberhard HJ:  Anaphylatoxin inactivator of human plasma: Its isolation and characterization as a carboxypeptidase . J Clin Invest 1970;;49:2427-2436.
16 +
Podack ER, Kolb WP, Müller-Eberhard HJ:  The C5b-6 complex: Formation, isolation, and inhibition of its activity by lipoprotein and the S-protein of human serum . J Immunol 1978;;120:1841-1848.
17 +
Daniels CA, Borsos T, Rapp HJ, et al:  Neutralization of sensitized virus by purified components of complement . Proc Natl Acad Sci USA 1970;;65:528-535.
18 +
Wallis C, Melnick JL:  Herpesvirus neutralization: The role of complement . J Immunol 1971;;107:1235-1242.
19 +
Leddy JP, Simons RL, Douglas RG:  Effect of selective complement deficiency on the rate of neutralization of enveloped viruses by human sera . J Immunol 1977;;118:28-34.
20 +
Mills BJ, Cooper NR:  Antibody-independent neutralization of vesicular stomatitis virus by human complement: I. Complement requirements . J Immunol 1978;;121:1549-1557.
21 +
Beebe DP, Cooper NR:  Neutralization of vesicular stomatitis virus (VSV) by human complement requires a natural IgM antibody present in human serum . J Immunol 1981;;126:1562-1568.
22 +
Dais de Silva W, Eisele JW, Lepow IH:  Complement as a mediator of inflammation: III. Purification of activity with anaphylatoxin properties operated by interaction of the first four components of complement and its identification as a cleavage product of C3 . J Exp Med 1968;;126:1027-1048.
23 +
Gigli I, Nelson RA:  Complement-dependent immune phagocytosis: I. Requirements for Cl, C4, C2, C3 . Exp Cell Res 1968;;51:45-67.
24 +
Miller GW, Nussenzweig V:  Complement as regulator of interactions between immune complexes and cell membranes . J Immunol 1974;;113:464-469.
25 +
Snyderman R, Phillips JK, Mergenhagen SE:  Biological activity of complement in vivo: Role of C5 in the accumulation of polymorphonuclear leukocytes in inflammatory exudates . J Exp Med 1971;;134:1131-1143.
26 +
Goldstein IM, Weissmann G:  Generation of C5-derived lysosomal enzyme-releasing activity (C5a) by lysates of leukocyte lysosomes . J Immunol 1974;;113:1583-1588.
27 +
Miller ME, Nilsson UR:  A major role of the fifth component of complement (C5) in the Opsonization of yeast particles: Partial dichotomy of function and immunochemical measurement . Clin Immunol Immunopathol 1974;;2:246-255.
28 +
Ward PA:  Complement-derived leukotactic factors in pathological fluids . J Exp Med 1971;;134( (suppl) ):109-113.
29 +
Peterson BH, Lee TJ, Snyderman R, et al:  Neisseria meningitidis and Neisseria gonorrhoeae bacteremia associated with C6, C7, or C8 deficiency . Ann Intern Med 1979;;90:917-920.
30 +
Agnello V:  Complement deficiency states . Medicine 1978;;57:1-23.
31 +
Maddison PJ, Provost TT, Reichlin M:  Serologic findings in patients with `ANA negative' systemic lupus erythematosus . Medicine 1981;;60:87-94.
32 +
Rosenfeld SI, Kelly ME, Leddy JP:  Hereditary deficiency of the fifth component of complement in man: I. Clinical, immunochemical, and family studies . J Clin Invest 1976;;57:1626-1634.
33 +
Jasin HE:  Absence of the eighth component of complement in association with systemic lupus erythematosus-like disease . J Clin Invest 1977;;60:709-715.
34 +
Alper CA, Colten HR, Rosen FS, et al:  Homozygous deficiency of C3 in a patient with repeated infections . Lancet 1972;;2:1179-1181.
35 +
Ballow M, Shira JE, Harden L, et al:  Complete absence of the third component of complement in man . J Clin Invest 1975;;56:703-710.
36 +
Hsieh KH, Lin CY, Lee TC:  Complete absence of the third component of complement in a patient with repeated infections . Clin Immunol Immunopathol 1981;;20:305-312.
37 +
McLean RH, Weinstein A, Chaptitis J, et al:  Familial partial deficiency of the third component of complement (C3) and the hypocomplementemic cutaneous vasculitis syndrome . Am J Med 1980;;68:549-558.
38 +
Haeney MR, Thompson RA, Faulkner J, et al:  Recurrent bacterial meningitis in patients with genetic defects of terminal complement components . Clin Exp Immunol 1980;;40:16-24.
39 +
Tappeiner G, Hinter H, Wolff K:  Reticulate urticarial erythema in hereditary angioedema . Br J Dermatol 1980;;102:621-622.
40 +
Williamson DM:  Reticulate erythema: A prodrome in hereditary angioedema . Br J Dermatol 1979;;101:549-552.
41 +
Jordon RE, McDuffie FC, Good RA, et al:  Diffuse normolipemic plane xanthomatosis: An abnormal complement component profile . Clin Exp Immunol 1974;;18:407-415.
42 +
Jones RR, Baughan ASJ, Cream JJ, et al:  Complement abnormalities in diffuse plane xanthomatosis with paraproteinemia . Br J Dermatol 1979;;101:711-716.
43 +
Bennett WM, Bardana EJ, Wuepper K, et al:  Partial lipodystrophy, C3 nephritic factor and clinically inapparent mesangiocapillary glomerulonephritis . Am J Med 1977;;62:757-760.
44 +
Miller ME, Nilsson UR:  A familial deficiency of the phagocytosisenhancing activity of serum related to a dysfunction of the fifth component of complement (C5) . N Engl J Med 1970;;282:354-358.
45 +
Haydey RP, deRohas MP, Gigli I:  A newly described control mechanism of complement activation in patients with essential mixed cryoglobulinemia (cryoglobulins and complement) . J Invest Dermatol 1980;;74:328-332.
46 +
Gigli I, Schothorst AA, Soter NA, et al:  Erythropoietic photoporphyria: Photoactivation of the complement system . J Clin Invest 1980;;66:517-522.

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1 +
Ehrlich P: Studies in Immunity , ed 2. New York, John Wiley & Sons Inc, 1910;, p 1.
2 +
Müller-Eberhard JH:  Complement . Annu Rev Biochem 1975;;44:697-724.
3 +
Gigli I:  The complement system: Mechanisms of action, biology, and participation in dermatologic diseases , in Safai B, Good RA (eds): Comprehensive Immunology: Immunodermatology . New York, Plenum Press Inc, 1981;, pp 65-100.
4 +
Mayer MM:  Membrane damage by complement . Johns Hopkins Med J 1981;;148:243-251.
5 +
Fearon DT, Austen KF:  Activation of the alternative complement pathway due to resistance of zymosan-bound amplification convertase to endogenous regulatory mechanisms . Proc Natl Acad Sci USA 1977;;74:1683-1687.
6 +
Schrieber RD, Medicus RG, Götze O, et al:  Properdin and nephritic factor-dependent C3 convertases: Requirement of native C3 for enzyme formation and the function of bound C3b as properdin receptor . J Exp Med 1975;;142:760-772.
7 +
Fearon DT, Austen KF:  Properdin binding to C3b and stabilization of C3b-dependent C3 convertase . J Exp Med 1975;;142:856-863.
8 +
Kolb WP, Müller-Eberhard HJ:  The membrane attack mechanism of complement: Isolation and subunit composition of the C5b-9 complex . J Exp Med 1975;;141:724-735.
9 +
Rommel FA, Mayer MM:  Studies of guinea pig complement component C9: Reaction kinetics and evidence that lysis of EAC1-8 results from a single membrane lesion caused by one molecule of C9 . J Immunol 1973;;110:637-647.
10 +
Ziccardi RJ, Cooper NR:  Modulation of the antigenicity of C1r and C1s by Cl inactivator . J Immunol 1978;;121:2148-2152.
11 +
Whaley K, Ruddy S:  Modulation of the alternative complement pathways by β1H-globulin . J Exp Med 1976;;144:1147-1163.
12 +
Weiler JM, Daha MR, Austen KF, et al:  Control of the amplification convertase of complement by the plasma protein β1H . Proc Natl Acad Sci USA 1976;;73:3268-3272.
13 +
Fujita T, Gigli I, Nussenzweig V:  Human C4-binding protein: II. Role in proteolysis of C4b by C3b inactivator . J Exp Med 1978;;148:1044-1051.
14 +
Conradie JD, Volanakis JE, Stround RM:  Evidence for a serum inhibitor of C1q . Immunochemistry 1975;;12:967-971.
15 +
Bokish VA, Müller-Eberhard HJ:  Anaphylatoxin inactivator of human plasma: Its isolation and characterization as a carboxypeptidase . J Clin Invest 1970;;49:2427-2436.
16 +
Podack ER, Kolb WP, Müller-Eberhard HJ:  The C5b-6 complex: Formation, isolation, and inhibition of its activity by lipoprotein and the S-protein of human serum . J Immunol 1978;;120:1841-1848.
17 +
Daniels CA, Borsos T, Rapp HJ, et al:  Neutralization of sensitized virus by purified components of complement . Proc Natl Acad Sci USA 1970;;65:528-535.
18 +
Wallis C, Melnick JL:  Herpesvirus neutralization: The role of complement . J Immunol 1971;;107:1235-1242.
19 +
Leddy JP, Simons RL, Douglas RG:  Effect of selective complement deficiency on the rate of neutralization of enveloped viruses by human sera . J Immunol 1977;;118:28-34.
20 +
Mills BJ, Cooper NR:  Antibody-independent neutralization of vesicular stomatitis virus by human complement: I. Complement requirements . J Immunol 1978;;121:1549-1557.
21 +
Beebe DP, Cooper NR:  Neutralization of vesicular stomatitis virus (VSV) by human complement requires a natural IgM antibody present in human serum . J Immunol 1981;;126:1562-1568.
22 +
Dais de Silva W, Eisele JW, Lepow IH:  Complement as a mediator of inflammation: III. Purification of activity with anaphylatoxin properties operated by interaction of the first four components of complement and its identification as a cleavage product of C3 . J Exp Med 1968;;126:1027-1048.
23 +
Gigli I, Nelson RA:  Complement-dependent immune phagocytosis: I. Requirements for Cl, C4, C2, C3 . Exp Cell Res 1968;;51:45-67.
24 +
Miller GW, Nussenzweig V:  Complement as regulator of interactions between immune complexes and cell membranes . J Immunol 1974;;113:464-469.
25 +
Snyderman R, Phillips JK, Mergenhagen SE:  Biological activity of complement in vivo: Role of C5 in the accumulation of polymorphonuclear leukocytes in inflammatory exudates . J Exp Med 1971;;134:1131-1143.
26 +
Goldstein IM, Weissmann G:  Generation of C5-derived lysosomal enzyme-releasing activity (C5a) by lysates of leukocyte lysosomes . J Immunol 1974;;113:1583-1588.
27 +
Miller ME, Nilsson UR:  A major role of the fifth component of complement (C5) in the Opsonization of yeast particles: Partial dichotomy of function and immunochemical measurement . Clin Immunol Immunopathol 1974;;2:246-255.
28 +
Ward PA:  Complement-derived leukotactic factors in pathological fluids . J Exp Med 1971;;134( (suppl) ):109-113.
29 +
Peterson BH, Lee TJ, Snyderman R, et al:  Neisseria meningitidis and Neisseria gonorrhoeae bacteremia associated with C6, C7, or C8 deficiency . Ann Intern Med 1979;;90:917-920.
30 +
Agnello V:  Complement deficiency states . Medicine 1978;;57:1-23.
31 +
Maddison PJ, Provost TT, Reichlin M:  Serologic findings in patients with `ANA negative' systemic lupus erythematosus . Medicine 1981;;60:87-94.
32 +
Rosenfeld SI, Kelly ME, Leddy JP:  Hereditary deficiency of the fifth component of complement in man: I. Clinical, immunochemical, and family studies . J Clin Invest 1976;;57:1626-1634.
33 +
Jasin HE:  Absence of the eighth component of complement in association with systemic lupus erythematosus-like disease . J Clin Invest 1977;;60:709-715.
34 +
Alper CA, Colten HR, Rosen FS, et al:  Homozygous deficiency of C3 in a patient with repeated infections . Lancet 1972;;2:1179-1181.
35 +
Ballow M, Shira JE, Harden L, et al:  Complete absence of the third component of complement in man . J Clin Invest 1975;;56:703-710.
36 +
Hsieh KH, Lin CY, Lee TC:  Complete absence of the third component of complement in a patient with repeated infections . Clin Immunol Immunopathol 1981;;20:305-312.
37 +
McLean RH, Weinstein A, Chaptitis J, et al:  Familial partial deficiency of the third component of complement (C3) and the hypocomplementemic cutaneous vasculitis syndrome . Am J Med 1980;;68:549-558.
38 +
Haeney MR, Thompson RA, Faulkner J, et al:  Recurrent bacterial meningitis in patients with genetic defects of terminal complement components . Clin Exp Immunol 1980;;40:16-24.
39 +
Tappeiner G, Hinter H, Wolff K:  Reticulate urticarial erythema in hereditary angioedema . Br J Dermatol 1980;;102:621-622.
40 +
Williamson DM:  Reticulate erythema: A prodrome in hereditary angioedema . Br J Dermatol 1979;;101:549-552.
41 +
Jordon RE, McDuffie FC, Good RA, et al:  Diffuse normolipemic plane xanthomatosis: An abnormal complement component profile . Clin Exp Immunol 1974;;18:407-415.
42 +
Jones RR, Baughan ASJ, Cream JJ, et al:  Complement abnormalities in diffuse plane xanthomatosis with paraproteinemia . Br J Dermatol 1979;;101:711-716.
43 +
Bennett WM, Bardana EJ, Wuepper K, et al:  Partial lipodystrophy, C3 nephritic factor and clinically inapparent mesangiocapillary glomerulonephritis . Am J Med 1977;;62:757-760.
44 +
Miller ME, Nilsson UR:  A familial deficiency of the phagocytosisenhancing activity of serum related to a dysfunction of the fifth component of complement (C5) . N Engl J Med 1970;;282:354-358.
45 +
Haydey RP, deRohas MP, Gigli I:  A newly described control mechanism of complement activation in patients with essential mixed cryoglobulinemia (cryoglobulins and complement) . J Invest Dermatol 1980;;74:328-332.
46 +
Gigli I, Schothorst AA, Soter NA, et al:  Erythropoietic photoporphyria: Photoactivation of the complement system . J Clin Invest 1980;;66:517-522.

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