0
September 1983, Vol 119, No. 9 >
Article | September 1983

Mechanisms of Immunologic Injury: Title and subTitle BreakPemphigus and Bullous Pemphigoid

Grant J. Anhalt, MD; Harish Patel, MD; Luis A. Diaz, MD
[+] Author Affiliations

Department of Dermatology Johns Hopkins University 600 N Wolfe St Baltimore, MD 21205; Baltimore


Arch Dermatol. 1983;119(9):711-714. doi:10.1001/archderm.1983.01650330003004
Text Size: A A A
Published online
Article
References
Comments

Our understanding of bullous skin disorders has increased enormously since the beginning of the century, when a patient with generalized blisters was firmly diagnosed as having pemphigus vulgaris only on his demise. Those fortunate patients that survived obviously did not have true pemphigus vulgaris, but likely had "pemphigus of the aged" or "bullous dermatitis herpetiformis." The key observations that defined these diseases more precisely were the careful histopathologic studies of Lever and the immunofluorescence microscopy studies of Beutner and Jordon2 and Jordon et al.3 The autoantibodies in pemphigus vulgaris and bullous pemphigoid (BP) that can be detected both bound in and around lesions and circulating in the serum samples of patients have proved to be not only crucial for the accurate diagnosis of these conditions, but appear to be of primary importance in the pathogenesis of the diseases as well. There is now substantial evidence that pemphigus autoantibodies

REFERENCES

1 +
Lever WF: Pemphigus and Pemphigoid . Springfield, Ill, Charles C Thomas Publisher, 1965;, pp 15-72.
2 +
Beutner EH, Jordon RE:  Demonstration of skin autoantibodies in sera of pemphigus vulgaris patients by indirect immunofluorescence staining . Proc Soc Exp Biol Med 1964;;117:505-510.
3 +
Jordon RE, Beutner EH, Witebsky E, et al:  Basement zone antibodies in bullous pemphigoid JAMA 1967;;200:751-756.
4 +
Rucco V:  Pathogenicity of the intercellular antibodies of pemphigus and their periodic removal from the circulation by plasmapheresis . Br J Dermatol 1977;;98:237-241.
5 +
Auerbach R, Bystryn J:  Plasmapheresis and immunosuppressive therapy: Effect on levels of intercellular antibodies in pemphigus vulgaris . Arch Dermatol 1979;;115:728-730.
6 +
Swanson DL, Dahl MV:  Pemphigus vulgaris and plasma exchange: Clinical and serologic studies . J Am Acad Dermatol 1981;;4:325-328.
7 +
Roujeau JC, Revuz J, Guillaume JC, et al:  Possible immune origin of pemphigus . N Engl J Med 1982;;307:1213.
8 +
Moncada B, Kettelsen S, Hernandes-Moctezuma JL, et al:  Neonatal pemphigus vulgaris: Role of passively transferred pemphigus antibodies , abstracted. Clin Res 1980;;28:251A.
9 +
Storer JS, Galen WK, Nesbitt LT, et al:  Neonatal pemphigus vulgaris . J Am Acad Dermatol 1982;;6:929-932.
10 +
Bellone AG, Leone V:  Ricerche sull' influenza esercitata da sieri di soggetti sani o affetti da pemfigo su pelle umana normale e pemfigosa coltivata `in vitro.' G Ital Dermatol Sif 1956;;97:97-109.
11 +
Schlitz JR, Michel B:  Production of epidermal acantholysis in normal human skin in vitro by the IgG fraction from pemphigus serum . J Invest Dermatol 1976;;67:254-260.
12 +
Morioka S, Naito K, Ogawa H:  The pathogenic role of pemphigus antibodies and proteinase in epidermal acantholysis . J Invest Dermatol 1981;;76:337-341.
13 +
Farb RM, Dykes R, Lazarus GS:  Anti-epidermal cell-surface pemphigus antibody detaches viable epidermal cells from culture plates by activation of proteinase . Proc Natl Acad Sci USA 1978;;75:459-463.
14 +
Diaz LA, Marcelo CL:  Pemphigoid and pemphigoid antigens in cultured epidermal cells . Br J Dermatol 1978;;98:631-637.
15 +
Woo TY, Hogan V, Patel H, et al:  Specificity and inhibition of the cell detachment induced by pemphigus IgG in vitro . J Invest Dermatol , in press.
16 +
Sams WJ Jr, Jordon RE:  Pemphigus antibodies: Their role in disease . J Invest Dermatol 1971;;56:474-479.
17 +
Beutner EH, Chorzelski TP, Jarzabek M, et al:  Studies in immunodermatology: I. Passive induction of intraepidermal clefts in rabbits by transfer of sera from Brazilian pemphigus foliaceus patients . Int Arch Allergy 1972;;42:545-555.
18 +
Wood GW, Beutner EH, Chorzelski TP:  Studies in immunodermatology: II. Production of pemphigus-like lesions by intradermal injections of monkeys with Brazilian pemphigus foliaceus sera . Int Arch Allergy 1972;;42:556-564.
19 +
Holubar K, Corzelski TP, Gauto M, et al:  Studies in immunodermatology: III. Induction of intraepithelial lesions in monkeys by intramucosal injections of pemphigus antibodies . Int Arch Allergy 1973;;44:631-643.
20 +
Buschard K, Dabelsteen E, Bretlau P:  A model for the study of autoimmune diseases applied to pemphigus: Transplants of human oral mucosa to athymic nude mice binds pemphigus antibodies in vivo . J Invest Dermatol 1973;;76:171-173.
21 +
Anhalt GJ, Labib RS, Voorhees JJ, et al:  Induction of pemphigus in neonatal mice by passive transfer of IgG from patients with the disease . N Engl J Med 1982;;306:1189-1196.
22 +
Witebsky E, Rose NR, Terplan K, et al:  Chronic thyroiditis and autoimmunization . JAMA 1957;;164:1439-1447.
23 +
Rothman S:  The role of enzymes in dermatology: Clinical implications of skin enzyme systems . Arch Dermatol 1957;;76:277-281.
24 +
Hashimoto K, Singer KH, Safran KM, et al:  Incubation of human epidermal cells with pemphigus IgG results in an increase in plasminogen activator activity , abstracted. Clin Res 1982;;30:588A.
25 +
Schlitz JR, Michel B, Papay R:  Appearance of `pemphigus acantholytic factor' in human skin cultured with pemphigus antibody . J Invest Dermatol 1979;;73:575-581.
26 +
Woo TY, Barouski-Miller PA, Gelehrter TD, et al:  Molecular mechanisms of pemphigus antibody action , abstracted. Clin Res 1982;;30:719A.
27 +
Van Joost T, Cormane MH, Pondman RW:  Direct immunofluorescent study of the skin on the occurrence of complement in pemphigus . Br J Dermatol 1972;;87:466-474.
28 +
Jordon RE, Schroeter AL, Rogers RS III, et al:  Classical and alternate pathway activation of complement in pemphigus vulgaris lesions . J Invest Dermatol 1974;;63:256-259.
29 +
Jordon RE:  Complement activation in pemphigus and bullous pemphigoid . J Invest Dermatol 1976;;67:366-371.
30 +
Jordon RE, Sams WF Jr, Diaz G, et al:  Negative complement immunofluorescence in pemphigus . J Invest Dermatol 1971;;57:407-410.
31 +
Emmerson RW, Wilson-Jones E:  Eosinophilic spongiosis in pemphigus: A report of an unusual histological change in pemphigus . Arch Dermatol 1968;;97:252-257.
32 +
Orr W, Varani J, Ward PA:  Characteristic of the chemotactic response of neoplastic cells to a factor derived from the fifth component of complement . Am J Pathol 1978;;93:405-422.
33 +
Ward PA:  A plasmin-split fragment of C3 as a new chemotactic factor . J Exp Med 1967;;126:189-206.
34 +
Beutner ER, Chorzelski TP, Jordon RE: Autosensitization in Pemphigus and Bullous Pemphigoid . Springfield, Ill, Charles C Thomas Publisher, 1970;.
35 +
Provost TT, Tomasi TB Jr:  Evidence for activation of complement via the alternate pathway, in skin diseases: I. Herpes gestationis, systemic lupus erythematosus and bullous pemphigoid . J Clin Invest 1973;;52:1779-1787.
36 +
Provost TT, Tomasi TB Jr:  Immunopathology of bullous pemphigoid: Basement membrane deposition of IgE, alternate pathway components, and fibrin . Clin Exp Immunol 1974;;18:193-200.
37 +
Jordon RE, Schoerter AL, Good RE, et al:  The complement system in bullous pemphigoid: II. Immunofluorescent evidence for both classical and alternate-pathway activation . Clin Immunol Immunopathol 1975;;3:307-314.
38 +
Jordon RE, Norby-McFarland JM, Tappeiner G:  The complement system in bullous pemphigoid: V. In vitro fixation of properdin by pemphigus antibody . J Clin Lab Immunol 1978;;1:59-65.
39 +
Diaz-Perez JL, Jordon RE:  The complement system in bullous pemphigoid: IV. Chemotactic activity in blister fluid . Clin Immunol Immunopathol 1976;;5:360-370.
40 +
Baba T, Sonozaki H, Seki K, et al:  An eosinophil chemotactic factor present in blister fluids of bullous pemphigoid patients . J Immunol 1976;;116:112-116.
41 +
Wintroub BU, Mihm MC Jr, Goetze EJ, et al:  Morphologic and functional evidence for release of mast-cell products in bullous pemphigoid . N Engl J Med 1978;;298:417-421.
42 +
Dvorak AM, Mihm MC Jr, Osage JE, et al:  Bullous pemphigoid, an ultrastructural study of the inflammatory response: Eosinophil, basophil, and mast cell granule changes in multiple biopsies from one patient . J Invest Dermatol 1982;;78:98-101.
43 +
Gammon WR, Lewis DM, Carlo JR, et al:  Pemphigoid antibody mediated attachment of peripheral blood leukocytes at the dermal-epidermal junction of human skin . J Invest Dermatol 1980;;75:334-339.
44 +
Naito KN, Morioka S, Ogawa H:  The pathogenic mechanisms of blister formation in bullous pemphigoid . J Invest Dermatol 1982;;79:303-306.
45 +
Anhalt GJ, Bahn CF, Labib RS, et al:  Pathogenic effects of bullous pemphigoid autoantibodies on rabbit corneal epithelium . J Clin Invest 1981;;68:1097-1101.
46 +
Lever WF, Schaumburg-Lever G: Histopathology of the Skin , ed 5. Philadelphia, JB Lippincott Co, 1975;.
47 +
Jordon RE, Day NK, Sams WM Jr, et al:  The complement system in bullous pemphigoid: I. Complement and component levels in sera and blister fluids . J Clin Invest 1973;;52:1207-1217.
48 +
Lohrisch I, Herkmann K, Haustein U-F:  Detection of basement membrane zone antigens and C3 fragments in the blister fluid of bullous pemphigoid . Br J Dermatol 1980;;102:135-143.
49 +
Standberg K, Hagermark O:  Prostaglandin E2 in blister fluid of bullous diseases and experimental suction blisters . Acta Derm Venereol 1977;;57:487-492.
50 +
Jordon RE, Struve MF, Bushkell LL:  Serum and blister fluid immune complexes in bullous pemphigoid: Detection with C1q and monoclonal rheumatoid factor . Clin Exp Immunol 1981;;45:29-36.
51 +
Mogavero HS Jr, Meier HS, Newball HH, et al:  Enzymatic activity in bullous pemphigoid blister fluids , abstracted. Clin Res 1982;;30:598A.
52 +
Peterson LL, Wuepper KD:  Isolation and purification of the pemphigus vulgaris antigen from human epidermis , abstracted. Clin Res 1983;;31:596A.

First Page Preview

First page PDF preview

Figures

Tables

Interactive Graphics

Video

Country-Specific Mortality and Growth Failure in Infancy and Yound Children and Association With Material Stature

Use interactive graphics and maps to view and sort country-specific infant and early dhildhood mortality and growth failure data and their association with maternal

1 +
Lever WF: Pemphigus and Pemphigoid . Springfield, Ill, Charles C Thomas Publisher, 1965;, pp 15-72.
2 +
Beutner EH, Jordon RE:  Demonstration of skin autoantibodies in sera of pemphigus vulgaris patients by indirect immunofluorescence staining . Proc Soc Exp Biol Med 1964;;117:505-510.
3 +
Jordon RE, Beutner EH, Witebsky E, et al:  Basement zone antibodies in bullous pemphigoid JAMA 1967;;200:751-756.
4 +
Rucco V:  Pathogenicity of the intercellular antibodies of pemphigus and their periodic removal from the circulation by plasmapheresis . Br J Dermatol 1977;;98:237-241.
5 +
Auerbach R, Bystryn J:  Plasmapheresis and immunosuppressive therapy: Effect on levels of intercellular antibodies in pemphigus vulgaris . Arch Dermatol 1979;;115:728-730.
6 +
Swanson DL, Dahl MV:  Pemphigus vulgaris and plasma exchange: Clinical and serologic studies . J Am Acad Dermatol 1981;;4:325-328.
7 +
Roujeau JC, Revuz J, Guillaume JC, et al:  Possible immune origin of pemphigus . N Engl J Med 1982;;307:1213.
8 +
Moncada B, Kettelsen S, Hernandes-Moctezuma JL, et al:  Neonatal pemphigus vulgaris: Role of passively transferred pemphigus antibodies , abstracted. Clin Res 1980;;28:251A.
9 +
Storer JS, Galen WK, Nesbitt LT, et al:  Neonatal pemphigus vulgaris . J Am Acad Dermatol 1982;;6:929-932.
10 +
Bellone AG, Leone V:  Ricerche sull' influenza esercitata da sieri di soggetti sani o affetti da pemfigo su pelle umana normale e pemfigosa coltivata `in vitro.' G Ital Dermatol Sif 1956;;97:97-109.
11 +
Schlitz JR, Michel B:  Production of epidermal acantholysis in normal human skin in vitro by the IgG fraction from pemphigus serum . J Invest Dermatol 1976;;67:254-260.
12 +
Morioka S, Naito K, Ogawa H:  The pathogenic role of pemphigus antibodies and proteinase in epidermal acantholysis . J Invest Dermatol 1981;;76:337-341.
13 +
Farb RM, Dykes R, Lazarus GS:  Anti-epidermal cell-surface pemphigus antibody detaches viable epidermal cells from culture plates by activation of proteinase . Proc Natl Acad Sci USA 1978;;75:459-463.
14 +
Diaz LA, Marcelo CL:  Pemphigoid and pemphigoid antigens in cultured epidermal cells . Br J Dermatol 1978;;98:631-637.
15 +
Woo TY, Hogan V, Patel H, et al:  Specificity and inhibition of the cell detachment induced by pemphigus IgG in vitro . J Invest Dermatol , in press.
16 +
Sams WJ Jr, Jordon RE:  Pemphigus antibodies: Their role in disease . J Invest Dermatol 1971;;56:474-479.
17 +
Beutner EH, Chorzelski TP, Jarzabek M, et al:  Studies in immunodermatology: I. Passive induction of intraepidermal clefts in rabbits by transfer of sera from Brazilian pemphigus foliaceus patients . Int Arch Allergy 1972;;42:545-555.
18 +
Wood GW, Beutner EH, Chorzelski TP:  Studies in immunodermatology: II. Production of pemphigus-like lesions by intradermal injections of monkeys with Brazilian pemphigus foliaceus sera . Int Arch Allergy 1972;;42:556-564.
19 +
Holubar K, Corzelski TP, Gauto M, et al:  Studies in immunodermatology: III. Induction of intraepithelial lesions in monkeys by intramucosal injections of pemphigus antibodies . Int Arch Allergy 1973;;44:631-643.
20 +
Buschard K, Dabelsteen E, Bretlau P:  A model for the study of autoimmune diseases applied to pemphigus: Transplants of human oral mucosa to athymic nude mice binds pemphigus antibodies in vivo . J Invest Dermatol 1973;;76:171-173.
21 +
Anhalt GJ, Labib RS, Voorhees JJ, et al:  Induction of pemphigus in neonatal mice by passive transfer of IgG from patients with the disease . N Engl J Med 1982;;306:1189-1196.
22 +
Witebsky E, Rose NR, Terplan K, et al:  Chronic thyroiditis and autoimmunization . JAMA 1957;;164:1439-1447.
23 +
Rothman S:  The role of enzymes in dermatology: Clinical implications of skin enzyme systems . Arch Dermatol 1957;;76:277-281.
24 +
Hashimoto K, Singer KH, Safran KM, et al:  Incubation of human epidermal cells with pemphigus IgG results in an increase in plasminogen activator activity , abstracted. Clin Res 1982;;30:588A.
25 +
Schlitz JR, Michel B, Papay R:  Appearance of `pemphigus acantholytic factor' in human skin cultured with pemphigus antibody . J Invest Dermatol 1979;;73:575-581.
26 +
Woo TY, Barouski-Miller PA, Gelehrter TD, et al:  Molecular mechanisms of pemphigus antibody action , abstracted. Clin Res 1982;;30:719A.
27 +
Van Joost T, Cormane MH, Pondman RW:  Direct immunofluorescent study of the skin on the occurrence of complement in pemphigus . Br J Dermatol 1972;;87:466-474.
28 +
Jordon RE, Schroeter AL, Rogers RS III, et al:  Classical and alternate pathway activation of complement in pemphigus vulgaris lesions . J Invest Dermatol 1974;;63:256-259.
29 +
Jordon RE:  Complement activation in pemphigus and bullous pemphigoid . J Invest Dermatol 1976;;67:366-371.
30 +
Jordon RE, Sams WF Jr, Diaz G, et al:  Negative complement immunofluorescence in pemphigus . J Invest Dermatol 1971;;57:407-410.
31 +
Emmerson RW, Wilson-Jones E:  Eosinophilic spongiosis in pemphigus: A report of an unusual histological change in pemphigus . Arch Dermatol 1968;;97:252-257.
32 +
Orr W, Varani J, Ward PA:  Characteristic of the chemotactic response of neoplastic cells to a factor derived from the fifth component of complement . Am J Pathol 1978;;93:405-422.
33 +
Ward PA:  A plasmin-split fragment of C3 as a new chemotactic factor . J Exp Med 1967;;126:189-206.
34 +
Beutner ER, Chorzelski TP, Jordon RE: Autosensitization in Pemphigus and Bullous Pemphigoid . Springfield, Ill, Charles C Thomas Publisher, 1970;.
35 +
Provost TT, Tomasi TB Jr:  Evidence for activation of complement via the alternate pathway, in skin diseases: I. Herpes gestationis, systemic lupus erythematosus and bullous pemphigoid . J Clin Invest 1973;;52:1779-1787.
36 +
Provost TT, Tomasi TB Jr:  Immunopathology of bullous pemphigoid: Basement membrane deposition of IgE, alternate pathway components, and fibrin . Clin Exp Immunol 1974;;18:193-200.
37 +
Jordon RE, Schoerter AL, Good RE, et al:  The complement system in bullous pemphigoid: II. Immunofluorescent evidence for both classical and alternate-pathway activation . Clin Immunol Immunopathol 1975;;3:307-314.
38 +
Jordon RE, Norby-McFarland JM, Tappeiner G:  The complement system in bullous pemphigoid: V. In vitro fixation of properdin by pemphigus antibody . J Clin Lab Immunol 1978;;1:59-65.
39 +
Diaz-Perez JL, Jordon RE:  The complement system in bullous pemphigoid: IV. Chemotactic activity in blister fluid . Clin Immunol Immunopathol 1976;;5:360-370.
40 +
Baba T, Sonozaki H, Seki K, et al:  An eosinophil chemotactic factor present in blister fluids of bullous pemphigoid patients . J Immunol 1976;;116:112-116.
41 +
Wintroub BU, Mihm MC Jr, Goetze EJ, et al:  Morphologic and functional evidence for release of mast-cell products in bullous pemphigoid . N Engl J Med 1978;;298:417-421.
42 +
Dvorak AM, Mihm MC Jr, Osage JE, et al:  Bullous pemphigoid, an ultrastructural study of the inflammatory response: Eosinophil, basophil, and mast cell granule changes in multiple biopsies from one patient . J Invest Dermatol 1982;;78:98-101.
43 +
Gammon WR, Lewis DM, Carlo JR, et al:  Pemphigoid antibody mediated attachment of peripheral blood leukocytes at the dermal-epidermal junction of human skin . J Invest Dermatol 1980;;75:334-339.
44 +
Naito KN, Morioka S, Ogawa H:  The pathogenic mechanisms of blister formation in bullous pemphigoid . J Invest Dermatol 1982;;79:303-306.
45 +
Anhalt GJ, Bahn CF, Labib RS, et al:  Pathogenic effects of bullous pemphigoid autoantibodies on rabbit corneal epithelium . J Clin Invest 1981;;68:1097-1101.
46 +
Lever WF, Schaumburg-Lever G: Histopathology of the Skin , ed 5. Philadelphia, JB Lippincott Co, 1975;.
47 +
Jordon RE, Day NK, Sams WM Jr, et al:  The complement system in bullous pemphigoid: I. Complement and component levels in sera and blister fluids . J Clin Invest 1973;;52:1207-1217.
48 +
Lohrisch I, Herkmann K, Haustein U-F:  Detection of basement membrane zone antigens and C3 fragments in the blister fluid of bullous pemphigoid . Br J Dermatol 1980;;102:135-143.
49 +
Standberg K, Hagermark O:  Prostaglandin E2 in blister fluid of bullous diseases and experimental suction blisters . Acta Derm Venereol 1977;;57:487-492.
50 +
Jordon RE, Struve MF, Bushkell LL:  Serum and blister fluid immune complexes in bullous pemphigoid: Detection with C1q and monoclonal rheumatoid factor . Clin Exp Immunol 1981;;45:29-36.
51 +
Mogavero HS Jr, Meier HS, Newball HH, et al:  Enzymatic activity in bullous pemphigoid blister fluids , abstracted. Clin Res 1982;;30:598A.
52 +
Peterson LL, Wuepper KD:  Isolation and purification of the pemphigus vulgaris antigen from human epidermis , abstracted. Clin Res 1983;;31:596A.

Correspondence

Submit a Letter
CME Course for:


You need to register in order to view this quiz.


To understand the clinical management of acute heart failure syndromes.
Accreditation Information The American Medical Association is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians.
The AMA designates this journal-based CME activity for a maximum of 1 AMA PRA Category 1 CreditTM per course. Physicians should claim only the credit commensurate with the extent of their participation in the activity.
Physicians who complete the CME course and score at least 80% correct on the quiz are eligible for AMA PRA Category 1 CreditTM.
Note: You must get at least of the answers correct to pass this quiz.
Note: You must get at least of the answers correct to pass this quiz.
You have not filled in all the answers to complete this quiz
The following questions were not answered:
Sorry, you have unsuccessfully completed this CME quiz with a score of
The following questions were not answered correctly:
For CME Course: A Proposed Model for Initial Assessment and Management of Acute Heart Failure Syndromes
Indicate what changes(s) you will implement in your practice, if any, based on this CME course.
To view and print your certificate and access a summary of your CME courses go to My CME.
NOTE:
Citing articles are presented as examples only. In non-demo SCM6 implementation, integration with CrossRef’s “Cited By” API will populate this tab (http://www.crossref.org/citedby.html).
Compression-Only CPR: Pushing the Science Forward
Cone
AAM 2010;304:1493-1495.
All you need to read in the other general journals
BMJ 2010;341:c5893-c1494.
Submit a Comment

Some tools below are only available to our subscribers or users with an online account.

Related Content

Customize your page view by dragging & repositioning the boxes below.

© 2012 American Medical Association. All Rights Reserved.
Powered bySilverchair Information Systems