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Table 1. 
Summary of Randomized Controlled Trials in the Treatment of Ocular Cicatricial Pemphigoid
Summary of Randomized Controlled Trials in the Treatment of Ocular Cicatricial Pemphigoid
Table 2. 
Adverse Effects Observed in Ocular Cicatricial Pemphigoid During 1-Year Treatment and Follow-up*
Adverse Effects Observed in Ocular Cicatricial Pemphigoid During 1-Year Treatment and Follow-up*
1.
Wojnarowska  FEady  RAJBurge  SM Bullous eruptions. Champion  RHBurton  JLBurns  DABreathnach  SMeds.Textbook of Dermatology Oxford, England Blackwell Science Ltd1998;1873- 18771884- 1887
2.
Fine  J-D Cicatricial pemphigoid. Wojnarowska  FBriggaman  RAeds.Management of Blistering Diseases. London, England Chapman & Hall1990;83- 92
3.
Briggaman  RAGammon  WRWoodley  DT Epidermolysis bullosa acquisita. Wojnarowska  FBriggaman  RAeds.Management of Blistering Diseases. London, England Chapman & Hall1990;127- 138
4.
Chan  LSAhmed  ARAnhalt  GJ  et al.  The first international consensus on mucous membrane pemphigoid: definition, diagnostic criteria, pathogenic factors, medical treatment, and prognostic indicators. Arch Dermatol. 2002;138370- 379
5.
Bernard  PVaillant  LLabeille  B  et al. for the French Bullous Study Group, Incidence and distribution of subepidermal autoimmune bullous skin diseases in three French regions. Arch Dermatol. 1995;13148- 52Article
6.
Zillikens  DWever  SRoth  ABröcker  EB Incidence of autoimmune subepidermal blistering dermatoses in a region of central Germany. Arch Dermatol. 1995;131957- 958Article
7.
Reiche  LWojnarowska  FMallon  E Combination therapy with nicotinamide and tetracyclines for cicatricial pemphigoid: further support for its efficacy. Clin Exp Dermatol. 1998;23254- 257Article
8.
Dragan  LEng  AMLam  SPersson  T Tetracycline and niacinamide: treatment alternatives in ocular cicatricial pemphigoid. Cutis. 1999;63181- 183
9.
Foster  CSAhmed  AR Intravenous immunoglobulin therapy for ocular cicatricial pemphigoid: a preliminary study. Ophthalmology. 1999;1062136- 2143Article
10.
Harman  KEBlack  MM High-dose intravenous immune globulin for the treatment of autoimmune blistering diseases: an evaluation of its use in 14 cases. Br J Dermatol. 1999;140865- 874Article
11.
Foster  CS Cicatricial pemphigoid. Trans Am Ophthalmol Soc. 1986;84527- 663
12.
Tauber  JSainz de la Maza  MFoster  CS Systemic chemotherapy for ocular cicatricial pemphigoid. Cornea. 1991;10185- 195Article
13.
Neumann  RTauber  JFoster  CS Remission and recurrence after withdrawal of therapy for ocular cicatricial pemphigoid. Ophthalmology. 1991;98858- 862Article
14.
Foster  CSNeumann  RTauber  J Long-term results of systemic chemotherapy for ocular cicatricial pemphigoid. Doc Ophthalmol. 1992;82223- 229Article
15.
Mondino  BJBrown  SI Immunosuppressive therapy in ocular cicatricial pemphigoid. Am J Ophthalmol. 1983;96453- 459
16.
Mondino  BJ Cicatricial pemphigoid and erythema multiforme. Ophthalmology. 1990;97939- 952Article
17.
Rogers III  RSSeehafer  JRPerry  HO Treatment of cicatricial (benign mucous membrane) pemphigoid with dapsone. J Am Acad Dermatol. 1982;6215- 223Article
18.
Rogers III  RS Dapsone and sulfapyridine therapy of pemphigoid diseases. Australas J Dermatol. 1986;2758- 63Article
19.
Rogers III  RSMehregan  DA Dapsone therapy of cicatricial pemphigoid. Semin Dermatol. 1988;7201- 205
20.
Sackett  DL Rules of evidence and clinical recommendations on the use of antithrombotic agents. Chest. 1989;95 (suppl 2) 2S- 4SArticle
21.
Hanson  RDOlsen  KDRogers III  RS Upper aerodigestive tract manifestations of cicatricial pemphigoid. Ann Otol Rhinol Laryngol. 1988;97493- 499
22.
McFadden  JPLeonard  JNPowles  AVRutman  AJFry  L Sulphamethoxypyridazine for dermatitis herpetiformis, linear IgA disease and cicatricial pemphigoid. Br J Dermatol. 1989;121759- 762Article
23.
Matthews  RWPinkney  RCScully  C The management of intransigent desquamative gingivitis with dapsone. Ann Dent. 1989;4841- 43
24.
Lamey  PJRees  TDBinnie  WHRankin  KV Mucous membrane pemphigoid: treatment experience at two institutions. Oral Surg Oral Med Oral Pathol. 1992;7450- 53Article
25.
Axt  MWever  SBaier  G  et al.  Cicatricial pemphigoid—a therapeutic problem. Hautarzt. 1995;46620- 627Article
26.
Vincent  SDLilly  GEBaker  KA Clinical, historic, and therapeutic features of cicatricial pemphigoid: a literature review and open therapeutic trial with corticosteroids. Oral Surg Oral Med Oral Pathol. 1993;76453- 459Article
27.
Carrozzo  MCarbone  MBroccoletti  RGarzino-Demo  PGandolfo  S Therapeutic management of mucous membrane pemphigoid: report of 11 cases. Minerva Stomatol. 1997;46553- 559
28.
Carbone  MCarrozzo  MCastellano  S  et al.  Systemic corticosteroid therapy of oral vesiculoerosive diseases (OVED): an open trial. Minerva Stomatol. 1998;47479- 487
29.
Nayar  MWojnarowska  F Cicatricial pemphigoid: a re-evaluation of therapy. J Dermatol Treat. 1993;489- 93Article
30.
Poskitt  LWojnarowska  F Minimizing cicatricial pemphigoid orodynia with minocycline. Br J Dermatol. 1995;132784- 789Article
31.
Wright  P Enigma of ocular cicatricial pemphigoid: a comparative study of clinical and immunological findings. Trans Ophthalmol Soc U K. 1979;99141- 145
32.
Bialasiewicz  AAForster  WRadig  H  et al.  Syngeneic transplantation of nasal mucosa and azathioprine medication for therapy of cicatricial ocular mucous membrane pemphigoid: study of 9 patients with 11 eyes. Ophthalmologe. 1994;91244- 250
33.
Francis  ICMcCluskey  PJWalls  RSWakefield  DBrewer  JM Ocular cicatricial pemphigoid. Aust N Z J Ophthalmol. 1990;18143- 150Article
34.
Elder  MJLightman  SDart  JK Role of cyclophosphamide and high dose steroid in ocular cicatricial pemphigoid. Br J Ophthalmol. 1995;79264- 266Article
35.
Foster  CSWilson  LAEkins  MB Immunosuppressive therapy for progressive ocular cicatricial pemphigoid. Ophthalmology. 1982;89340- 353Article
36.
Fern  AIJay  JLYoung  HMacKie  R Dapsone therapy for the acute inflammatory phase of ocular pemphigoid. Br J Ophthalmol. 1992;76332- 335Article
37.
Elder  MJLeonard  JDart  JK Sulphapyridine—a new agent for the treatment of ocular cicatricial pemphigoid. Br J Ophthalmol. 1996;80549- 552Article
38.
Gillies  MFrancis  IMcCluskey  PWakefield  D Local interferon alfa-2b for ocular cicatricial pemphigoid [letter]. Br J Ophthalmol. 1996;80927Article
39.
Secchi  AGTognon  MS Intraoperative mitomycin C in the treatment of cicatricial obliterations of conjunctival fornices. Am J Ophthalmol. 1996;122728- 730
40.
Donnenfeld  EDPerry  HDWallerstein  A  et al.  Subconjunctival mitomycin C for the treatment of ocular cicatricial pemphigoid. Ophthalmology. 1999;10672- 79Article
41.
Kirtschig  GWojnarowska  FMarsden  RA  et al.  Acquired bullous disease of childhood: re-evaluation of diagnosis by indirect immunofluorescence examination and immunoblotting. Br J Dermatol. 1994;130610- 616Article
42.
Luke  MCDarling  TNHsu  R  et al.  Mucosal morbidity in patients with epidermolysis bullosa acquisita. Arch Dermatol. 1999;135954- 959Article
43.
Gordon  KBChan  LSWoodley  DT Treatment of refractory epidermolysis bullosa acquisita with extracorporeal photochemotherapy. Br J Dermatol. 1997;136415- 420Article
44.
Cunningham  BBKirchmann  TTWoodley  D Colchicine for epidermolysis bullosa acquisita. J Am Acad Dermatol. 1996;34781- 784Article
45.
Megahed  MScharffetter-Kochanek  K Epidermolysis bullosa acquisita—successful treatment with colchicine. Arch Dermatol Res. 1994;28635- 46Article
46.
Gupta  AKEllis  CNNickoloff  BJ  et al.  Oral cyclosporine in the treatment of inflammatory and noninflammatory dermatoses: a clinical and immunopathologic analysis. Arch Dermatol. 1990;126339- 350Article
47.
Rappersberger  KKonrad  KSchenk  PTappeiner  G Acquired epidermolysis bullosa: a clinico-pathologic study. Hautarzt. 1988;39355- 362
48.
Arpey  CJElewski  BEMoritz  DKGammon  WR Childhood epidermolysis bullosa acquisita. J Am Acad Dermatol. 1991;24706- 714Article
49.
Edwards  SWakelin  SHWojnarowska  F  et al.  Bullous pemphigoid and epidermolysis bullosa acquisita: presentation, prognosis, and immunopathology in 11 children. Pediatr Dermatol. 1998;15184- 190Article
50.
Callot-Mellot  CBodemer  CCaux  F  et al.  Epidermolysis bullosa acquisita in childhood. Arch Dermatol. 1997;1331122- 1126Article
Evidence-Based Dermatology: Original Contribution
March 2002

Interventions for Mucous Membrane Pemphigoid/Cicatricial Pemphigoid and Epidermolysis Bullosa AcquisitaA Systematic Literature Review

Author Affiliations
 

DamianoAbeniMD, MPHMichaelBigbyMDPaoloPasquiniMD, MPHMoysesSzkloMD, MPH, DrPHHywelWilliamsMD

Arch Dermatol. 2002;138(3):380-384. doi:10.1001/archderm.138.3.380
Abstract

Objective  To identify and critically evaluate evidence from randomized controlled trials for the efficacy of treatments for mucous membrane pemphigoid (MMP)/cicatricial pemphigoid (CP) and epidermolysis bullosa acquisita (EBA).

Search Strategy  Review of MEDLINE from 1966 through March 2000, EMBASE from 1980 through March 2000, and the Cochrane Controlled Trials Register (February 28, 2001) to identify randomized controlled trials for the efficacy of treatments in MMP/CP and EBA.

Selection Criteria  All randomized controlled trials of therapeutic interventions that included patients with MMP/CP or EBA confirmed by immunofluorescence study findings. All age groups were included.

Results  We found 2 small randomized controlled trials of MMP/CP, both conducted in patients with severe eye involvement. We were not able to identify a randomized controlled trial of therapeutic interventions in EBA.

Conclusions  There is evidence from 2 small trials that severe ocular CP responds best to treatment with cyclophosphamide, and mild to moderate disease seems effectively suppressed by treatment with dapsone. No treatment recommendations can be made for EBA because to our knowledge no randomized controlled trials are published. Even though systemic corticosteroids are regarded as the gold standard in the treatment of MMP/CP and EBA, there is poor evidence from the literature that they are the best treatment for these diseases.

MUCOUS membrane pemphigoid (MMP) and epidermolysis bullosa acquisita (EBA) are acquired autoimmune bullous disorders of the skin.13 In the present study, we have followed the suggestion by Chan et al4 that MMP should replace the name cicatricial pemphigoid. In MMP and EBA, direct immunofluorescence demonstrates deposits of IgG and C3 at the dermoepidermal junction, and on indirect immunofluorescence, circulating autoantibodies may be detected.13

The incidence of MMP and EBA in western Europe is calculated to be about 1 and 0.2 new cases per 1 000 000 inhabitants per year, respectively.5,6 Scar formation is a characteristic feature in both and may lead to major disability (eg, blindness) and life-threatening situations (eg, respiratory obstruction).

Both MMP and EBA are highly variable and often take a protracted course in contrast to bullous pemphigoid (BP), which usually remits within 5 years.13 Some patients with localized disease (eg, oral involvement [only in MMP]) remain stable for years in the absence of aggressive therapy. Some other patients may develop rapidly progressive ocular involvement despite treatment with immunosuppressants. The standard treatment for progressive disease is the administration of systemic corticosteroids at a dose of 1 to 2 mg of prednisolone equivalent per 1 kg of body weight, which is often combined with cyclophosphamide, azathioprine, or methotrexate; dapsone seems to be an alternative treatment in milder disease.13 These drugs, however, are accompanied by potentially life-threatening complications and may still not lead to the desired therapeutic effect. It is therefore reasonable to search for other treatment options with less severe adverse effects. Newer treatment regimens involve antibiotics, nicotinamide, and immunoglobulins, and these medications are usually better tolerated.710 Initial reports are promising, but it is not known whether recent alternative treatment regimens are equally or even more effective in patients with progressive disease than traditional medication. A review of the evidence is therefore needed to determine the following:

  • Which are the most effective drugs or interventions with the least adverse effects?

  • Does combination therapy (eg, azathioprine plus steroids) offer any advantages over single drugs (eg, oral steroids alone)?

  • Are antibiotics such as tetracyclines, erythromycin, dapsone, and sulfonamides useful?

  • Is systemic treatment better than topical treatment (topical cyclosporine or interferon) in patients with MMP or EBA?

METHODS
SEARCH STRATEGIES FOR THE IDENTIFICATION OF STUDIES

Randomized controlled trials (RCTs) were identified by searching MEDLINE and EMBASE from their inception (1966 and 1980, respectively) up to March 2000. For MEDLINE, the Cochrane Collaborative Review Group search strategy for RCTs was used. The search terms we used were cicatricial pemphigoid, mucous membrane pemphigoid, epidermolysis bullosa acquisita NOT bullous pemphigoid NOT pemphigoid gestationis AND therapy OR treatment, medication, predniso*, corticosteroid*, steroid*, immunosupp*, azathioprin*, cyclophosphamid*, methotrexat*, chlorambucil*, cyclosporin*, antibiotic*, dapson*, sulph*, erythromycin*, tetracyclin*, minocin*, minocyclin*, nicotinamid*, plasmaph*, plasmaexchange*, surg*, and immunoglob*.

The Cochrane Controlled Trials Register (CCTR) was examined (February 28, 2001). The bibliographies from identified studies were searched, and the author (C. S. Foster) who had conducted randomized clinical trials11 in the field was contacted to identify unpublished trials.

STUDY SELECTION

The primary goal of this review was to identify RCTs, since these are considered the best measure for evaluating efficacy of treatment. However, because MMP and EBA are rare diseases, we did not expect to find many RCTs and therefore searched for therapeutic trials involving 2 or more patients with EBA and 5 or more patients with MMP. Participants were male and female patients (all ages) with a diagnosis of MMP or EBA confirmed by immunofluorescence study findings and who received medical intervention for MMP or EBA. Abstracts of potentially relevant studies were screened by 2 reviewers (G.K. and N.K.). Articles that potentially could have been RCTs were assessed for eligibility using the inclusion criteria. The identified studies were individually critically appraised to assess methodological quality. The criteria were randomization, method of randomization, allocation concealment, blinded outcome assessment, and inclusion of all randomized patients in the analysis.

The MEDLINE search found 298 references for MMP and 150 references for EBA; EMBASE identified 200 references for MMP and 108 for EBA. No RCTs were identified through searching the abstracts. The CCTR contained 16 references involving pemphigoid, but none of these studies were RCTs that met our inclusion criteria for MMP. No references were found when searching for EBA. Only 2 RCTs of treatment for MMP were identified by reading through single articles (abstracts of the 2 RCTs were not available in MEDLINE, EMBASE, or the Cochrane Library).11 The author (C. S. Foster) who conducted the 2 trials in MMP was contacted; no more randomized trials could be identified. No RCTs could be found for the treatment of EBA.

DATA EXTRACTION

Data from eligible RCTs were extracted and summarized using a data extraction sheet based on the outcome measures. Three reviewers (G.K., N.K., and D.M.) extracted data independently and subsequently checked for discrepancies. The data were then loaded onto Review Manager software (RevMan 4.0; Cochrane Collaboration, Oxford, England). Outcome measures included the following:

  • Rate of regression or of healing of the skin and mucosal lesions

  • Duration of remissions after stopping treatment

  • Complications of the primary disease (MMP and EBA), such as scarring leading to blindness or airway obstruction

  • Adverse effects of treatment, such as systemic infection, organ failure, allergic and/or toxic reactions

  • Mortality as a result of primary disease or as a result of treatment

RESULTS AND COMMENT

Ten reports of treatment for EBA involving 2 or more patients and 31 reports of treatment for MMP involving 5 or more patients were found. Some of the reports represent follow-ups of earlier studies.1219 Two RCTs (trials 1 and 2) on the treatment of MMP were identified11; no RCT for EBA was found.

The 2 RCTs compared treatments of progressive MMP affecting the eyes (Table 1).11 Both were small, random ized double-blind, non–placebo-controlled studies (grade C according to the criteria set out by Sackett20). One included 24 patients with bilateral ocular stage III MMP (symblepharon formation); treatment with cyclophosphamide plus prednisone vs prednisone alone was tested. The second trial included 40 patients with stage III ocular MMP, but it did not mention if both eyes were affected; treatment with dapsone vs cyclophosphamide was tested. The 64 patients in the 2 trials were part of a study population of 130 patients with MMP involving the eyes collected between 1975 and 1985 at the Immunology and Uveitis Unit, Harvard Medical School, Boston, Mass. All 130 patients had bulbar conjunctival biopsies for histological investigation and direct immunofluorescence. All 64 RCT patients showed linear deposition of immunoglobulins at the basement membrane zone on direct immunofluorescence. All patients entered into the 2 trials completed the studies; none were lost to follow-up.

Trial 1 demonstrates a superior effect of cyclophosphamide and prednisone in combination compared with prednisone alone in the treatment of bilateral stage III MMP involving the eyes. The difference is statistically significant (χ2 analysis, P<.005). It was not clear from the article whether the treatment was stopped after the 6-month treatment period. In discussing the treatment with C. S. Foster (the author of the 2 RCTs), we found out that the recommended duration of treatment is at least 1 year and usually longer.

In trial 2, cyclophosphamide is shown to be superior to dapsone in the treatment of patients with MMP and severe (4+) inflammation of the eyes. The 6 dapsone treatment failures included all 4 of the patients with 4+ conjunctival inflammation prior to therapy. The 2 remaining treatment failures had 3+ activity before treatment. All 6 patients responded well to cyclophosphamide therapy after 3 months of treatment with dapsone had failed to improve their disease. Foster concludes that dapsone is a reasonable first-choice medication for patients with MMP without very active and rapidly progressive disease, provided they are not glucose-6-phosphate dehydrogenase deficient. Foster recommends a dose starting at 25 mg twice daily for 1 week, increasing to 50 mg twice daily, with dosage adjustments based on therapeutic response and drug tolerance. A response can be expected within 4 weeks of treatment.

Adverse effects observed in both trials are listed in Table 2. None of the patients died during treatment or follow-up in either trial.

In trial 1, none of the patients withdrew from systemic immunosuppression because of adverse effects, and none required hospitalization for intervention of any adverse effect. The hair loss was reversible when treatment with cyclophosphamide was discontinued. Leukopenia was a routine finding in all patients successfully treated with cyclophosphamide. The leukopenia was reversible, and the cyclophosphamide dose was adjusted to achieve a leukocyte count between 2500/µL and 4000/µL. Macrocytic anemia was asymptomatic and of mild to moderate degree. Microcytic hematuria was discovered in routine urinalysis; an alteration in timing of cyclophosphamide administration and increased fluid intake eliminated this potentially serious adverse effect. Foster believes that systemic immunosuppression poses fewer risks if properly used compared with long-term corticosteroid therapy.

In trial 2, microcytic hematuria developed in 2 patients, necessitating a reduction of cyclophosphamide. Foster11 emphasizes that dapsone is not a benign drug, and death may occur as a result of agranulocytosis, aplastic anemia, or hemolytic anemia.

We found 30 additional studies of treatment in MMP involving 5 or more patients. Fourteen studies investigated patients with oral and generalized MMP,7,1719,2130 7 of which comment on sulfa drugs (dapsone, sulfapyridine, and sulfamethoxypyridazine); 68 of 106 patients benefited from this medication.1719,22,23,29 Of the 14 studies, 3 discuss the use of oral vs topical steroids in oral MMP, the results of which are controversial.2628 Minocycline treatment is reported in 25 patients with generalized MMP.7,29,30 This medication seems beneficial in oral MMP (orodynia), although little effect is seen in ocular disease. Sixteen articles present patients with mainly ocular MMP.9,1216,3140 Three of these articles support the effectiveness of sulfa drugs in moderate ocular MMP12,36,37; Elder et al37 found sulfapyridine to be superior to dapsone. Early studies suggest that ocular MMP shows less progression when patients are immunosuppressed; treatment with cyclophosphamide in addition to oral corticosteroids seems to be more effective than, for example, azathioprine.1216,31

Recent trials report topical mitomycin to be beneficial in severe ocular MMP (14 patients)39,40; Donnenfeld et al40 describe no progression in 8 of 9 treated eyes vs progression in 5 of 9 untreated eyes (internal control). Intravenous immunoglobulins have been successfully used in one study of 10 patients with ocular MMP resistant to conventional treatment.9

We identified 11 articles on treatment in EBA involving 2 or more patients, detailing results in 20 adults and 11 children.10,4150 The adult patients were treated with various medications including systemic corticosteroids, immunosuppressants, dapsone, colchicine, and intravenous immunoglobulins; it is not possible to draw any conclusion regarding the superiority of any of these treatments. Most children were treated with systemic corticosteroids and/or dapsone.41,4850 In children, EBA seems to remit within a few years, and it is not possible to judge if this is due to treatment or represents a spontaneous remission.

CONCLUSIONS

It is not possible to draw definite conclusions for the treatment of MMP or EBA. Long-term corticosteroid treatment puts patients at risk of serious complications and seems to be less effective than cyclophosphamide in suppressing scarring MMP involving the eyes. Mild to moderate MMP involving the eyes seems to respond well to dapsone in most patients; however, dapsone has potentially serious adverse effects as well.

No evidence-based recommendation can be given for the treatment of EBA. However, uncontrolled studies suggest that children seem to respond to treatment with a combination of systemic corticosteroids and dapsone. International multicenter RCTs involving larger numbers of patients are required to assess the best treatment for MMP and EBA. Perhaps newer treatments with anti-inflammatory antibiotics such as tetracycline and minocycline are as effective as dapsone and have the benefit of fewer adverse effects.

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Article Information

Accepted for publication November 29, 2001.

We thank Anna Truelove for reviewing the manuscript and her helpful linguistic comments.

A cooperative effort of the Clinical Epidemiology Unit of the Istituto Dermopatico dell'Immacolata–Istituto di Ricovero e Cura a Carattere Scientifico (IDI-IRCCS) and the Archives of Dermatology

Corresponding author: Gudula Kirtschig, MD, Vrije Universiteit, Academisch Ziekenhuis, Department of Dermatology, Postbus 7057, 1007 MB Amsterdam, the Netherlands (e-mail: G.Kirtschig@vumc.nl).

References
1.
Wojnarowska  FEady  RAJBurge  SM Bullous eruptions. Champion  RHBurton  JLBurns  DABreathnach  SMeds.Textbook of Dermatology Oxford, England Blackwell Science Ltd1998;1873- 18771884- 1887
2.
Fine  J-D Cicatricial pemphigoid. Wojnarowska  FBriggaman  RAeds.Management of Blistering Diseases. London, England Chapman & Hall1990;83- 92
3.
Briggaman  RAGammon  WRWoodley  DT Epidermolysis bullosa acquisita. Wojnarowska  FBriggaman  RAeds.Management of Blistering Diseases. London, England Chapman & Hall1990;127- 138
4.
Chan  LSAhmed  ARAnhalt  GJ  et al.  The first international consensus on mucous membrane pemphigoid: definition, diagnostic criteria, pathogenic factors, medical treatment, and prognostic indicators. Arch Dermatol. 2002;138370- 379
5.
Bernard  PVaillant  LLabeille  B  et al. for the French Bullous Study Group, Incidence and distribution of subepidermal autoimmune bullous skin diseases in three French regions. Arch Dermatol. 1995;13148- 52Article
6.
Zillikens  DWever  SRoth  ABröcker  EB Incidence of autoimmune subepidermal blistering dermatoses in a region of central Germany. Arch Dermatol. 1995;131957- 958Article
7.
Reiche  LWojnarowska  FMallon  E Combination therapy with nicotinamide and tetracyclines for cicatricial pemphigoid: further support for its efficacy. Clin Exp Dermatol. 1998;23254- 257Article
8.
Dragan  LEng  AMLam  SPersson  T Tetracycline and niacinamide: treatment alternatives in ocular cicatricial pemphigoid. Cutis. 1999;63181- 183
9.
Foster  CSAhmed  AR Intravenous immunoglobulin therapy for ocular cicatricial pemphigoid: a preliminary study. Ophthalmology. 1999;1062136- 2143Article
10.
Harman  KEBlack  MM High-dose intravenous immune globulin for the treatment of autoimmune blistering diseases: an evaluation of its use in 14 cases. Br J Dermatol. 1999;140865- 874Article
11.
Foster  CS Cicatricial pemphigoid. Trans Am Ophthalmol Soc. 1986;84527- 663
12.
Tauber  JSainz de la Maza  MFoster  CS Systemic chemotherapy for ocular cicatricial pemphigoid. Cornea. 1991;10185- 195Article
13.
Neumann  RTauber  JFoster  CS Remission and recurrence after withdrawal of therapy for ocular cicatricial pemphigoid. Ophthalmology. 1991;98858- 862Article
14.
Foster  CSNeumann  RTauber  J Long-term results of systemic chemotherapy for ocular cicatricial pemphigoid. Doc Ophthalmol. 1992;82223- 229Article
15.
Mondino  BJBrown  SI Immunosuppressive therapy in ocular cicatricial pemphigoid. Am J Ophthalmol. 1983;96453- 459
16.
Mondino  BJ Cicatricial pemphigoid and erythema multiforme. Ophthalmology. 1990;97939- 952Article
17.
Rogers III  RSSeehafer  JRPerry  HO Treatment of cicatricial (benign mucous membrane) pemphigoid with dapsone. J Am Acad Dermatol. 1982;6215- 223Article
18.
Rogers III  RS Dapsone and sulfapyridine therapy of pemphigoid diseases. Australas J Dermatol. 1986;2758- 63Article
19.
Rogers III  RSMehregan  DA Dapsone therapy of cicatricial pemphigoid. Semin Dermatol. 1988;7201- 205
20.
Sackett  DL Rules of evidence and clinical recommendations on the use of antithrombotic agents. Chest. 1989;95 (suppl 2) 2S- 4SArticle
21.
Hanson  RDOlsen  KDRogers III  RS Upper aerodigestive tract manifestations of cicatricial pemphigoid. Ann Otol Rhinol Laryngol. 1988;97493- 499
22.
McFadden  JPLeonard  JNPowles  AVRutman  AJFry  L Sulphamethoxypyridazine for dermatitis herpetiformis, linear IgA disease and cicatricial pemphigoid. Br J Dermatol. 1989;121759- 762Article
23.
Matthews  RWPinkney  RCScully  C The management of intransigent desquamative gingivitis with dapsone. Ann Dent. 1989;4841- 43
24.
Lamey  PJRees  TDBinnie  WHRankin  KV Mucous membrane pemphigoid: treatment experience at two institutions. Oral Surg Oral Med Oral Pathol. 1992;7450- 53Article
25.
Axt  MWever  SBaier  G  et al.  Cicatricial pemphigoid—a therapeutic problem. Hautarzt. 1995;46620- 627Article
26.
Vincent  SDLilly  GEBaker  KA Clinical, historic, and therapeutic features of cicatricial pemphigoid: a literature review and open therapeutic trial with corticosteroids. Oral Surg Oral Med Oral Pathol. 1993;76453- 459Article
27.
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