 |
|
Interventions for Mucous Membrane Pemphigoid/Cicatricial Pemphigoid and Epidermolysis Bullosa Acquisita
A Systematic Literature Review
Gudula Kirtschig, MD;
Dédée Murrell, FAAD;
Fenella Wojnarowska, DM;
Nonhlanhla Khumalo
Arch Dermatol. 2002;138:380-384.
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.
INTRODUCTION
MUCOUS membrane pemphigoid (MMP) and epidermolysis bullosa acquisita
(EBA) are acquired autoimmune bullous disorders of the skin.1-3
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.1-3
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.1-3 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.1-3
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.7-10
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.12-19
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.
|
|
|
|
Table 1. Summary of Randomized Controlled Trials in the Treatment of
Ocular Cicatricial Pemphigoid
|
|
|
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
( 2 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.
|
|
|
|
Table 2. Adverse Effects Observed in Ocular Cicatricial Pemphigoid
During 1-Year Treatment and Follow-up*
|
|
|
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, 17-19,21-30
7 of which comment on sulfa drugs (dapsone, sulfapyridine, and sulfamethoxypyridazine);
68 of 106 patients benefited from this medication.17-19,22-23,29
Of the 14 studies, 3 discuss the use of oral vs topical steroids in oral MMP,
the results of which are controversial.26-28
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, 12-16,31-40
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.12-16,31
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, 41-50
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, 48-50
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.
AUTHOR 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{at}vumc.nl).
From the Department of Dermatology, Oxford Radcliffe Hospital, Oxford,
England (Drs Kirtschig and Wojnarowska and Ms Khumalo); the Department of
Dermatology, Academisch Ziekenhuis, Vrije Universiteit, Amsterdam, the Netherlands
(Dr Kirtschig); the Department of Dermatology, St George Hospital, University
of New South Wales, Sydney, Australia (Dr Murrell); and the Department of
Dermatology, Observatory, Groote Schuur Hospital, Capetown, South Africa (Ms
Khumalo).
REFERENCES
| |
1. Wojnarowska F, Eady RAJ, Burge SM. Bullous eruptions. In: Champion RH, Burton JL, Burns DA, Breathnach SM, eds. Textbook of Dermatology. Oxford, England: Blackwell Science Ltd; 1998:1873-1877,
1884-1887.
2. Fine J-D. Cicatricial pemphigoid. In: Wojnarowska F, Briggaman RA, eds. Management
of Blistering Diseases. London, England: Chapman & Hall; 1990:83-92.
3. Briggaman RA, Gammon WR, Woodley DT. Epidermolysis bullosa acquisita. In: Wojnarowska F, Briggaman RA, eds. Management
of Blistering Diseases. London, England: Chapman & Hall; 1990:127-138.
4. Chan LS, Ahmed AR, Anhalt GJ, et al. The first international consensus on mucous membrane pemphigoid: definition,
diagnostic criteria, pathogenic factors, medical treatment, and prognostic
indicators. Arch Dermatol. 2002;138:370-379.
FREE FULL TEXT
5. Bernard P, Vaillant L, Labeille 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;131:48-52.
ABSTRACT
6. Zillikens D, Wever S, Roth A, Bröcker EB. Incidence of autoimmune subepidermal blistering dermatoses in a region
of central Germany. Arch Dermatol. 1995;131:957-958.
FULL TEXT
|
ISI
| PUBMED
7. Reiche L, Wojnarowska F, Mallon E. Combination therapy with nicotinamide and tetracyclines for cicatricial
pemphigoid: further support for its efficacy. Clin Exp Dermatol. 1998;23:254-257.
FULL TEXT
|
ISI
| PUBMED
8. Dragan L, Eng AM, Lam S, Persson T. Tetracycline and niacinamide: treatment alternatives in ocular cicatricial
pemphigoid. Cutis. 1999;63:181-183.
PUBMED
9. Foster CS, Ahmed AR. Intravenous immunoglobulin therapy for ocular cicatricial pemphigoid:
a preliminary study. Ophthalmology. 1999;106:2136-2143.
FULL TEXT
|
ISI
| PUBMED
10. Harman KE, Black 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;140:865-874.
FULL TEXT
|
ISI
| PUBMED
11. Foster CS. Cicatricial pemphigoid. Trans Am Ophthalmol Soc. 1986;84:527-663.
PUBMED
12. Tauber J, Sainz de la Maza M, Foster CS. Systemic chemotherapy for ocular cicatricial pemphigoid. Cornea. 1991;10:185-195.
FULL TEXT
|
ISI
| PUBMED
13. Neumann R, Tauber J, Foster CS. Remission and recurrence after withdrawal of therapy for ocular cicatricial
pemphigoid. Ophthalmology. 1991;98:858-862.
ISI
| PUBMED
14. Foster CS, Neumann R, Tauber J. Long-term results of systemic chemotherapy for ocular cicatricial pemphigoid. Doc Ophthalmol. 1992;82:223-229.
FULL TEXT
|
ISI
| PUBMED
15. Mondino BJ, Brown SI. Immunosuppressive therapy in ocular cicatricial pemphigoid. Am J Ophthalmol. 1983;96:453-459.
ISI
| PUBMED
16. Mondino BJ. Cicatricial pemphigoid and erythema multiforme. Ophthalmology. 1990;97:939-952.
ISI
| PUBMED
17. Rogers III RS, Seehafer JR, Perry HO. Treatment of cicatricial (benign mucous membrane) pemphigoid with dapsone. J Am Acad Dermatol. 1982;6:215-223.
ISI
| PUBMED
18. Rogers III RS. Dapsone and sulfapyridine therapy of pemphigoid diseases. Australas J Dermatol. 1986;27:58-63.
PUBMED
19. Rogers III RS, Mehregan DA. Dapsone therapy of cicatricial pemphigoid. Semin Dermatol. 1988;7:201-205.
ISI
| PUBMED
20. Sackett DL. Rules of evidence and clinical recommendations on the use of antithrombotic
agents. Chest. 1989;95(suppl 2):2S-4S.
21. Hanson RD, Olsen KD, Rogers III RS. Upper aerodigestive tract manifestations of cicatricial pemphigoid. Ann Otol Rhinol Laryngol. 1988;97:493-499.
ISI
| PUBMED
22. McFadden JP, Leonard JN, Powles AV, Rutman AJ, Fry L. Sulphamethoxypyridazine for dermatitis herpetiformis, linear IgA disease
and cicatricial pemphigoid. Br J Dermatol. 1989;121:759-762.
PUBMED
23. Matthews RW, Pinkney RC, Scully C. The management of intransigent desquamative gingivitis with dapsone. Ann Dent. 1989;48:41-43.
PUBMED
24. Lamey PJ, Rees TD, Binnie WH, Rankin KV. Mucous membrane pemphigoid: treatment experience at two institutions. Oral Surg Oral Med Oral Pathol. 1992;74:50-53.
FULL TEXT
|
ISI
| PUBMED
25. Axt M, Wever S, Baier G, et al. Cicatricial pemphigoid—a therapeutic problem. Hautarzt. 1995;46:620-627.
FULL TEXT
|
ISI
| PUBMED
26. Vincent SD, Lilly GE, Baker 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;76:453-459.
FULL TEXT
|
ISI
| PUBMED
27. Carrozzo M, Carbone M, Broccoletti R, Garzino-Demo P, Gandolfo S. Therapeutic management of mucous membrane pemphigoid: report of 11
cases. Minerva Stomatol. 1997;46:553-559.
PUBMED
28. Carbone M, Carrozzo M, Castellano S, et al. Systemic corticosteroid therapy of oral vesiculoerosive diseases (OVED):
an open trial. Minerva Stomatol. 1998;47:479-487.
PUBMED
29. Nayar M, Wojnarowska F. Cicatricial pemphigoid: a re-evaluation of therapy. J Dermatol Treat. 1993;4:89-93.
30. Poskitt L, Wojnarowska F. Minimizing cicatricial pemphigoid orodynia with minocycline. Br J Dermatol. 1995;132:784-789.
ISI
| PUBMED
31. Wright P. Enigma of ocular cicatricial pemphigoid: a comparative study of clinical
and immunological findings. Trans Ophthalmol Soc U K. 1979;99:141-145.
PUBMED
32. Bialasiewicz AA, Forster W, Radig 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;91:244-250.
PUBMED
33. Francis IC, McCluskey PJ, Walls RS, Wakefield D, Brewer JM. Ocular cicatricial pemphigoid. Aust N Z J Ophthalmol. 1990;18:143-150.
PUBMED
34. Elder MJ, Lightman S, Dart JK. Role of cyclophosphamide and high dose steroid in ocular cicatricial
pemphigoid. Br J Ophthalmol. 1995;79:264-266.
FREE FULL TEXT
35. Foster CS, Wilson LA, Ekins MB. Immunosuppressive therapy for progressive ocular cicatricial pemphigoid. Ophthalmology. 1982;89:340-353.
ISI
| PUBMED
36. Fern AI, Jay JL, Young H, MacKie R. Dapsone therapy for the acute inflammatory phase of ocular pemphigoid. Br J Ophthalmol. 1992;76:332-335.
FREE FULL TEXT
37. Elder MJ, Leonard J, Dart JK. Sulphapyridine—a new agent for the treatment of ocular cicatricial
pemphigoid. Br J Ophthalmol. 1996;80:549-552.
FREE FULL TEXT
38. Gillies M, Francis I, McCluskey P, Wakefield D. Local interferon alfa-2b for ocular cicatricial pemphigoid [letter]. Br J Ophthalmol. 1996;80:927.
39. Secchi AG, Tognon MS. Intraoperative mitomycin C in the treatment of cicatricial obliterations
of conjunctival fornices. Am J Ophthalmol. 1996;122:728-730.
ISI
| PUBMED
40. Donnenfeld ED, Perry HD, Wallerstein A, et al. Subconjunctival mitomycin C for the treatment of ocular cicatricial
pemphigoid. Ophthalmology. 1999;106:72-79.
FULL TEXT
|
ISI
| PUBMED
41. Kirtschig G, Wojnarowska F, Marsden RA, et al. Acquired bullous disease of childhood: re-evaluation of diagnosis by
indirect immunofluorescence examination and immunoblotting. Br J Dermatol. 1994;130:610-616.
FULL TEXT
|
ISI
| PUBMED
42. Luke MC, Darling TN, Hsu R, et al. Mucosal morbidity in patients with epidermolysis bullosa acquisita. Arch Dermatol. 1999;135:954-959.
FREE FULL TEXT
43. Gordon KB, Chan LS, Woodley DT. Treatment of refractory epidermolysis bullosa acquisita with extracorporeal
photochemotherapy. Br J Dermatol. 1997;136:415-420.
FULL TEXT
|
ISI
| PUBMED
44. Cunningham BB, Kirchmann TT, Woodley D. Colchicine for epidermolysis bullosa acquisita. J Am Acad Dermatol. 1996;34:781-784.
FULL TEXT
|
ISI
| PUBMED
45. Megahed M, Scharffetter-Kochanek K. Epidermolysis bullosa acquisita—successful treatment with colchicine. Arch Dermatol Res. 1994;286:35-46.
PUBMED
46. Gupta AK, Ellis CN, Nickoloff BJ, et al. Oral cyclosporine in the treatment of inflammatory and noninflammatory
dermatoses: a clinical and immunopathologic analysis. Arch Dermatol. 1990;126:339-350.
ABSTRACT
47. Rappersberger K, Konrad K, Schenk P, Tappeiner G. Acquired epidermolysis bullosa: a clinico-pathologic study. Hautarzt. 1988;39:355-362.
PUBMED
48. Arpey CJ, Elewski BE, Moritz DK, Gammon WR. Childhood epidermolysis bullosa acquisita. J Am Acad Dermatol. 1991;24:706-714.
PUBMED
49. Edwards S, Wakelin SH, Wojnarowska F, et al. Bullous pemphigoid and epidermolysis bullosa acquisita: presentation,
prognosis, and immunopathology in 11 children. Pediatr Dermatol. 1998;15:184-190.
FULL TEXT
|
ISI
| PUBMED
50. Callot-Mellot C, Bodemer C, Caux F, et al. Epidermolysis bullosa acquisita in childhood. Arch Dermatol. 1997;133:1122-1126.
ABSTRACT
Section Editors:
Damiano Abeni, MD, MPH,
Istituto Dermopatico dell'Immacolata, Rome, Italy
Michael Bigby, MD, Beth Israel Deaconess Medical Center, Harvard Medical
School, Boston, Mass
Paolo Pasquini, MD, MPH,
Istituto Dermopatico dell'Immacolata, Rome, Italy
Moyses Szklo, MD, MPH, DrPH, The Johns Hopkins University, Baltimore,
Md
Hywel Williams, MD, Queens Medical Centre,
Nottingham, England
RELATED ARTICLES
The First International Consensus on Mucous Membrane Pemphigoid: Definition, Diagnostic Criteria, Pathogenic Factors, Medical Treatment, and Prognostic Indicators
Lawrence S. Chan, A. Razzaque Ahmed, Grant J. Anhalt, Wolfgang Bernauer, Kevin D. Cooper, Mark J. Elder, Jo-David Fine, C. Stephen Foster, Reza Ghohestani, Takashi Hashimoto, Thanh Hoang-Xuan, Gudula Kirtschig, Neil J. Korman, Susan Lightman, Francina Lozada-Nur, M. Peter Marinkovich, Bartly J. Mondino, Catherine Prost-Squarcioni, Roy S. Rogers III, Jane F. Setterfield, Dennis P. West, Fenella Wojnarowska, David T. Woodley, Kim B. Yancey, Detlef Zillikens, and John J. Zone
Arch Dermatol. 2002;138(3):370-379.
ABSTRACT
| FULL TEXT
Archives of Dermatology Reader's Choice: Continuing Medical Education
Arch Dermatol. 2002;138(3):421-422.
FULL TEXT
THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES
|
Clinical Response of Severe Mechanobullous Epidermolysis Bullosa Acquisita to Combined Treatment With Immunoadsorption and Rituximab (Anti-CD20 Monoclonal Antibodies)
Niedermeier et al.
Arch Dermatol 2007;143:192-198.
ABSTRACT
| FULL TEXT
Development of ocular disease in patients with mucous membrane pemphigoid involving the oral mucosa
Higgins et al.
Br. J. Ophthalmol. 2006;90:964-967.
ABSTRACT
| FULL TEXT
Successful Adjuvant Treatment of Recalcitrant Epidermolysis Bullosa Acquisita With Anti-CD20 Antibody Rituximab.
Schmidt et al.
Arch Dermatol 2006;142:147-150.
FULL TEXT
Consensus Statement on the Use of Intravenous Immunoglobulin Therapy in the Treatment of Autoimmune Mucocutaneous Blistering Diseases
Ahmed and Dahl
Arch Dermatol 2003;139:1051-1059.
ABSTRACT
| FULL TEXT
|
