Differences and similarities among expert opinions on the diagnosis and treatment of pemphigus vulgaris

Daniel Mimouni, Carlos H. Nousari, Deborah L. Cummins, David J. Kouba, Michael David, Grant James Anhalt

Research output: Contribution to journalArticle

Abstract

Background: As a result of a lack of large-scale controlled studies, the diagnosis and management of pemphigus vulgaris (PV) has been solely on the basis of expert opinion, rather than on empirical evidence. We have completed a survey of worldwide experts on the diagnostic and therapeutic approaches to PV. Methods: We conducted a telephone-based survey of 24 physicians from academic, tertiary care centers worldwide with an average of 20 years experience treating pemphigus. Survey questions included referral patterns, diagnostic techniques, and therapeutic regimens. Results: Of those surveyed, 50% receive referrals within 6 months after onset of symptoms, 17% within 1 year, and 8% within 3 years. Diagnosis is secured by 96% using skin biopsy specimen with direct immunofluorescence, and by indirect immunofluorescence alone for 4%. None of the participating physicians make the diagnosis of PV solely on clinical and histologic evidence. Of the physicians, 75% initially treat with prednisone and 25% use other agents or attempt to eliminate potential triggers. The physicians who initially used noncorticosteroid drugs did so with no relation to the nature or extent of the disease. Of those surveyed, 50% use prednisone doses of 1 mg/kg/d, 31% use 1 to 1.5 mg/kg/d, and 19% use 1.5 to 3 mg/kg/d. Azathioprine is used as an adjuvant by 44%, mycophenolate mofetil by 20%, cyclophosphamide by 16%, and methotrexate by 8%. Complete discontinuation of prednisone was the goal for 37% whereas others were satisfied with doses from 2.5 to 10 mg/d. Conclusion: Wide variation exists in diagnostic techniques and treatment of PV, even among the world's experts. The lag time from symptom onset to referral emphasizes the need for heightened awareness. There is clearly a need for consensus standards with regard to patient stratification and randomized controlled trials.

Original languageEnglish (US)
Pages (from-to)1059-1062
Number of pages4
JournalJournal of the American Academy of Dermatology
Volume49
Issue number6
DOIs
StatePublished - Dec 2003

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Pemphigus
Expert Testimony
Prednisone
Physicians
Referral and Consultation
Mycophenolic Acid
Therapeutics
Direct Fluorescent Antibody Technique
Azathioprine
Indirect Fluorescent Antibody Technique
Telephone
Tertiary Care Centers
Methotrexate
Cyclophosphamide
Randomized Controlled Trials
Biopsy
Skin
Pharmaceutical Preparations
Surveys and Questionnaires

ASJC Scopus subject areas

  • Dermatology

Cite this

Differences and similarities among expert opinions on the diagnosis and treatment of pemphigus vulgaris. / Mimouni, Daniel; Nousari, Carlos H.; Cummins, Deborah L.; Kouba, David J.; David, Michael; Anhalt, Grant James.

In: Journal of the American Academy of Dermatology, Vol. 49, No. 6, 12.2003, p. 1059-1062.

Research output: Contribution to journalArticle

Mimouni, Daniel ; Nousari, Carlos H. ; Cummins, Deborah L. ; Kouba, David J. ; David, Michael ; Anhalt, Grant James. / Differences and similarities among expert opinions on the diagnosis and treatment of pemphigus vulgaris. In: Journal of the American Academy of Dermatology. 2003 ; Vol. 49, No. 6. pp. 1059-1062.
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abstract = "Background: As a result of a lack of large-scale controlled studies, the diagnosis and management of pemphigus vulgaris (PV) has been solely on the basis of expert opinion, rather than on empirical evidence. We have completed a survey of worldwide experts on the diagnostic and therapeutic approaches to PV. Methods: We conducted a telephone-based survey of 24 physicians from academic, tertiary care centers worldwide with an average of 20 years experience treating pemphigus. Survey questions included referral patterns, diagnostic techniques, and therapeutic regimens. Results: Of those surveyed, 50{\%} receive referrals within 6 months after onset of symptoms, 17{\%} within 1 year, and 8{\%} within 3 years. Diagnosis is secured by 96{\%} using skin biopsy specimen with direct immunofluorescence, and by indirect immunofluorescence alone for 4{\%}. None of the participating physicians make the diagnosis of PV solely on clinical and histologic evidence. Of the physicians, 75{\%} initially treat with prednisone and 25{\%} use other agents or attempt to eliminate potential triggers. The physicians who initially used noncorticosteroid drugs did so with no relation to the nature or extent of the disease. Of those surveyed, 50{\%} use prednisone doses of 1 mg/kg/d, 31{\%} use 1 to 1.5 mg/kg/d, and 19{\%} use 1.5 to 3 mg/kg/d. Azathioprine is used as an adjuvant by 44{\%}, mycophenolate mofetil by 20{\%}, cyclophosphamide by 16{\%}, and methotrexate by 8{\%}. Complete discontinuation of prednisone was the goal for 37{\%} whereas others were satisfied with doses from 2.5 to 10 mg/d. Conclusion: Wide variation exists in diagnostic techniques and treatment of PV, even among the world's experts. The lag time from symptom onset to referral emphasizes the need for heightened awareness. There is clearly a need for consensus standards with regard to patient stratification and randomized controlled trials.",
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