Guidelines for the management of bullous pemphigoid

“…Previous studies have identified a number of negative prognostic factors, such as age, low Karnofsky performance score and use of corticosteroids [16,18,19]. Immunosuppressive agents, systematic use of which has not been validated, may also affect the mortality rate [20]. …”

Section: Introductionmentioning

confidence: 99%

Mortality Rate in Bullous Pemphigoid: A Retrospective Monocentric Cohort Study

et al. 2012

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Background: Bullous pemphigoid (BP) is the most common autoimmune blistering skin disease and is associated with an increased mortality. The end points of our study were to evaluate the mortality rate in a retrospective cohort of BP patients followed up to 5 years after the diagnosis and to determine prognostics factors. Methods: All new cases of BP diagnosed between 1990 and 2003 in the University Hospital of Geneva were retrospectively collected. 60 patients were included, 47 (88.6%) of whom were treated with a combination of corticosteroids and chlorambucil. Results: The 1-year, 2-year and 5-year probabilities of death were 26.7, 37.1 and 60.8%, respectively. Old age, dementia and use of chlorambucil at initial doses of 6 mg/day, but not at lower doses, were associated with poor prognosis in multivariate analysis. Conclusion: Our study confirms that BP is associated with a high mortality. The observed mortality rates are however higher than those of previous studies, which is probably related to the inclusion of more debilitated patients.

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“…Recognizing this form of BP is important, since a high suspicion rate, extended and repeated diagnostic procedures are prerequisites for establishing the diagnosis of BP. Once the diagnosis is made, appropriate therapy of secondary infection and of BP along the proposed guidelines [33] will usually be successful.…”

Section: Discussionmentioning

confidence: 99%

Ecthyma-Gangrenosum-Like Bullous Pemphigoid

et al. 2010

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Bullous pemphigoid (BP) is a chronic, autoimmune, subepidermal blistering skin disease with varied clinical presentations. Diagnosis is based on the clinical picture, histopathological findings, and direct and indirect immunofluorescence studies. In unclear cases, ELISA or Western blot analysis helps to establish a definite diagnosis by the detection of immunoglobulin G autoantibodies specific for the hemidesmosomal BP antigens BP230 and BP180. We report 3 cases of BP with an as yet not characterized, distinctive ecthyma-gangrenosum-like presentation. Patients were female, above 80 years of age, physically immobile, and skin lesions showed truncal localization and bacterial colonization. Factors contributing to physical immobility were a high body mass index, psychiatric disease, sedative medication and rheumatic disease. The clinical picture resembled ecthyma gangrenosum but lacked systemic infection with Pseudomonas aeruginosa. Lesional bacteriological studies revealed Staphylococcusaureus and/or P. aeruginosa. Diagnosis proved challenging in all cases. Suspicion has to be high, and repeated diagnostic procedures and additional laboratory studies may be necessary to establish a definitive diagnosis of BP. In summary, we propose this combination of truncal ecthyma-gangrenosum-like lesions with bacterial colonization in the context of older age and immobility as a clinically distinct presentation or variant of BP.

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Guidelines for the management of bullous pemphigoid

Cited by 131 publications

References 47 publications

Role of Corticosteroids in Oral Lesions

Role of Corticosteroids in Oral Lesions

Mortality Rate in Bullous Pemphigoid: A Retrospective Monocentric Cohort Study

Ecthyma-Gangrenosum-Like Bullous Pemphigoid

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