Pemphigus
Резюме
Определение
Анамнез и осмотр
Ключевые диагностические факторы
- chronic erosive blistering of the skin, mucosa, or both
- chronic mouth erosions (PV, PNP)
- painful lips (PNP)
- shortness of breath (PNP)
Другие диагностические факторы
- pruritic scalp (PV, PF)
- bloody nose (PV, PNP)
- painful skin (PV, PF, PNP)
- dysphagia (PV)
- pruritic skin (PV, PF)
- conjunctivitis (PV, PNP)
Факторы риска
- increasing age
- HLA DR4 (PV)
- HLA DQ1 (PV)
- HLA DRB1 (PNP)
- associated malignancy (PNP)
- penicillamine use
- ACE inhibitors use
- penicillin use
- tiopronin use
- interleukin-2 use
- nifedipine use
- rifampin use
- exposure to hematophagous insects
Диагностические исследования
Исследования, которые показаны в первую очередь
- skin biopsy, hematoxylin and eosin stain
- skin biopsy, direct immunofluorescence
Исследования, проведение которых нужно рассмотреть
- indirect immunofluorescence on serum
- serum ELISA
- upper gastrointestinal endoscopy
- CXR
- chest CT scan
- PFT
- serum immunoblot (Western blot)
Алгоритм лечения
mild pemphigus vulgaris
mild pemphigus foliaceus
moderate to severe pemphigus vulgaris or pemphigus foliaceus
paraneoplastic pemphigus
moderate to severe pemphigus vulgaris or pemphigus foliaceus 6 months after initial therapy: with disease control/complete remission
moderate to severe pemphigus vulgaris or pemphigus foliaceus 6 months after initial therapy: without complete remission
moderate to severe pemphigus vulgaris or pemphigus foliaceus 12 to 18 months after initial therapy: complete remission
severe/refractory pemphigus
Составители
Авторы
Jon H. Meyerle, MD
Associate Professor
Department of Dermatology
Uniformed Services University of the Health Sciences
Bethesda
MD
Раскрытие информации
JHM declares that he has no competing interests.
Grant J. Anhalt, MD
Professor
Department of Dermatology
Johns Hopkins Medical Institution
Baltimore
MD
Раскрытие информации
GJA declares that he has no competing interests.
Рецензенты
Daniel Mimouni, MD
Professor
Department of Dermatology
Rabin Medical Center
Petah Tikva
Israel
Раскрытие информации
DM declares that he has no competing interests.
Vesna Petronic-Rosic, MD, MSc
Associate Professor and Clinical Director
Section of Dermatology
University of Chicago
Chicago
IL
Раскрытие информации
VPR declares that she has no competing interests.
Timothy Patton, MD
Assistant Professor of Dermatology
Department of Dermatology
University of Pittsburgh
Pittsburgh
PA
Раскрытие информации
TP declares that he has no competing interests.
Peer reviewer acknowledgements
BMJ Best Practice topics are updated on a rolling basis in line with developments in evidence and guidance. The peer reviewers listed here have reviewed the content at least once during the history of the topic.
Disclosures
Peer reviewer affiliations and disclosures pertain to the time of the review.
Список литературы
Основные статьи
Joly P, Horvath B, Patsatsi Α, et al. Updated S2K guidelines on the management of pemphigus vulgaris and foliaceus initiated by the european academy of dermatology and venereology (EADV). J Eur Acad Dermatol Venereol. 2020 Sep;34(9):1900-13.Полный текст Аннотация
Antiga E, Bech R, Maglie R, et al. S2k guidelines on the management of paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome initiated by the European Academy of Dermatology and Venereology (EADV). J Eur Acad Dermatol Venereol. 2023 Jun;37(6):1118-34.Полный текст Аннотация
Murrell DF, Peña S, Joly P, et al. Diagnosis and management of pemphigus: recommendations of an international panel of experts. J Am Acad Dermatol. 2020 Mar;82(3):575-85.e1.Полный текст Аннотация
Joly P, Maho-Vaillant M, Prost-Squarcioni C, et al. First-line rituximab combined with short-term prednisone versus prednisone alone for the treatment of pemphigus (Ritux 3): a prospective, multicentre, parallel-group, open-label randomised trial. Lancet. 2017 May 20;389(10083):2031-40. Аннотация
Статьи, указанные как источники
A full list of sources referenced in this topic is available to users with access to all of BMJ Best Practice.
Отличия
- Familial benign pemphigus (Hailey-Hailey disease)
- Bullous pemphigoid (BP)
- Linear IgA bullous dermatosis
Больше ОтличияРекомендации
- Updated S2K guidelines on the management of paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome initiated by the European Academy of Dermatology and Venereology (EADV)
- S2k guidelines on the management of paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome initiated by the European Academy of Dermatology and Venereology (EADV)
Больше Рекомендации
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