Summary
Definition
History and exam
Key diagnostic factors
- presença de fatores de risco
- formação de bolhas erosivas crônica da pele, mucosa ou ambas
- erosões crônicas da boca (pênfigo vulgar [PV], pênfigo paraneoplásico [PPN])
- lábios dolorosos (PPN)
- dispneia (PPN)
Other diagnostic factors
- couro cabeludo pruriginoso (PV, PF)
- epistaxe (PV, PPN)
- pele dolorosa (PV, PF, PPN)
- disfagia (PV)
- pele pruriginosa (PV, PF)
- conjuntivite (PV, PPN)
Risk factors
- idade mais avançada
- HLA DR4 (pênfigo vulgar [PV])
- HLA DQ1 (PV)
- HLA DRB1 (pênfigo paraneoplásico [PPN])
- neoplasia maligna subjacente (PPN)
- D-penicilamina
- inibidores da enzima conversora da angiotensina (ECA)
- penicilina
- tiopronina
- interleucina-2
- nifedipino
- rifampicina
- exposição a insetos hematófagos
Diagnostic tests
1st tests to order
- biópsia de pele, coloração de hematoxilina e eosina
- biópsia de pele, imunofluorescência direta
Tests to consider
- imunofluorescência indireta em soro
- ensaio de imunoadsorção enzimática (ELISA) sérico
- endoscopia digestiva alta
- radiografia torácica
- tomografia do tórax
- testes de função pulmonar (TFPs)
- imunoblot sérico (Western Blot)
Treatment algorithm
pênfigo vulgar leve
pênfigo foliáceo leve
pênfigo vulgar ou pênfigo foliáceo moderado a grave
pênfigo paraneoplásico
pênfigo vulgar ou pênfigo foliáceo moderado a grave 6 meses após a terapia inicial: com controle da doença/remissão completa
pênfigo vulgar ou pênfigo foliáceo moderado a grave 6 meses após a terapia inicial: com remissão completa
pênfigo vulgar ou pênfigo foliáceo moderado a grave 12 a 18 meses após a terapia inicial: remissão completa
pênfigo grave/refratário
Contributors
Authors
Jon H. Meyerle, MD
Associate Professor
Department of Dermatology
Uniformed Services University of the Health Sciences
Bethesda
MD
Disclosures
JHM declares that he has no competing interests.
Grant J. Anhalt, MD
Professor
Department of Dermatology
Johns Hopkins Medical Institution
Baltimore
MD
Disclosures
GJA is a consultant for Janssen on a clinical trial design for an investigational drug for bullous pemphigoid.
Peer reviewers
Daniel Mimouni, MD
Professor
Department of Dermatology
Rabin Medical Center
Petah Tikva
Israel
Disclosures
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
Disclosures
VPR declares that she has no competing interests.
Timothy Patton, MD
Assistant Professor of Dermatology
Department of Dermatology
University of Pittsburgh
Pittsburgh
PA
Disclosures
TP declares that he has no competing interests.
Differentials
- Pênfigo benigno familiar (doença de Hailey-Hailey)
- Penfigoide bolhoso (PB)
- Dermatose linear bolhosa por imunoglobulina A (IgA)
More DifferentialsGuidelines
- Updated S2K guidelines on the management of pemphigus vulgaris and foliaceus initiated by the European Academy of Dermatology and Venereology (EADV)
- Diagnosis and management of pemphigus: recommendations by an international panel of experts
More Guidelines
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