Pemphigus

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Teoría 
Diagnóstico 
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Última revisión: 12 Dec 2025

Última actualización: 20 May 2025

Pemphigus encompasses a group of potentially life-threatening autoimmune bullous diseases characterized by blisters and erosions of the mucous membranes and skin.

Pemphigus vulgaris (PV) is the most common variant and affects skin and mucosa.

Pemphigus foliaceus (PF) is confined to the skin. Paraneoplastic pemphigus (PNP) is rare; it can involve the skin and mucosal surfaces of the eyes, mouth, nasopharynx, and esophagus.

Diagnosis is based on clinical findings of erosions (epidermal loss) and blisters of the skin, mucosa, or both.

Direct immunofluorescence staining of biopsied skin shows immunoglobulin and C3 deposits on the keratinocyte surface, and histology reveals keratinocyte acantholysis.

Treatment aims to reduce or eliminate pathologic autoantibodies and consists of long-term immunosuppressive therapy.

Definición

Pemphigus describes a group of autoimmune blistering diseases that involve the epidermal surfaces of the skin, mucosa, or both. There are three broad categories: pemphigus vulgaris (PV), pemphigus foliaceus (PF), and paraneoplastic pemphigus (PNP).

Underlying intraepithelial blister formation is caused by IgG autoantibodies against desmoglein 3 (Dsg3) and/or desmoglein 1 (Dsg1) on the cell surface of epidermal keratinocytes.[1]

Pemphigus is associated with pain and impaired sleep. Swallowing difficulties are present if there is esophageal involvement. It is associated with increased rates of depression.[2]

Anamnesis y examen

Principales factores de diagnóstico

chronic erosive blistering of the skin, mucosa, or both
chronic mouth erosions (PV, PNP)
painful lips (PNP)
shortness of breath (PNP)

Todos los datos

Otros factores de diagnóstico

pruritic scalp (PV, PF)
bloody nose (PV, PNP)
painful skin (PV, PF, PNP)
dysphagia (PV)
pruritic skin (PV, PF)
conjunctivitis (PV, PNP)

Todos los datos

Factores de riesgo

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

Todos los datos

Pruebas diagnósticas

Primeras pruebas diagnósticas para solicitar

skin biopsy, hematoxylin and eosin stain
skin biopsy, direct immunofluorescence

Todos los datos

Pruebas diagnósticas que deben considerarse

indirect immunofluorescence on serum
serum ELISA
upper gastrointestinal endoscopy
CXR
chest CT scan
PFT
serum immunoblot (Western blot)

Todos los datos

Algoritmo de tratamiento

Agudo

mild pemphigus vulgaris

mild pemphigus foliaceus

moderate to severe pemphigus vulgaris or pemphigus foliaceus

paraneoplastic pemphigus

En curso

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

Colaboradores

Autores

Jon H. Meyerle, MD

Associate Professor

Department of Dermatology

Uniformed Services University of the Health Sciences

Bethesda

Declarações

JHM declares that he has no competing interests.

Grant J. Anhalt, MD

Professor

Department of Dermatology

Johns Hopkins Medical Institution

Baltimore

Declarações

GJA declares that he has no competing interests.

Revisores

Daniel Mimouni, MD

Professor

Department of Dermatology

Rabin Medical Center

Petah Tikva

Israel

Declarações

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

Declarações

VPR declares that she has no competing interests.

Timothy Patton, MD

Assistant Professor of Dermatology

Department of Dermatology

University of Pittsburgh

Pittsburgh

Declarações

TP declares that he has no competing interests.

Créditos aos pareceristas

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Principais artigos

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.Texto completo Resumo

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.Texto completo Resumo

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.Texto completo Resumo

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. Resumo

Artigos de referência

Uma lista completa das fontes referenciadas neste tópico está disponível para os usuários com acesso total ao BMJ Best Practice.

Diagnósticos diferenciais
- Familial benign pemphigus (Hailey-Hailey disease)
- Bullous pemphigoid (BP)
- Linear IgA bullous dermatosis
Mais Diagnósticos diferenciais
Diretrizes
- 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)
Mais Diretrizes
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Pemphigus

Resumen

Definición

Anamnesis y examen

Principales factores de diagnóstico

Otros factores de diagnóstico

Factores de riesgo

Pruebas diagnósticas

Primeras pruebas diagnósticas para solicitar

Pruebas diagnósticas que deben considerarse

Algoritmo de tratamiento

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

Colaboradores

Autores

Jon H. Meyerle, MD

Declarações

Grant J. Anhalt, MD

Declarações

Revisores

Daniel Mimouni, MD

Declarações

Vesna Petronic-Rosic, MD, MSc

Declarações

Timothy Patton, MD

Declarações

Créditos aos pareceristas

Declarações

Referências

Principais artigos

Artigos de referência

Uma lista completa das fontes referenciadas neste tópico está disponível para os usuários com acesso total ao BMJ Best Practice.

Diagnósticos diferenciais

Diretrizes

Conectar-se ou assinar para acessar todo o BMJ Best Practice

Conectar-se ou assinar para acessar todo o BMJ Best Practice

Conectar-se para acessar todo o BMJ Best Practice