Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2011 Jul;29(3):405-12, viii.
doi: 10.1016/j.det.2011.03.012.

Diagnosis and clinical features of pemphigus foliaceus

Kirk A James  1 , Donna A Culton, Luis A Diaz
Affiliations
Review

Diagnosis and clinical features of pemphigus foliaceus

Kirk A James et al. Dermatol Clin. 2011 Jul.

Abstract

Pemphigus foliaceus is an acquired autoimmune blistering disease in which the body's immune system produces IgG autoantibodies that target the intercellular adhesion glycoprotein desmoglein-1, which is principally expressed in the granular layer of the epidermis, resulting in the loss of intercellular connections between keratinocytes (acantholysis) and the formation of subcorneal blisters within the epidermis. This article summarizes the epidemiology, clinical features, techniques for diagnosis, and drugs associated with treatment of this rare disease.

PubMed Disclaimer

Conflict of interest statement

Financial Disclosure/Conflict of Interest Statement:

The authors of this paper have no financial disclosures or conflicts of interest to express.

Figures

Fig 1
Fig 1
Intact blister filled with inflammatory exudate on left side of chest.
Fig 2
Fig 2
Isolated scaly, erythematous plaque with peripheral erosion on left cheek.
Fig 3
Fig 3
Disseminated papulosquamous eruption on back evolving from the preceding superficial blisters and secondary erosions of PF.
Fig 4
Fig 4
Severe exfoliative erythroderma.
Fig 5
Fig 5
Biopsy of PF lesion. Subcorneal blister formation due to acantholysis of keratinocytes.
Fig 6
Fig 6
Positive indirect immunofluorescent staining using PF patient serum on human skin substrate. This staining pattern directed against the intercellular space (ICS) is characteristic of pemphigus.
See this image and copyright information in PMC

References

    1. Rock B, Martins CR, Theofilopoulos AN, et al. The pathogenic effect of IgG4 autoantibodies in endemic pemphigus foliaceus (fogo selvagem) N Engl J Med. 1989;320:1463–1469. - PubMed
    1. Sekiguchi M, Futei Y, Fujii Y. Dominant autoimmune epitopes recognized by pemphigus antibodies map to the N-terminal adhesive region of desmogleins. J Immunol. 2001;167:5439–5448. - PubMed
    1. Li N, Aoki V, Hans-Filho G, et al. The role of intramolecular epitope spreading in the pathogenesis of endemic pemphigus foliaceus (fogo selvagem) J Exp Med. 2003;197:1501–1510. - PMC - PubMed
    1. Rubenstein DS, Diaz LA. Pemphigus antibody induced phosphorylation of keratinocyte proteins. Autoimmunity. 2006;39:577–586. - PubMed
    1. Berkowitz P, Chua M, Liu Z, et al. Autoantibodies in the autoimmune disease pemphigus foliaceus induce blistering via p38 mitogen-activated protein kinase-dependent signaling in the skin. Am J Pathol. 2008;173:1628–1636. - PMC - PubMed