UVB-induced acantholysis in endemic pemphigus foliaceus (fogo selvagem) and pemphigus vulgaris

2000 ◽  
Vol 42 (4) ◽  
pp. 571-576 ◽  
Author(s):  
Vitor M.S. Reis ◽  
Roseli P. Toledo ◽  
Argelia Lopez ◽  
Luis A. Diaz ◽  
Jose E.C. Martins
Keyword(s):  
Pemphigus Vulgaris ◽  
Pemphigus Foliaceus ◽  
Endemic Pemphigus Foliaceus

scholarly journals Endemic pemphigus in the peruvian Amazon: epidemiology and risk factors for the development of complications during treatment

2012 ◽  
Vol 87 (6) ◽  
pp. 838-845 ◽  
Author(s):  
Willy Ramos ◽  
Gina Rocio Chacon ◽  
Carlos Galarza ◽  
Ericson Leonardo Gutierrez ◽  
Maria Eugenia Smith ◽  
...  
Keyword(s):  
Risk Factors ◽  
Binary Logistic Regression ◽  
Pemphigus Vulgaris ◽  
Pemphigus Foliaceus ◽  
Peruvian Amazon ◽  
Clinical Form ◽  
Autoimmune Blistering Disease ◽  
Clinical Variant ◽  
Endemic Pemphigus Foliaceus ◽  
Epidemiologic Characteristics

BACKGROUND: Pemphigus is an autoimmune blistering disease. According to a report, in areas of endemic pemphigus foliaceus (EPF) in Peru there are cases of pemphigus vulgaris with epidemiologic, clinical and histopathologic characteristics similar to those of "endemic pemphigus vulgaris" (EPV) in Brazil. OBJECTIVES: To determine the clinical and epidemiologic characteristics of endemic pemphigus and the risk factors of patients for developing complications during treatment. METHODS: A study was carried out from July 2003 to March 2008. The study population was 60 patients with EPF and 7 patients with EPV evaluated in hospitals and clinics in the Peruvian Amazon and Lima. A multivariate analysis was carried out using binary logistic regression. RESULTS: The average age of EPF patients was 31.4 years; 55% were men; 60% presented the generalized clinical variant. Non-compliance with the treatment was seen in 57.1% of the patients. Thirty-five percent presented complications (e.g. pyodermitis and pyelonephritis) during treatment. The risk factors for developing complications during treatment were non-compliance with the treatment and having the generalized clinical form. In the EPV group, the average age was 21.7 years; 71.4% were men. All patients presented with the mucocutaneous clinical variant and the initial presentation consisted of oral mucosa lesions; 71.4% presented complications during treatment, pyodermitis being the most frequent. CONCLUSIONS: Non-compliance with the treatment and the generalized clinical form are risk factors for the development of complications during treatment of patients with EPF. Peru indeed has EPV cases with epidemiologic characteristics similar to EPF. Living in a rural area may represent a risk factor for the development of complications during treatment of patients with EPV.

Anti-Desmoglein 1 and 3 Autoantibody Levels in Endemic Pemphigus Foliaceus and Pemphigus Vulgaris from Brazil

2016 ◽  
Vol 62 (07/2016) ◽  
Author(s):  
Liana Oliveira ◽  
Alfredo Marquart-Filho ◽  
Gerson Trevilato ◽  
Rodolfo Timoteo ◽  
Maira Mukai ◽  
...  
Keyword(s):  
Pemphigus Vulgaris ◽  
Pemphigus Foliaceus ◽  
Endemic Pemphigus Foliaceus

scholarly journals Endemic pemphigus foliaceus (fogo selvagem) and pemphigus vulgaris: immunoglobulin G heterogeneity detected by indirect immunofluorescence

2004 ◽  
Vol 59 (5) ◽  
pp. 251-256 ◽  
Author(s):  
Valéria Aoki ◽  
Milian H. T. Huang ◽  
Alexandre M. Périgo ◽  
Lígia M. I. Fukumori ◽  
Celina W. Maruta ◽  
...  
Keyword(s):  
Disease Activity ◽  
Indirect Immunofluorescence ◽  
Immunoglobulin G ◽  
Clinical Remission ◽  
Pemphigus Vulgaris ◽  
Pemphigus Foliaceus ◽  
Igg Subclass ◽  
Immunoglobulin G4 ◽  
Endemic Pemphigus Foliaceus ◽  
Active Disease

Pemphigus are autoimmune intraepidermal blistering diseases in which immunoglobulin G (IgG) autoantibodies are directed against desmosomal glycoproteins. The aim of this study was to determine the IgG subclass profile of endemic pemphigus foliaceus (fogo selvagem) and pemphigus vulgaris utilizing indirect immunofluorescence. PATIENTS AND METHODS: Twenty-five patients with pemphigus vulgaris, 25 with endemic pemphigus foliaceus (fogo selvagem), and 25 healthy controls were analyzed by indirect immunofluorescence for circulating autoantibodies (total IgG and its subclasses). RESULTS: Our data revealed a significant correlation (P <.05) of disease activity and autoantibody levels in both forms of pemphigus, i.e., negative titers related to clinical remission, whereas positive results related to active disease. Immunoglobulin G subclass analysis in fogo selvagem demonstrated that in patients in remission, 56% showed positive immunoglobulin G4; in active disease, immunoglobulin G4 was the predominant subclass (100% positive in all cases). The IgG subclass profile in pemphigus vulgaris showed that in patients in remission, only 10% were positive for immunoglobulin G4; in active disease, positivity for immunoglobulin G4 was present in 78% to 88% of the cases. CONCLUSION: Subclass characterization of immunoglobulin G autoantibodies is a useful tool for pemphigus follow-up, since immunoglobulin G4 (IgG4) is the subclass that is closely related to recognition of pathogenic epitopes, and consequently with disease activity. Careful monitoring should be performed for fogo selvagem in clinical remission with a homogeneous IgG4 response, since this may indicate more frequent relapses.

scholarly journals A Hypothesis Concerning a Potential Involvement of Ceramide in Apoptosis and Acantholysis Induced by Pemphigus Autoantibodies

2010 ◽  
Vol 2010 ◽  
pp. 1-8
Author(s):  
Wendy B. Bollag
Keyword(s):  
Autoimmune Diseases ◽  
Ultraviolet Irradiation ◽  
Cell Adhesion Molecule ◽  
Sun Exposure ◽  
Pemphigus Vulgaris ◽  
Skin Lesions ◽  
Epidermal Keratinocytes ◽  
Pemphigus Foliaceus ◽  
Endemic Pemphigus Foliaceus ◽  
A Cell

Autoimmune diseases affect more than 50 million Americans, resulting in significant healthcare costs. Most autoimmune diseases occur sporadically; however, endemic pemphigus foliaceus (EPF) is an autoimmune skin disease localized to specific geographic loci. EPF, and the related diseases pemphigus vulgaris (PV) and pemphigus foliaceus (PF), are characterized by skin lesions and autoantibodies to molecules found on epidermal keratinocytes. A variant of EPF in patients from El Bagre, Colombia, South America, has recently been reported to be distinct from previously described loci in Brazil and Tunisia epidemiologically and immunologically. As in PF and EPF, El Bagre EPF patients exhibit autoantibodies towards desmoglein-1, a cell adhesion molecule critical for maintaining epidermal integrity. An association of El Bagre EPF with sun exposure has been detected, and ultraviolet irradiation also exacerbates symptoms in PV, PF and EPF. Our hypothesis is that: (1) the autoantibodies generate pathology through an alteration in ceramide metabolism in targeted keratinocytes, resulting in apoptosis and/or cell death and acantholysis, but only when the cell's ability to metabolize ceramide is exceeded, and (2) apoptosis in response to this altered ceramide metabolism is initiated and/or exacerbated by other agents that increase ceramide levels, such as cytokines, ultraviolet irradiation, and senescence.

Drug-induced pemphigus foliaceus with features of pemphigus vulgaris

2001 ◽  
Vol 144 (2) ◽  
pp. 421-422 ◽  
Author(s):  
K. Ogata ◽  
H. Nakajima ◽  
M. Ikeda ◽  
Y. Yamamoto ◽  
M. Amagai ◽  
...  
Keyword(s):  
Pemphigus Vulgaris ◽  
Pemphigus Foliaceus ◽  
Drug Induced
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