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Luis A Diaz - One of the best experts on this subject based on the ideXlab platform.
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childhood Pemphigus foliaceus presenting as a polycyclic eruption case report and review of the literature
Pediatric Dermatology, 2019Co-Authors: Megan Evans, Luis A Diaz, Donna A. Culton, Paul B Googe, Dean S MorrellAbstract:Pemphigus foliaceus (PF) is an autoimmune bullous disorder that has occasionally been reported to present as a polycyclic or arcuate eruption in children. We present a case of childhood PF presenting as an annular and polycyclic eruption, which initially led to a diagnostic conundrum and a delay in diagnosis but which ultimately responded well to therapy with systemic steroids and rituximab infusions. We briefly review the literature on polycyclic presentations of PF in childhood as well as the use of rituximab for pediatric Pemphigus.
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IgG Autoantibody Response Against Keratinocyte Cadherins in Endemic Pemphigus foliaceus (Fogo Selvagem)
2016Co-Authors: Gustavo Flores, Gunter Hans-filho, Donna A. Culton, Phillip Prisayanh, Bahjat F. Qaqish, Mike Maldonado, Kirk James, Ro A. Rivitti, Luis A DiazAbstract:It is well established that autoantibodies against desmoglein 3 and desmoglein 1 are relevant in the pathogenesis of Pemphigus vulgaris and Pemphigus foliaceus, including its endemic form, Fogo Selvagem (FS). Isolated reports have shown that in certain patients with these diseases, autoantibodies against other desmosomal cadherins and E-cadherin may also be present. The goal of this investigation was to determine if FS patients and normal individuals living in endemic areas possess autoantibodies against other desmosomal cadherins and E-cadherin. Testing a large number of FS and endemic control sera by ELISA we find a consistent and specific autoantibody response against desmoglein 1 and other keratinocyte cadherins in these individuals, which is quite different from US controls. Overall, the highest correlations among the autoantibody responses tested are in the endemic controls, followed by FS patients, and lowest in the US controls. These findings suggest that multiple, perhaps cross reactive, keratinocyte cadherins are recognized by FS patients and endemic controls
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Update on fogo selvagem, an endemic form of Pemphigus foliaceus
The Journal of dermatology, 2015Co-Authors: Evandro A. Rivitti, Luis A DiazAbstract:Pemphigus are organ-specific autoimmune diseases, where autoantibodies (mainly immunoglobulin [Ig]G) directed against epidermal targets (glycoproteins of the desmosomal core) are detected. Endemic Pemphigus foliaceus or fogo selvagem (FS) is one of the variants of Pemphigus foliaceus Pemphigus foliaceus that shares the same clinical and immunopathological features of the classic non-endemic Pemphigus foliaceus form, including pathogenic IgG (mainly IgG4) autoantibodies directed against the ectodomain of desmoglein 1 (Dsg1), that lead to acantholysis. Pathogenesis of FS is complex, involving genetic, environmental and immunological factors. Human leukocyte antigen (HLA)-DRB1 alleles DRB1*0404, *1402, *1406 or *0102 have been previously identified as risk factors for FS (relative risk, >14). Individuals exposed to hematophagous insects are more susceptible to develop the disease. Non-pathogenic anti-Dsg1 antibodies of the IgG1 subclass, directed against the extracellular 5 domain of Dsg1, are detected in patients in the preclinical stage of the disease, and also in healthy controls living in endemic areas. In counterpart, patients with FS show pathogenic anti-Dsg1 IgG4 autoantibodies that bind the pathogenic extracellular 1 and 2 domains of Dsg1, emphasizing the intramolecular epitope-spreading hypothesis. A possible explanation for the development of the autoimmune process would be antigenic mimicry, initiated by environmental stimuli in those genetically predisposed individuals. Characterization of the pathogenesis of FS will allow the development of specific therapeutic targets, and the elucidation of other autoimmune processes.
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Pemphigus foliaceus and endemic forms
2015Co-Authors: Kirk James, Valeria Aoki, Luis A DiazAbstract:Pemphigus foliaceus can be divided into a non-endemic and endemic form. Both types share similar clinical features but differ in age of onset, geographic correlation, and familial incidence. The initial formation of fragile superficial blisters on the skin is quickly followed by the development of erosions with variable degrees of scaling and crusting. The severity of disease ranges from mild and localized to fulminant and widespread.
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IgG autoantibody response against keratinocyte cadherins in endemic Pemphigus foliaceus (fogo selvagem).
The Journal of investigative dermatology, 2012Co-Authors: Gustavo Flores, Gunter Hans-filho, Evandro A. Rivitti, Donna A. Culton, Phillip Prisayanh, Bahjat F. Qaqish, Kirk A. James, Mike Maldonado, Luis A DiazAbstract:It is well established that autoantibodies against desmoglein 3 and desmoglein 1 (Dsg1) are relevant in the pathogenesis of Pemphigus vulgaris and Pemphigus foliaceus, including its endemic form fogo selvagem (FS). Isolated reports have shown that in certain patients with these diseases, autoantibodies against other desmosomal cadherins and E-cadherin may also be present. The goal of this investigation was to determine whether FS patients and normal individuals living in endemic areas possess autoantibodies against other desmosomal cadherins and E-cadherin. By testing a large number of FS and endemic control sera by ELISA, we found a consistent and specific autoantibody response against Dsg1 and other keratinocyte cadherins in these individuals, which is quite different from healthy individuals from the United States (US controls). Overall, the highest correlations among the autoantibody responses tested were in the endemic controls, followed by FS patients, and lowest in the US controls. These findings suggest that multiple, perhaps cross-reactive, keratinocyte cadherins are recognized by FS patients and endemic controls.
Evandro A. Rivitti - One of the best experts on this subject based on the ideXlab platform.
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Update on fogo selvagem, an endemic form of Pemphigus foliaceus
The Journal of dermatology, 2015Co-Authors: Evandro A. Rivitti, Luis A DiazAbstract:Pemphigus are organ-specific autoimmune diseases, where autoantibodies (mainly immunoglobulin [Ig]G) directed against epidermal targets (glycoproteins of the desmosomal core) are detected. Endemic Pemphigus foliaceus or fogo selvagem (FS) is one of the variants of Pemphigus foliaceus Pemphigus foliaceus that shares the same clinical and immunopathological features of the classic non-endemic Pemphigus foliaceus form, including pathogenic IgG (mainly IgG4) autoantibodies directed against the ectodomain of desmoglein 1 (Dsg1), that lead to acantholysis. Pathogenesis of FS is complex, involving genetic, environmental and immunological factors. Human leukocyte antigen (HLA)-DRB1 alleles DRB1*0404, *1402, *1406 or *0102 have been previously identified as risk factors for FS (relative risk, >14). Individuals exposed to hematophagous insects are more susceptible to develop the disease. Non-pathogenic anti-Dsg1 antibodies of the IgG1 subclass, directed against the extracellular 5 domain of Dsg1, are detected in patients in the preclinical stage of the disease, and also in healthy controls living in endemic areas. In counterpart, patients with FS show pathogenic anti-Dsg1 IgG4 autoantibodies that bind the pathogenic extracellular 1 and 2 domains of Dsg1, emphasizing the intramolecular epitope-spreading hypothesis. A possible explanation for the development of the autoimmune process would be antigenic mimicry, initiated by environmental stimuli in those genetically predisposed individuals. Characterization of the pathogenesis of FS will allow the development of specific therapeutic targets, and the elucidation of other autoimmune processes.
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IgG autoantibody response against keratinocyte cadherins in endemic Pemphigus foliaceus (fogo selvagem).
The Journal of investigative dermatology, 2012Co-Authors: Gustavo Flores, Gunter Hans-filho, Evandro A. Rivitti, Donna A. Culton, Phillip Prisayanh, Bahjat F. Qaqish, Kirk A. James, Mike Maldonado, Luis A DiazAbstract:It is well established that autoantibodies against desmoglein 3 and desmoglein 1 (Dsg1) are relevant in the pathogenesis of Pemphigus vulgaris and Pemphigus foliaceus, including its endemic form fogo selvagem (FS). Isolated reports have shown that in certain patients with these diseases, autoantibodies against other desmosomal cadherins and E-cadherin may also be present. The goal of this investigation was to determine whether FS patients and normal individuals living in endemic areas possess autoantibodies against other desmosomal cadherins and E-cadherin. By testing a large number of FS and endemic control sera by ELISA, we found a consistent and specific autoantibody response against Dsg1 and other keratinocyte cadherins in these individuals, which is quite different from healthy individuals from the United States (US controls). Overall, the highest correlations among the autoantibody responses tested were in the endemic controls, followed by FS patients, and lowest in the US controls. These findings suggest that multiple, perhaps cross-reactive, keratinocyte cadherins are recognized by FS patients and endemic controls.
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The Prevalence of Antibodies against Desmoglein 1 in Endemic Pemphigus foliaceus in Brazil
The New England journal of medicine, 2000Co-Authors: Simon Warren, Vandir Dos Santos, George J. Giudice, Mong Shang Lin, Raymond G. Hoffmann, Gunter Hans-filho, Evandro A. Rivitti, Luis A DiazAbstract:Background Pemphigus foliaceus is an autoimmune skin disease mediated by autoantibodies against desmoglein 1. The endemic form is thought to have an environmental cause. The Terena reservation of Limao Verde in Mato Grosso do Sul, Brazil, is a recently identified focus of the disease, with a prevalence of 3.4 percent in the population. We tested the hypothesis that normal subjects living in an endemic area have antibodies against desmoglein 1. Methods We used an enzyme-linked immunosorbent assay to detect antibodies against desmoglein 1 in serum samples from 60 patients with endemic Pemphigus foliaceus (fogo selvagem) who lived in Limao Verde or elsewhere in Brazil, 372 normal subjects (without Pemphigus foliaceus) from Limao Verde and surrounding locations, and 126 normal subjects from the United States and Japan. Results Antibodies against desmoglein 1 were detected in 59 of the 60 patients with fogo selvagem (98 percent) but in only 3 of the 126 normal subjects from the United States and Japan (2 perce...
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endemic Pemphigus foliaceus fogo selvagem 1998
Clinics in Dermatology, 1999Co-Authors: Gunter Hansfilho, Donald P Eaton, Mong Shang Lin, Evandro A. Rivitti, Luis A DiazAbstract:The clinical, histological and immunological features of the endemic form of Pemphigus foliaceus (PF), also known as fogo selvagem (FS), are similar to those patients suffering the nonendemic form of PF seen in North America, Europe, and other parts of the world as originally described by Cazenave.1–3 FS is an autoimmune disease that has remarkable features. The disease is characterized by superficial subcorneal blisters and pathogenic antiepidermal autoantibodies that recognize the desmosomal core glycoprotein known as desmoglein 1 (dsg1).4 In addition, FS possesses unique epidemiological features, such as a high prevalence of the disease in certain regions of Brazil, an increased frequency of familial cases, and an increased frequency of cases among young adults and children. Commonly, FS patients are poor farmers who live and work in certain rural areas of Brazil where the disease is endemic. Recently, other endemic foci of PF have been reported in other South American countries such as Columbia and Tunisia.5–8 Several etiological agents are thought to precipitate FS; however, there is no convincing experimental evidence supporting any of these hypotheses. The disease is treated with systemic steroids, and in certain cases, with immunosuppressive agents. The prognosis of FS has improved with the use of these new therapies.
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Pemphigus herpetiformis is a rare clinical expression of nonendemic Pemphigus foliaceus, fogo selvagem, and Pemphigus vulgaris
Journal of the American Academy of Dermatology, 1996Co-Authors: Claudia Giuli Santi, Valeria Aoki, Celina Wakisaka Maruta, Evandro A. Rivitti, Mirian Nacagami Sotto, Luis A DiazAbstract:Abstract Background: Pemphigus herpetiformis is a rare and atypical variant of Pemphigus that resembles dermatitis herpetiformis. Most patients show antiepidermal autoantibodies that stain the epidermal intercellular spaces by immunofluorescence, similar to Pemphigus autoantibodies, and lack the immunopathologic features of dermatitis herpetiformis. Objective: The study was aimed at characterizing the specificity of the antiepidermal autoantibodies in seven patients with Pemphigus herpetiformis. Methods: The antiepidermal autoantibodies were characterized by immunofluorescence, immunoblotting, and immunoprecipitation studies in seven patients who fulfilled the clinical, histologic, and immunofluorescence diagnostic criteria for Pemphigus herpetiformis. Results: Five patients with features of Pemphigus herpetiformis either had classic Pemphigus foliaceus, or their disease evolved into classic Pemphigus foliaceus. One of these patients had fogo selvagem. Two of the seven patients showed features of or had disease that evolved into Pemphigus vulgaris. The antiepidermal autoantibodies present in all seven patients recognized desmoglein 1. Conclusion: Pemphigus herpetiformis is a rare clinical and histologic expression of nonendemic Pemphigus foliaceus, fogo selvagem, and Pemphigus vulgaris.
Mong Shang Lin - One of the best experts on this subject based on the ideXlab platform.
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A Subset of Pemphigus foliaceus Patients Exhibits Pathogenic Autoantibodies Against Both Desmoglein-1 and Desmoglein-3
The Journal of investigative dermatology, 2002Co-Authors: Luis A Arteaga, Luis A Diaz, Simon Warren, Zhi Liu, Philip S. Prisayanh, Mong Shang LinAbstract:In Pemphigus vulgaris the major pathogenic antibody binds desmoglein-3, and mediates mucosal disease. Development of cutaneous disease is associated with acquisition of antibodies to desmoglein-1. In Pemphigus foliaceus, and its endemic form, fogo selvagem by contrast, the major pathogenic antibody recognizes desmoglein-1 and mediates cutaneous disease only. In this study, we sought to determine the prevalence of antibodies to desmoglein-3 in patients with Pemphigus foliaceus and fogo selvagem. We produced recombinant desmoglein-1 and desmoglein-3, and used them in highly sensitive and specific enzyme-linked immunosorbent assays, as well as immunoprecipitation assays. We detected antibodies to desmoglein-3 in 19 of 276 patients with Pemphigus foliaceus and fogo selvagem, who had cutaneous disease only. We showed that these antibodies to desmoglein-3 could be absorbed in a concentration-dependent manner by desmoglein-3 but not by desmoglein-1. Also antibodies to desmoglein-1 could be absorbed in a concentration-dependent manner by desmoglein-1 but not desmoglein-3. This suggests that two separate species of antibody are present rather than one antibody capable of cross-reacting with both desmoglein-1 and desmoglein-3. Finally, it was shown that affinity-purified antibodies to desmoglein-3 from patients with Pemphigus foliaceus and fogo selvagem induced a Pemphigus vulgaris-like skin disease in mice by passive transfer. These results suggest that a subset of patients with Pemphigus foliaceus and fogo selvagem have antibodies to desmoglein-3 that may be involved in the pathogenesis of their cutaneous disease.
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The Prevalence of Antibodies against Desmoglein 1 in Endemic Pemphigus foliaceus in Brazil
The New England journal of medicine, 2000Co-Authors: Simon Warren, Vandir Dos Santos, George J. Giudice, Mong Shang Lin, Raymond G. Hoffmann, Gunter Hans-filho, Evandro A. Rivitti, Luis A DiazAbstract:Background Pemphigus foliaceus is an autoimmune skin disease mediated by autoantibodies against desmoglein 1. The endemic form is thought to have an environmental cause. The Terena reservation of Limao Verde in Mato Grosso do Sul, Brazil, is a recently identified focus of the disease, with a prevalence of 3.4 percent in the population. We tested the hypothesis that normal subjects living in an endemic area have antibodies against desmoglein 1. Methods We used an enzyme-linked immunosorbent assay to detect antibodies against desmoglein 1 in serum samples from 60 patients with endemic Pemphigus foliaceus (fogo selvagem) who lived in Limao Verde or elsewhere in Brazil, 372 normal subjects (without Pemphigus foliaceus) from Limao Verde and surrounding locations, and 126 normal subjects from the United States and Japan. Results Antibodies against desmoglein 1 were detected in 59 of the 60 patients with fogo selvagem (98 percent) but in only 3 of the 126 normal subjects from the United States and Japan (2 perce...
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endemic Pemphigus foliaceus fogo selvagem 1998
Clinics in Dermatology, 1999Co-Authors: Gunter Hansfilho, Donald P Eaton, Mong Shang Lin, Evandro A. Rivitti, Luis A DiazAbstract:The clinical, histological and immunological features of the endemic form of Pemphigus foliaceus (PF), also known as fogo selvagem (FS), are similar to those patients suffering the nonendemic form of PF seen in North America, Europe, and other parts of the world as originally described by Cazenave.1–3 FS is an autoimmune disease that has remarkable features. The disease is characterized by superficial subcorneal blisters and pathogenic antiepidermal autoantibodies that recognize the desmosomal core glycoprotein known as desmoglein 1 (dsg1).4 In addition, FS possesses unique epidemiological features, such as a high prevalence of the disease in certain regions of Brazil, an increased frequency of familial cases, and an increased frequency of cases among young adults and children. Commonly, FS patients are poor farmers who live and work in certain rural areas of Brazil where the disease is endemic. Recently, other endemic foci of PF have been reported in other South American countries such as Columbia and Tunisia.5–8 Several etiological agents are thought to precipitate FS; however, there is no convincing experimental evidence supporting any of these hypotheses. The disease is treated with systemic steroids, and in certain cases, with immunosuppressive agents. The prognosis of FS has improved with the use of these new therapies.
Cassian Sitaru - One of the best experts on this subject based on the ideXlab platform.
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prediction of survival for patients with Pemphigus vulgaris and Pemphigus foliaceus a retrospective cohort study
Orphanet Journal of Rare Diseases, 2015Co-Authors: Adrian Baican, Roxana Chiorean, Daniel Corneliu Leucuta, Corina Baican, Sorina Danescu, Dorina Ciuce, Cassian SitaruAbstract:Factors associated with survival in Pemphigus have not yet been thoroughly addressed. Therefore, in the present study, risk factors for overall mortality in a large group of patients with Pemphigus vulgaris and foliaceus were investigated. A retrospective hospital-based cohort study was carried out, between October 1998 and November 2012, in the Department of Dermatology of the University of Medicine and Pharmacy “Iuliu Hatieganu”, Cluj-Napoca, Romania. The investigated prognostic endpoint was the overall survival of the patients. A total of 130 patients were studied (108 with Pemphigus vulgaris and 22 with Pemphigus foliaceus). In Pemphigus vulgaris group, univariate analysis found a statistically significant association between the age of onset ≥ 65 years (p < 0.001), presence of coronary heart disease (p = 0.006), presence of cardiac arrhythmia (p = 0.004), level of anti-desmoglein1 autoantibodies ≥ 100 U/mL (p = 0.047) at diagnosis and the survival of the patients. An age-adjusted analysis showed significant results for coronary heart disease. Multivariate analysis identified the age of onset ≥ 65 years and the presence of coronary heart disease at diagnosis as independent risk factors associated with overall mortality. In patients with Pemphigus foliaceus, age of onset ≥ 65 years (p = 0.021) was associated with poor survival. In addition to common prognostic factors, including older age and cardiovascular comorbidities, level of autoantibodies was found to be a disease-specific factor associated with overall mortality in Pemphigus vulgaris. The newly identified factors have major implications for the stratification of patients and should greatly facilitate further epidemiological studies in Pemphigus. In addition, they provide useful information for the design of personalized therapeutic plans in the clinical setting.
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transition from Pemphigus foliaceus to bullous pemphigoid intermolecular b cell epitope spreading without igg subclass shifting
Journal of The American Academy of Dermatology, 2009Co-Authors: Andreas Recke, Detlef Zillikens, Cassian Sitaru, Christian Rose, Enno Schmidt, Evabettina BrockerAbstract:Pemphigus and the pemphigoid group of diseases are distinct autoimmune conditions in which autoantibodies with different specificities cause skin blistering by different mechanisms. Transitions and associations between these two groups of autoimmune diseases are rare. Here, we report a patient with long-standing Pemphigus foliaceus, in whom clinical remission was eventually induced. Shortly thereafter, he developed the clinical, histologic, and immunopathological changes of bullous pemphigoid. This case offered the rare opportunity to serologically monitor serum levels of both anti-BP180 and BP230 during the preclinical stage of bullous pemphigoid. Of interest, although the autoimmune response clearly shifted with regard to the target antigens, the patient's autoantibodies against desmosomal and hemidesmosomal components showed the same IgG subclass distribution.
O. Abida - One of the best experts on this subject based on the ideXlab platform.
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Autoimmune reactivity against precursor form of desmoglein 1 in healthy Tunisians in the area of endemic Pemphigus foliaceus.
Journal of dermatological science, 2013Co-Authors: Amina Toumi, O. Abida, Abderrahmen Masmoudi, Hamida Turki, S. Makni, Marwah A. Saleh, Jun Yamagami, Maryem Kallel, Takahisa Hachiya, Keiko KurodaAbstract:Background Desmoglein 1 (Dsg1), the Pemphigus foliaceus (PF) antigen, is produced as a precursor (preDsg1) and is transported to the cell surface as the mature form (matDsg1). Recent studies show that B cells from North American individuals without Pemphigus can potentially produce anti-preDsg1 IgG antibodies, but ELISA screening of large numbers of normal people in North America and Japan hardly ever shows circulating antibodies against preDsg1 or matDsg1. In contrast, in Tunisia, where PF is endemic, anti-Dsg1 IgGs are frequently detected in healthy individuals.
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tunisian endemic Pemphigus foliaceus is associated with the hla dr3 gene anti desmoglein 1 antibody positive healthy subjects bear protective alleles
British Journal of Dermatology, 2009Co-Authors: O. Abida, M. Zitouni, M. Mokni, B. Fezzaa, M Kallelsellami, Nadia Mahfoudh, Arwa Kammoun, Ben M Ayed, A Masmoudi, Ben A OsmanAbstract:Summary Background Pemphigus foliaceus is an autoimmune blistering skin disease that partly results from genetic factors, especially human leucocyte antigen (HLA) class II genes. Objectives The aim of the study was to determine the HLA DR/DQ markers of susceptibility and protection in the Tunisian endemic form. Methods Genomic DNA from 90 patients with Pemphigus foliaceus recruited from all parts of the country and matched by age, sex and geographical origin with 270 healthy individuals, was genotyped. Results Firstly, when the whole patient population was studied, DRB1*03, DQB1*0302 and DRB1*04 alleles were significantly associated with the disease while a significant decrease of, in particular, DRB1*11 and DQB1*0301 was observed in patients compared with controls. DRB1*0301 was the dominant allele in DR3-positive patients and controls, while DRB1*0402 was found in 42% of DR4-positive patients. Secondly, when the HLA DR/DQ allele distribution was studied after dividing patients according to their geographical origin, the southern group, which consisted exclusively of patients with the endemic form of the disease, showed the same associations as the whole Pemphigus foliaceus population, particularly with DRB1*03. In the northern group, only the DRB1*04 and DQB1*0301 alleles were found to be associated. Interestingly, anti-desmoglein 1 antibody-positive healthy controls did not carry susceptibility alleles but, in contrast, most carried negatively associated alleles. Conclusions These observations indicate that a particular genetic background characterizes the Tunisian endemic form of Pemphigus foliaceus and that HLA class II genes control the pathogenic properties of the autoimmune response rather than the initial breakage of B-cell tolerance.
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Anti-desmoglein 1 antibodies in healthy related and unrelated subjects and patients with Pemphigus foliaceus in endemic and non-endemic areas from Tunisia.
Journal of the European Academy of Dermatology and Venereology : JEADV, 2009Co-Authors: O. Abida, M. Kallel-sellami, Pascal Joly, M. Ben Ayed, M. Zitouni, Abderrahmen Masmoudi, M. Mokni, B. Fezzaa, A. Ben Osman, KammounAbstract:Background Pemphigus foliaceus is an autoimmune blistering skin disease characterized by the production of pathogenic IgG autoantibodies directed against desmoglein 1. Aim To determine the prevalence of anti-desmoglein 1 antibodies in healthy subjects and their distribution in the different regions of Tunisia and to better identify endemic areas of Pemphigus foliaceus. Methods We tested, by enzyme-linked immunoserbent assay, sera of 270 normal subjects recruited from different Tunisian areas and 203 related healthy relatives to 90 Tunisian Pemphigus foliaceus patients. Results Seventy-six patients (84.4%), 20 healthy controls (7.4%), and 32 relatives (15.76%) had anti-desmoglein 1 antibodies. In southern regions where Pemphigus foliaceus is associated with a significant sex ratio imbalance (9 female : 1 male in the south vs. 2.3 : 1 in the north) and a lower mean age of disease onset (33.5 in the south vs. 45 years in the north), a higher prevalence of anti-desmoglein 1 antibodies in healthy controls was observed (9.23% vs. 5.71% in the north). Interestingly, the highest prevalence of anti-desmoglein 1 antibodies in healthy relatives (up to 22%) was observed in the most rural southern localities. More than half anti-desmoglein 1–positive healthy controls were living in rural conditions with farming as occupation, which suggests that this activity may expose the subjects to particular environmental conditions. Conclusion These results show that the endemic features of Tunisian Pemphigus foliaceus are focused in these southern areas more than in other areas and that both environmental and genetic factors contribute to the disease. Conflicts of interest None declared.