Endemic pemphigus foliaceus is an autoimmune disease that has remarkable features. Endemic foci are found in characteristic environments within the interior of Brazil. The epidemiologic data strongly suggest that an environmental factor initiates the autoantibody response in the host. As such it is an important disease for in-depth study. A group of interested investigators in both Brazil and the United States has been formed to attempt to do just that. As part of the overall effort, this Cooperative Research Group for the Study of Fogo Selvagem presents a definition of the disease, a proposed clinical classification for various forms of the disease, and an outline of what is currently known of its immunopathologic characteristics.

Endemic pemphigus foliaceus (fogo seivagem). I. Clinical features and immunopathology

Serologic abnormalities in patients with endemic pemphigus foliaceus (Fogo selvagem), their relatives, and normal donors from endemic and non-endemic areas of Brazil.

Background: Autoimmune blistering diseases are a group of bullous disorders characterized by pathogenic antibodies directed at the target antigens, which are components of the desmosomes or adhesion complex at the dermoepidermal junction. Direct immunofluorescence (DIF) is invaluable in the diagnosis of these lesions. Aim: The aim of this study was to evaluate the sensitivity of DIF in immunobullous dermatoses and to study the pattern of DIF. The study also aims to correlate DIF with clinical and histologic findings and to analyze discrepancies. Materials and Methods: Total 100 biopsies received over a period of 2 years in the Department of Pathology were analyzed. DIF, histopathology and clinical data were reviewed. Results: Out of 100, 89 cases showed DIF patterns concordant with clinical/histologic diagnosis. The sensitivity of DIF was 94.44% (51/58) in the pemphigus and 84% (21/25) in the bullous pemphigoid (BP) group, 100% each in dermatitis herpetiformis (2/2) and linear IgA disease (1/1). A total of 11 histologically proven cases of immunobullous lesions were DIF negative-four (three of pemphigus vulgaris and one of BP) due to having no epidermis, three (cases of BP) owing to sampling/technical errors and the remaining four (cases of pemphigus vulgaris) due to being on treatment. Conclusion: Immunofluorescence helps confirm the diagnosis of bullous lesions in which there is clinical and the histopathologic overlap. Sampling errors contributed to false negative (FN) results.

A Cross-sectional Study of Direct Immunofluorescence in the Diagnosis of Immunobullous Dermatoses.

Pemphigus erythematosus, initially described as a combination of pemphigus with lupus erythematosus, and pemphigus foliaceus are now frequently considered localized and generalized variants of superficial pemphigus. Yet diagnostic criteria for pemphigus erythematosus remain controversial. Distinct from pemphigus foliaceus, pemphigus erythematosus displays immune depositions at the dermal-epidermal junction, which suggests additional immunopathological mechanisms. We present three patients with clinical and histopathologic signs of superficial pemphigus, who all exhibited an immunomorphology characteristic of pemphigus erythematosus. Complement depositions in a granular-linear fashion were consistently found at the dermal-epidermal junction besides in vivo bound and circulating antikeratinocyte cell-surface autoantibodies. Histopathology showed subcorneal acantholysis, and all sera contained antidesmoglein 1 but not antidesmoglein 3 autoantibodies detected by enzyme-linked immunosorbent assays (ELISA). Additional autoantibodies against a 230-kDa protein and against a 190-kDa protein comigrating with bullous pemphigoid antigen 1 (BP230) and periplakin, respectively, were present in all the patients' sera. As two sera specifically reacted with BP230 by ELISA, the presence of BP230-specific autoantibodies could be associated with dermal-epidermal immune staining in these patients. In pemphigus erythematosus, dermal-epidermal immune staining is generally attributed to the deposition of immune complexes, while the presence of BP230-specific autoantibodies has not been reported in this disease previously. Perhaps, the unique autoantibody profile of the patients in the study permits discrimination between patients with superficial pemphigus that display additional dermal-epidermal immune staining from those with conventional pemphigus foliaceus on a molecular basis. Further studies will be required to substantiate the frequency of this occurrence and to unravel its pathogenic significance.

230-kDa and 190-kDa proteins in addition to desmoglein 1 as immunological targets in a subset of pemphigus foliaceus with a combined cell-surface and basement membrane zone immune staining pattern.

The aim of the present study was to investigate a broad spectrum of autoantibodies in patients with endemic pemphigus foliaceus (EPF)—fogo selvagem—and to determine the possible association between EPF and other autoimmune diseases. Indirect immunofluorescence was used to test 120 patients with EPF and 200 healthy controls for the presence of the following autoantibodies: anti-desmoglein-1 (APF), anti-neutrophil cytoplasmic (ANCA), anti-smooth muscle (SMA), anti-mitochondrial (AMA), anti-nuclear (ANA), anti-liver kidney microsomal (LKM), anti-gastric parietal cells (GPCA) and anti-thyroid microsome (TMA). APF antibodies were detected in 62.5% of the patients (75/120), ANA and SMA in 0.8% (1/120), and TMA in 1.6% (2/120). None of the patients was positive for ANCA, AMA, LKM or GPCA. In the control group, a positivity of 2% was observed for SMA (4/200), 1.5% for TMA (3/200), and 0.5% (1/200) for ANA and GPCA. None of the controls was positive for APF, LKM, AMA or ANCA. The prevalence of the autoantibodies ANA, SMA, AMA, GPCA, LKM and ANCA in patients with EPF was similar to that observed in the control group. No association with clinical or laboratory manifestations of other concomitant autoimmune diseases was observed in EPF patients. These results confirm the concept that EPF is an organ-specific autoimmune disease.

Prevalence of autoantibodies in patients with endemic pemphigus foliaceus (fogo selvagem).

The two variants of superficial pemphigus, pemphigus erythematosus (Senear-Usher syndrome) and pemphigus foliaceus, share common histopathologic and indirect immunofluorescence findings. They differ in that pemphigus erythematosus is limited in distribution and usually has concomitant basement membrane zone deposition of immunoglobulin and complement in lesional skin in addition to intercellular space staining in the epidermis, whereas pemphigus foliaceus is a generalized process that reputedly lacks basement membrane zone staining. A 49-year-old man with an array of immunologic abnormalities, including pernicious anemia and Sjogren's syndrome consistent with broad immunologic dysfunction, developed generalized superficial pemphigus (pemphigus foliaceus) with concomitant basement membrane zone immunoreactant deposition. The demonstration of basement zone immunofluorescent staining lends additional support to the basic viewpoint that pemphigus erythematosus and pemphigus foliaceus are variable phenotypic expressions of the same basic disease process.

https://www.jaad.org/article/S0190-9622(82)70154-9/pdf

Pemphigus foliaceus: A case with serologic features of Senear-Usher syndrome and other autoimmune abnormalities

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Pemphigus foliaceus: A case with serologic features of Senear-Usher syndrome and other autoimmune abnormalities