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* Context.--Pemphigus is an autoimmune blister disease that causes blisters on the skin and mucosal surfaces. Direct immunofluorescence (DIF) testing is critical for the clinical diagnosis of pemphigus. However, it is limited to fresh tissue specimens and fluorescence microscopy. Objective.--To assess the value of C3d immunohistochemistry (IHC) on paraffin-embedded skin tissue for the diagnosis of pemphigus by comparing C3d-IHC results to DIF and enzyme-linked immunosorbent assay testing in pemphigus and other blister-related skin diseases. Design.--C3d-IHC assays were retrospectively performed on paraffin-embedded skin tissue sections from 115 patients (63 with pemphigus and 52 controls). Both the case group and the control group underwent the same protocol, and cases with C3d position in the peripheral spinous layer were considered as positive samples. Results.--C3d-IHC and DIF testing had similar performance for pemphigus diagnosis, with a sensitivity of 71.0% (95% CI, 51.8%-85.1 %) and 77.4% (95% CI, 58.5%-89.7%), specificity of 96.4% (95% CI, 79.8%-99.8%) and 100% (95% CI, 85.0%-100%), positive predictive value of 95.7% (95% CI, 76.0%-99.8%) and 100% (95% CI, 82.8%-100%), and a negative predictive value of 75.0% (95% CI, 57.5%-87.3%) and 80.0% (95% CI, 62.5%-90.9%), respectively. Conclusions.--Our study indicated that C3d-IHC results for paraffin-fixed tissues were not significantly different from DIF results for the diagnosis of pemphigus. The C3dIHC assay has the potential for routine diagnosis of pemphigus, especially in the absence of fresh-frozen tissue. (Arch Pathol Lab Med. 2024;148:1022-1027; doi: 10.5858/arpa.2023-0274-OA), Pemphigus is an autoimmune blister disease that causes blisters on the skin and mucosal surfaces (1) and is characterized by autoantibodies against desmoglein (Dsg)1 or Dsg3. (2-4) There are 2 [...]

ABSTRACT Pemphigus vulgaris is an autoimmune disease characterized by intraepidermal blister formation. The treatment of pemphigus vulgaris is generally regarded as difficult. Corticosteroids, the drug class of first choice, often [...]

A molecular pathophysiology of pemphigus is explained to determine the mechanism of the formation of blisters in bullous impetigo and staphylococcal scalded-skin syndrome and to improve on the diagnosis and treatment of it. It is found that manipulation of T cells that are engaged in the control of a variety of physiologic and pathologic immune responses provides a promising option for the treatment of pemphigus.

Dentists have a unique opportunity to recognize pemphigus vulgaris early because it often occurs first in the mouth. Pemphigus vulgaris is an autoimmune disease that causes blistering. A complete description of the oral blisters is presented.

Abstract The life-saving management of pemphigus requires extended use of systemic therapies. Presently, there is no standard treatment for pemphigus, and each therapeutic approach carries potential benefits and risks. Recently [...]

Pemphigus vulgaris is a potentially fatal autoimmune blistering disease that is rare in childhood. We report 2 recent cases seen contemporaneously in adolescents who presented with chronic oral mucosal lesions and conjunctivitis. The previously unemphasized ocular manifestations are described and the importance of a biopsy in establishing the diagnosis in instances of unexplained chronic mucositis is stressed., ABBREVIATIONS. PV, pemphigus vulgaris; IgG, immunoglobulin G; IV, intravenous. Pemphigus, derived from the Greek word meaning bubble or blister, refers to a group of uncommon autoimmune skin conditions characterized by [...]

Context.--Pemphigus is a group of autoimmune vesiculobullous diseases characterized by immunoglobulin G (IgG) antibodies directed against desmosomal adhesion proteins, with IgG4 being the predominant subclass in active diseases. Direct immunofluorescence for IgG performed on fresh-frozen tissue plays a crucial role in diagnosing pemphigus. However, the diagnosis might be hindered when frozen tissue is not available. Objective.--To evaluate the usefulness of immunohistochemistry for IgG4 performed on paraffin sections as a diagnostic test for pemphigus. Design.--Eighteen immunofluorescence-proven pemphigus cases (12 pemphigus vulgaris, 6 pemphigus foliaceus) were studied. Four normal skin specimens and 32 nonpemphigus vesiculobullous disease specimens served as controls. Paraffin sections of all cases were examined immunohistochemically for IgG4 expression. Positivity was defined as distinct, condensed, continuous immunoreactivity localized to the intercellular junctions of keratinocytes. Results.--The immunostains were independently evaluated in a masked manner by 3 pathologists, with a 100% interobserver agreement. Nine of 12 pemphigus vulgaris cases (sensitivity 75.0%), and 4 of 6 pemphigus foliaceus cases (sensitivity 66.7%), were positive for IgG4 immunostain. The overall sensitivity was 72.2%. One control specimen (bullous pemphigoid) showed IgG4 positivity (specificity 97.2%). In specimens demonstrating acantholysis, 8 of 10 pemphigus vulgaris cases (sensitivity 80.0%) and 4 of 4 pemphigus foliaceus cases (sensitivity 100.0%) were positive for IgG4. The overall sensitivity for specimens with acantholytic lesions was 85.7%. Conclusion.--Immunohistochemistry for IgG4 provides a reasonably sensitive and highly specific test for diagnosing pemphigus, especially when frozen tissue is not available, and active acantholytic lesions are examined. (Arch Pathol Lab Med. 2012; 136:1402-1407; doi: 10.5858/arpa.2011-0425-OA), Pemphigus is a group of chronic, autoimmune, potentially fatal vesiculobullous diseases of the mucous membranes and skin. Pemphigus consists of 3 primary subsets (pemphigus vulgaris, pemphigus foliaceus, and paraneoplastic pemphigus) [...]

Byline: Adem. Koslu, Ilteris. Topal, Tugba. Ekmekci Sir, Pemphigus is an autoimmune blistering disease in which an antibody interacts with desmosomal proteins, leading to acantholysis of epidermal cells. One of [...]

* Context.--Endemic pemphigus foliaceus, or fogo selvagem, is a chronic autoimmune disease characterized by the formation of intraepidermal blisters that reduce adhesion between keratinocytes. Endemic pemphigus foliaceus is associated with the presence of autoantibodies and high levels of cytokines involved in the inflammatory response. Objectives.--To evaluate the expression of the proinflammatory cytokines interleukin 1, interferon [gamma], and tumor necrosis factor ; the proapoptotic inducers Fas and inducible nitric oxide synthase; and the apoptosis inhibitor Bcl-2; and to evaluate the presence of apoptosis. Design.--Skin biopsies from 13 patients with endemic pemphigus foliaceus and controls were evaluated by immunohistochemistry and apoptosis was determined by terminal deoxynucleotidyl transferase-mediated dUTP nickend labeling assay. Results.--Proinflammatory cytokines were only detected in cells of the inflammatory exudate. Inducible nitric oxide synthase, Fas, and Bcl-2 were expressed by both epithelial and inflammatory cells. Epithelial apoptosis was observed in 12 cases (92.3%), and subepithelial apoptosis in 11 cases (85%). Conclusions.--This study suggests that apoptosis as well as the local production of proinflammatory cytokines are associated with endemic pemphigus foliaceus lesions. These results may contribute to the development of new therapeutic approaches to endemic pemphigus foliaceus., Endemic pemphigus foliaceus (EPF), or fogo selvagem, is a chronic autoimmune disease (1)fc characterized by the formation of intraepidermal blisters, which are attributed to the presence of immunoglobulin G4 (IgG4) [...]

Byline: Archana. Singal, Deepika. Pandhi Sir, Pemphigus vulgaris (PV) is an autoimmune blistering disease characterized by extensive mucocutaneous vesicobullous lesions. Generally, the mucosal lesions precede skin lesions by weeks or [...]

Byline: N. El Fekih, M. Kharfi, A. Karaa, M. Kamoun Sir, Pemphigus is an uncommon mucocutaneous disease caused by autoantibodies against desmosomal antigens. Juvenile cases are rare. The diagnosis is [...]

Byline: Kidangazhiyathmana. Kumar Background: Pemphigus is a common autoimmune blistering disorder in India. However, there are no population based surveys from India available to study the prevalence of this disease. [...]

Byline: J. Pasricha, Poonam. Background: If administered properly, dexamethasone cyclophosphamide pulse (DCP) therapy has the potential to effect lifelong recovery from pemphigus. Aims: The objective of this paper is to [...]

Byline: Bhushan. Kumar, Sunil. Arora, Muthu. Kumaran, Rajesh. Jain, Sunil. Dogra Objectives : To conduct a cross-sectional study to compare Dsg1 and Dsg3 antibody levels independently with severity of disease [...]