Involvement in pemphigus vulgaris of the female genital tract including the vulva, vagina and cervix has previously been described. In all these cases other cutaneous and mucosal sites have also been affected at some time. We describe a case of pemphigus vulgaris which only involved the vaginal mucosa. The patient presented with a persistent vaginal discharge and examination showed extensive vaginal erosions. Histology of vaginal biopsies was non-diagnostic. The recognition that the vaginal changes may represent an immunobullous disease led to further vaginal biopsies on which direct immunofluorescence studies were performed. These biopsies showed IgG and C3 in the intercellular epidermis, suggesting a diagnosis of pemphigus vulgaris. During the 3-year period that the patient has been under review there have never been any other cutaneous or mucosal lesions. To our knowledge, this is the first case of pemphigus vulgaris localized exclusively to the vaginal mucosa. There was considerable delay in diagnosis and this case highlights how important it is to recognize that chronic mucosal lesions at genital sites may be caused by immunobullous diseases such as cicatricial pemphigoid and pemphigus, and to institute appropriate investigations.
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http://dx.doi.org/10.1046/j.1365-2133.1999.02832.x | DOI Listing |
Expert Opin Drug Saf
January 2025
Section of Dermatology - Department of Clinical Medicine and Surgery, University of Naples Federico II, Naples, Italy.
Introduction: Bruton's tyrosine kinase (BTK) is a cytoplasmic signaling protein expressed across a variety of immune cells, terminally differentiated plasma cells, and natural killer cells. Due to the signal potential and targetable nature of BTK, the use of BTK inhibitors (BTKis) has been proposed for the management of several diseases. Currently, the use of BTKis is under investigations for several dermatological conditions such as pemphigus, systemic lupus erythematosus, hidradenitis suppurativa, atopic dermatitis, and chronic spontaneous urticaria (CSU).
View Article and Find Full Text PDFFront Immunol
January 2025
Department of Rheumatology, Shanghai Skin Disease Hospital, Tongji University School of Medicine, Shanghai, China.
Pemphigus vegetans (P Veg), the rarest subtype of pemphigus, is characterized by vegetative plaques, primarily affecting intertriginous areas. The most common autoantibodies target desmoglein 3 (Dsg3). A 60-year-old female patient presented with well-demarcated red vegetative plaques on her feet, vulva, and thigh, accompanied by surrounding pustules.
View Article and Find Full Text PDFFront Immunol
January 2025
Department of Dermatology, Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, Buffalo, NY, United States.
Autoimmune diseases (AID) are defined by immune dysregulation characterized by specific humoral and/or cell mediated responses directed against the body's own tissues. Cytokines in particular play a pivotal role in the pathogenesis of AID, with proinflammatory cytokines contributing to the initiation and propagation of autoimmune inflammation, whereas anti-inflammatory cytokines facilitate regression of inflammation and recovery from acute phases of the disease. Parallel work by our group evaluating a comprehensive set of pro- and anti-inflammatory serum cytokines in Pemphigus vulgaris (PV) as well as Alopecia areata (AA) uncovered a similar pattern of inheritance specific immune dysregulation in these two distinct autoimmune skin diseases.
View Article and Find Full Text PDFMed J Armed Forces India
August 2022
Professor & Head (Dermatology), DY Patil Medical College & Research Centre, Pune, India.
Background: Autoimmune bullous disorder (AIBD) is a diverse group of blistering dermatoses that affects the skin and mucous membrane, characterized by the formation of autoantibodies against the desmosomal glycoproteins and adhesion molecular components of the basement membrane zone. Various immunoassay techniques for serological diagnosis are Direct Immunofluorescence (DIF), Indirect Immunofluorescence (IIF), Enzyme Linked Immunosorbent Assay (ELISA) and immunoblotting. Quantitative ELISA titer can also be used to monitor the disease activity and response to treatment.
View Article and Find Full Text PDFBr J Dermatol
January 2025
Institute of Anatomy and Cell Biology, Department I, Faculty of Medicine, Ludwig-Maximilians-Universität, Munich, Germany.
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