Retrospective Analysis of a Single- Kuwaiti Center Clinical Experience Toward Development of Proper Controlling Treatment of 84 Pemphigus Patients with a Long-Term Follow-Up
Pemphigus is the name of a group of autoimmune pathological entities characterized by the formation of intraepithelial blisters in the skin and/or mucosa. Pemphigus vulgaris (PV) and Pemphigus foliaceus (PF) are considered as classical forms. Rare, non-classical forms of Pemphigus include: Pemphigus herpetiformis, IgA pemphigus, Paraneoplastic pemphigus, IgG/IgA pemphigus. Pemphigus is the name of a group of autoimmune pathological entities characterized by the formation of intraepithelial blisters in the skin and/or mucosa . Autoantibodies are directed against desmogleins (DsGs) on the surface of keratinocytes, which results in loss of cellular adhesion, and formation of intraepidermal bullae, the triggering event leading to antibody formation is unknown. Pemphigus vulgaris (PV) and Pemphigus foliaceus (PF) are considered as classical forms. Rare, non-classical forms of Pemphigus include: Pemphigus herpetiformis, IgA pemphigus, Paraneoplastic pemphigus, IgG/IgA pemphigus . The overall incidence of pemphigus is estimated at 0.076 to 5 in 100,000 person per year . The incidence of pemphigus vulgaris is higher in women (Male: Female=1: 1.1–2.25) . PV is the most common variant of Pemphigus, usually begins with painful ulcerations on oral and other mucosa. The primary skin lesions of pemphigus vulgaris are flaccid, thin-walled, easily ruptured blisters that appear anywhere on the skin surface . Histological studies of PV lesions usually demonstrate acantholysis in the supra-basilar part of the epidermis . Without appropriate treatment, pemphigus vulgaris can be fatal because large areas of the skin lose epidermal barrier function, leading to loss of body fluids or secondary bacterial infection.
The primary lesions in PF are flaccid, superficial vesicles and bullae of the skin. These lesions some-times may not be seen on examination because of their fragile and subsequent transient nature. More often, only secondary lesions, such as shallow erosions, are seen . There are no mucosal lesions, even with widespread disease. Intradermal acantholysis occurs in the granular layer . The intraepithelial expression patterns of Dsg1and Dsg3 in skin and mucous membranes differ. Dsg1 is expressed throughout the epidermis, but more intensely in the superficial layers, while Dsg3 is expressed in the lower portion of the epidermis, primarily in the basal and supra-basal layers. In contrast, Dsg1 and Dsg3 are expressed throughout the squamous layer of the mucosa, but the former is expressed at a much lower level than the latter . The diagnosis of the pemphigus group of diseases is based on clinical picture and history, histopathologic diagnosis, and direct and indirect immunofluorescence . Direct immunofluorescence (DIF) demonstrates an intercellular deposition of IgG and C3 in a “chicken-wire” lattice pattern. For diagnosis of any pemphigus disease, sensitivity of DIF has been found to range from 80–95% . The substrate for indirect immune-florescence (IIF) usually Monkey esophagus (for PV) and Normal Human Skin (for PF); if autoantibodies from the patient’s serum are present and bound, the fluorescein conjugated IgG anti-serum will fluorescence under microscopy , seen as an intracellular staining. The test is reported as a titer.
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Journal of Clinical & Experimental Dermatology Research