ISSN: 2574-7800
Authors: Verbytska LV*
Pemphigus is a group of potentially life-threatening autoimmune diseases characterized by the formation of skin and / or mucous membranes. Among the many types of pemphigus, the most rare clinical option is vegetative. It is often difficult to diagnose and clinically recognize this pathology because of the variety of expressions and the presence of verrucous formations. Therefore, the purpose of this work is to highlight the issues of the clinic, diagnosis and treatment of vegetative pemphigus (PV). The literature review and analysis of the results of clinical examination of a patient with PV were performed.The vegetative pemphigus accounts for 1 to 2% of all cases of pemphigus disease, and worldwide the incidence of vegetative vesicle is approximately 0.7 per 100,000. In most patients, the initial manifestations are stomatitis. From several weeks to months after the onset of mucous membranes, skin pustules and / or flaccid bullae are formed on unmodified skin, usually affecting the torso, arms, legs, and flexion areas. The skin formations are destroyed, eroded, form vegetations, covering with vegetative plaques the eroded surface characterized by abnormal growth of keratinocytes. These hyperkeratotic lesions are typically present in the intertriginous areas - including the groin / inguinal folds, armpits, hips, and flexion. The pathological process is based on the formation of IgG autoantibodies against desmoglins, one of the major cell adhesion proteins of the desmosome, causing loss of cell adhesion. This loss of adhesion leads to acantholysis. Histopathologically early lesions of pemphigus vulgaris and pemphigus vegetans demonstrate transbasal acantholysis. Pemphigus vegetans also exhibits epidermal hyperplasia, papillomatosis and intraepidermal eosinophilic abscesses according to the duration of the lesion. The diagnosis of a vegetative vesicle is made on the basis of clinical features, histological examination and detection of autoantibodies. The most widely used methods of detecting pemphigus autoantibodies include direct and indirect immunofluorescence, immunoprecipitation, immunoblotting, and enzyme-linked immunosorbent assay (ELISA). Treatment recommended by European and Japanese guides includes topical internal focal therapy, systemic therapy, wound care, and oral treatment features. This article presents a clinical case of a patient diagnosed with Vegetative Pemphigus. The severity and course of the disease is variable, but before the onset of steroids, most patients died. Treatment with systemic steroids reduced mortality rates. Morbidity and mortality are associated with the degree of disease, the maximum dose of prednisolone required to induce remission, and the presence of other diseases. The prognosis is worse for elderly patients and patients with comorbidities.
Keywords: Pemphigus vegetans; Acantolysi; Treatment; Desmosom; Diagnostic Vegetative pemphigus
