Summary. Pemphigus is a chronic malignant disease. It is clinically expressed by the formation of blisters on non-inflamed skin and mucous membranes. Bubbles and erosion tend to merge and generalize the process, which, in the absence of timely and adequate therapy, leads to the death of the patient. Changes in the cells of the basal and spiny layers of the epidermis in pemphigus are the main cause of the development of apoptosis, then acantholysis and rupture of intercellular bonds (desmosomes), which indicates disturbances in the epidermal regulatory system that precede and then, at the final stage, contribute to the development of the autoimmune process.
Almeida HL Jr, Monteiro LM, Goetze FM,Silva RM, Rocha NM. Clinical variability indystrophic epidermolysis bullosa andfindings with scanning electron microscopy.An Bras Dermatol. 2012;87:127-30.
Cunha PR, Barraviera SRCS . Autoimmunebullous dermatoses. An Bras Dermatol2009;84:111-24.
Di Zenzo G, Amber KT, Sayar BS, Mü llerEJ, Borradori L. Immune response inpemphigus and beyond: progresses andemerging concepts. Semin Immunopathol.(2016) 38:57–74. doi: 10.1007/s00281-015-0541-1
Gordon MK, Hahn RA. Collagens CellTissue Res. 2010;339:247-57.
Michenko A.V., Znamenskaya L.F., LvovA.N. Methods for revealing therapeutictargets in case of true acantholyticpemphigus. Vestn dermatol i venerol. 2012;5: 38-43. [Миченко А.В., Знаменская Л.Ф.,Львов А.Н. и соавт. Методы выявлениятерапевти- ческих мишеней при истиннойакантолитической пузырчатке. Вестндерматол и венерол 2012; (5): 38-43.]
Oliveira ZNP, Perigo AM, Fukumori LMI,Aoki V. Immunological mapping inhereditary epidermolysis bullosa An BrasDermatol. 2010;85:856-61
Palleschi GM, Torchia D, Pacini P.Scanning electron microscopy in ultrastructural morphology of perilesional keratinocytes in pemphigus foliaceous. Acta Cytol. 2007;51:941-3.
Reshetnikova Т.В., Yefremov A.V. Complextherapy for acantholytic pemphigus. Rus Jof skin and sexually transmitted disiases2005; 5: 28-31. [Решетникова Т.Б.,Ефремов А.В. Комплексная терапияакантолитической пузырчатки. Росс журнкож и вен бол 2005; (5): 28-31.]
Schmidt E, Kasperkiewicz M, Joly P.Pemphigus. Lancet. (2019) 394:882–94.
Suga H, Tsunemi Y, Sugaya M, ShinkumaS, Akiyama M, Shimizu H, et al. Hair shaftabnormalities in localized autosomalrecessive hypotrichosis 2 and a review ofother non-syndromic human alopecias. ActaDerm Venereol. 2011;91:486-8