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• W2056586604 abstract "The term pemphigus refers to a group of cutaneous diseases that are characterized by the development of intra-epidermal blisters and, sometimes, mucosal erosions [l] (Table 1). All forms of pemphigus are characterized by epidermal cell-cell detachment (acantholysis) which leads to (intra-epidermal) vesicle formation, and by the presence of IgG autoantibodies directed against antigenic determinants present on the cell surfaces of differentiating keratinocytes [2]. The most severe form of pemphigus is pemphigus vulgaris (PV) (Figure l), which may occur at any age, although its most common onset is in the fourth, fifth and sixth decades. PV is characterized by the presence of flaccid, exceedingly fragiIe noninflammatory bullae which usually arise on normal appearing skin. These bullae have a tendency to coalesce and rupture easily resulting in large denuded areas which are, in fact, the predominant clinical feature of this type of pemphigus. PV involves both the skin and mucous membranes and, in virtually all cases, the initial lesion affects the oral mucosa. Prior to the introduction of corticosteroids in the 195Os, PV was considered almost uniformly fatal mainly due to protein, fluid and electrolyte losses and/ or uncontrollable sepsis. Acantholysis, the histological hallmark of PV, starts with the development of epidermal intercellular edema leading to dissolution of intercellular ‘bridges’ and widening of intercellular spaces (ICS), finally ending in cell to cell detachment. These microscopic changes occur in the suprabasilar area, i.e. between the basal and spinous cell layers. Basal cells remain attached to the dermis, but are laterally detached, resembling a ‘row of tombstones’ [ 11. Endemic pemphigus foliaceus (PF) or fogo selvagem (FS) (Figure 2) is clinically and immunopathologically similar to the non-endemic form seen in other parts of the world. Clinically, PF is characterized by superficial blistering and erosive lesions that affect the skin and rarely involve mucosal surfaces [3-61. Histologically, PF is characterized by acantholysis involving the subcorneal layers of the epidermis. In" @default.
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• W2056586604 date "1991-02-01" @default.
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• W2056586604 title "Immunopathology of pemphigus" @default.
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• W2056586604 doi "https://doi.org/10.1016/0896-8411(91)90003-u" @default.
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