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Blister fluids from 39 previously untreated patients with pemphigus vulgaris (PV) and 28 patients with bullous pemphigoid (BP) were tested for the presence of inflammatory mediators. Blister fluids from patients with PV and BP showed interleukin 1 (IL-1)- and interleukin 2 (IL-2)-like activity and contained prostaglandin (PG) E2, F1 alpha, and F2 alpha, thromboxane B2 (TB2), leukotriene (LT) B4 and C4, serine esterases, and proteolysis inhibitors. Pemphigus blister fluid was distinguished by high IL-1-like activity, proteolytic activity, and high concentrations of TB2 and LTB4, while pemphigoid blister fluid was characterized by higher IL-2-like activity, antiprotease activity, and high PGE2 concentration. We also determined that the content of mediators of inflammation varied depending on the duration of blister development. In both PV and BP, the initially high IL-1-like activity, amount of serine proteases, and concentrations of PGF2 alpha, TB2, LTB4, and LTC4 decreased by the fifth day of blister existence, whereas antiprotease activity as well as PGE2 and PGF1 alpha concentrations gradually increased as blisters developed. These findings suggest certain differences in the character of the bullous inflammatory process in pemphigus and pemphigoid.

We investigated skin biopsies from pemphigus vulgaris (PV) patients by light, fluorescent and electron microscopy in order to study the ultrastructural appearances of epidermis at the pre-acantholytic stage. The biopsies were obtained from uninvolved forearm skin in 10 patients with PV in the acute stage of the disease, from perilesional skin of the same patients as well as from the forearm skin of 10 healthy subjects. Light microscopy showed no pathological changes in clinically uninvolved skin of pemphigus patients. Direct immunofluorescence confirmed the presence of IgG auto-antibodies fixed in intercellular space of the spinous-cell layer of uninvolved skin. Electron microscopy of the uninvolved skin biopsies revealed the following changes: disintegration of desmosomes of spinous cells with their replacement by finger-shaped protrusions of cytoplasm; clarification of the nuclear matrix; widening of the perinuclear slit; an increased number of secondary lysosomes in cells; oedema and swelling of mitochondria with destruction of their cristae. The cells retained their polygonal shape and the intercellular distance did not increase. We conclude that at the pre-acantholytic stage the breakage and dissolution of desmosomes precedes the increase in the intercellular space.

The present study deals with the effect of pemphigus antibodies upon immunoregulatory activity of normal human keratinocytes. In vitro experiments were carried out to determine the effect of epidermal keratinocyte culture supernatants (EKCS) upon the ability of peripheral blood mononuclear cells from healthy subjects to give lymphoproliferative response to lectins, and to induce production of interleukin I and interleukin 2 activity, and exogenous interleukin 2 absorption. It was found that EKCS, obtained in response to epidermal keratinocyte cultivation in the presence of I mg/ml pemphigus antibodies, inhibit both interleukin cascade reactions and mitogen-induced lymphoproliferative response. It is supposed that the interleukin production deficiency in pemphigus may emerge as a result of suppressive effect made by autoantibodies on epidermal keratinocytes.

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