History and clinical significance of mechanical symptoms in blistering dermatoses: a reappraisal.

In the era before the advent of routine microscopy and immunopathology, mechanical symptoms were described in an attempt to facilitate clinical diagnosis of blistering dermatoses. A plethora of eponyms and signs were described, but no proper original quotations exist. All the eponyms haunt the student and specialist.

Ultrastructural study of clinically uninvolved skin of patients with pemphigus vulgaris.

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.

Pemphigus and pemphigoid epidermocytes interfere with interleukin cascade reactions.

The aim of this study was to reveal the origin of interleukin cascade reaction disturbances in pemphigus vulgaris (PV) and bullous pemphigoid (BP). We investigated the influence of culture supernatant of epidermal keratinocytes from the skin of PV and BP patients on functional activity of donor peripheral blood mononuclears. It was found that epidermal keratinocyte culture supernatant in the acute phase of PV and BP decreased the ability of mononuclears to proliferate in response to PHA and Con A, secrete interleukin-1 and interleukin-2, and absorb an exogenous interleukin-2.

Clinical and laboratory evaluation of hemocarboadsorption in autoimmune bullous dermatoses.

Hemocarboadsorption (HCA) with the following immunosuppressive therapy was used to treat 48 patients with pemphigus vulgaris (PV) and 31 patients with bullous pemphigoid (BP) in the acute phase. The levels of autoantibodies, eicosanoids, endoproteases and their inhibitors as well as interleukin (IL) 1 and IL 2 production and absorption of exogenous IL 2 were examined in the treatment dynamics. The therapeutic effect of HCA was displayed in prompt disease remission in 43 PV and 29 BP patients.

Stimulating effect of pemphigus antibodies upon interleukin inhibitor production by normal human epidermocytes.

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.

Mediators of inflammation in blister fluids from patients with pemphigus vulgaris and bullous pemphigoid.

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.

The effect of experimental haemocarbofiltration upon activity of mononuclear cells from normal and autoimmune patients.

We examined the functional activity of peripheral blood mononuclears (PBM) of 18 healthy subjects, 18 patients with pemphigus vulgaris, 12 with bullous pemphigoid and nine with discoid lupus erythematosus, after haemofiltration on carbon haemo-adsorbents of 'SKN' type. Proliferation in the response to PHA and Con A, for IL-1 and IL-2 production, and exogenous IL-2 absorption were assayed. The presence of IL-1 and IL-2 inhibitors in haemocarbo-adsorbent eluants was shown.

Cytotoxic proteases in blister fluid of pemphigus and pemphigoid patients.

Investigation of blister fluid (BF) from 49 pemphigus vulgaris and 27 bullous pemphigoid patients revealed direct interrelation between proteolytic and cytotoxic activities of BF. Human epidermal keratinocytes proved to be more sensitive to the cytolytic effect of BF as compared to human endotheliocytes and fibroplasts. Epidermal keratinocyte cultivation in patients' BF led to proteolytic activity enhancement in culture supernatant.

Autoreactive cytotoxic T lymphocytes in pemphigus and pemphigoid.

In order to know whether effector cells are capable of recognizing the epidermal targets in pemphigus vulgaris (PV) and bullous pemphigoid (BP), non-adherent cells (NAC) of peripheral blood from 27 primary PV, 19 BP patients and 12 healthy volunteers were used in cytotoxic tests. Autologous, allogenic and murine epidermal keratinocytes as well as autologous and allogenic fibroblasts were employed as targets. Total esterase activity of serine proteinases was measured in supernatants of samples and the results obtained were compared with the data received in 51Cr-release assay.

Serine proteinase esterolytic activity as an assay of cytotoxic reactions.

A method for assessing cytotoxicity in cell-mediated cytolysis as well as in reactions of natural killing and antibody-dependent cellular cytotoxicity through assaying the total esterolytic activity of serine proteinases (TEASP) is both sensitive and specific. TEASP levels in all cytotoxic tests correlated with the percentage of lysis of 51Cr-labelled target cells. The method permits the differential assessment of endoproteases in both the effector cell and the target cell.

Interleukin cascade reaction inhibition by supernatants of the cultures of antibody-transformed human basal keratinocytes.

We investigated the ability of normal human basal keratinocytes, treated with antibodies against basal keratinocytes, to produce an effect upon the activity of allogenic peripheral blood mononuclear cells. We determined the influence of basal keratinocyte culture supernatants (BKCS) upon the mononuclear cell ability for proliferation in the response to PHA and Con A, for IL 1 and IL 2 production as well as for absorption of exogenous IL 2. It was found that BKCS inhibit the lectin-dependent transformation of mononuclears and interleukin cascade reactions.