SUMMARY AND CONCLUSION
Pemphigus and bullous pemphigoid are rare autoimmune bullous diseases characterized by blisters with intraepidermal “pemphigus” or dermo-epidermal “pemphigoid” localization.
The pathogenesis of these diseases is based on autoimmunity, pemphigus lesions are mediated by auto-IgG directed against keratinocytes “Desmogleins” while bullous pemphigoid “BP” blisters present a cleavage between the epidermis and lamina lucida and are caused by autoantibodies “mainly IgG” directed against proteins of 230 and 180 KD “major antigens” or proteins ranging between 18 and 200 KD “minor antigens”.
Inflammatory processes are characterized by proinflammatory cytokine over production and subsequent membrane molecule induction or enhancement in cytokine receptor-bearing cells that are activated by these different biological mediators. Stimulated cells shed many of these molecules in microenvironmental fluids after cleavage modifying the concentrations of shed molecules in the circulating blood.
Serum determination of CAMs, may help to evaluate the activity of the different cellular components especially when the molecule considered is restricted to a single cell type.
Soluble E-selectin is the soluble isoform of membrane adhesion molecules able to bind leucocytes. It is exclusively expressed by activated endothelial cells, and its induction is enhanced by IL-1 and TNF-. Adhesion molecules are increased in different inflammatory diseases. Little informations are available regarding the serum soluble E-selectin level in patients with PV and BP.
The aim of our study was to evaluate serum level of soluble
E-selectin in PV and BP patients in relation to the number of skin lesions as a non-specific follow-up marker suitable to gauge disease intensity overtime. Thirty-five patients were included in the study, twenty with PV “14 females and 6 males”, fifteen with BP “10 males and 5 females”, complete history taking, general examination and dermatological examination were done and the clinical activity of the disease was estimated by simple scoring system.
The diagnosis of the diseases was confirmed by H & E examination, immunoperoxidase study on lesional skin and IIF test. Serum samples were taken from the patients, before treatment, and 2, 4 and 6 weeks after treatment to estimate the serum soluble E-selectin level comparing with its level in the serum of healthy ten control subjects.
In this study, males outnumbered females in BP while female predominance was observed in PV patients. The mean age was 44.25 9.18 year in PV while it was 57.75 8.87 year in BP group. By H & E staining there was suprabasal bullae in PV and subepidermal bullae with inflammatory infiltrate in BP. Immunoperoxidase detected intercellular (PV) or subepidermal IgG (BP) deposition in all patients and C3 deposition in 55% in PV and 66.6% in BP patients. IIF detected circulating intercellular (PV) or subepidermal (BP) IgG on monkey oesophagus as a substrate.
The serum soluble E-selectin levels correlated with the number of visible lesions of both PV & BP patients, concomitantly dropped with the decrease in the number of the lesions. The median serum soluble E-selectin level was 86.76 55.95 ng/ml before treatment while it became 32.37 7.70 ng/ml after 6 weeks in PV patients, while it was 114.21 53.39 ng/ml before treatment and became 36.44 10.84 ng/ml after 6 weeks in BP patients.
These data further underline endothelium involvement in these bullous dermatoses and stress the possibility of employing sE-selectin as a non-specific follow-up marker of both PV and BP patients, also determination of sE-selectin could represent a clinically useful indicator to monitor therapy.
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