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Pemphigus as a life-threatening autoimmune bullous disorder
Date Issued |
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2018-10-04 |
Bibliogr.: p. 58
Introduction & Objectives. Pemphigus vulgaris (PV) is an autoimmune, intraepithelial, blistering disease affecting the skin and mucous membranes. It is mediated by circulating antidesmoglein (anti-Dsg) 3 and anti-Dsg 1, which are antibodies directed against keratinocyte cell surfaces. A life-threatening disease being managed with systemic corticosteroids and immunosuppressives often complicates with sepsis and cardiac failure and it has a mortality rate of approximately 10% [1,2]. It is higher in patients with predominantly mucocutaneous PV (8-42%) compared with mucosal PV (1-17%) [2,3]. We present non-consecutive case series of PV with good clinical response but severe side effects because of immunosuppressive therapy. Case reports. A 51-year-old male suffered from PV for almost 3 years. Since 2015 year the patient was treated with continuous course of oral corticosteroids starting from 90 mg/d, as well as adjuvant azathioprine100 mg/d. A long-term treatment with high dose systemic corticosteroids arose severe psychiatric disorders (delusions, anxiety, depression), Cushing's syndrome, secondary diabetes mellitus and artery hypertension, osteoporosis with spontaneous vertebral fracture, secondary skin and mucous infections, including phlegmon of the arm. A 44-year-old male patient has been treated for PV for 5 years. Clinical response to high-dose corticosteroid (start dose 90 mg/d) and azathioprine 150-100 mg/d therapy was achieved, but also drug adverse effects appeared progressively, including depression, weight gain and secondary viral and bacterial infections. At the exacerbation of PV, ELISA values of serum anti-Dsg 1 and anti Dsg 3 autoantibodies were detected, accordingly 7.53 and 8.4 U. A 53-year-old woman experienced a severe form of PV for 3 years. Corticosteroids (start dose 90 mg/d) and methotrexate 10 mg/wk did not control the disease activity, therefore azathioprine 50-100 mg/d was changed to met[...].