engleski

CASE OF CONCURRENT EPIDERMOLYSIS BULLOSA ACQUISITA AND ANTI&not ; p200 PEMPHIGOID -HOW TO TREAT IT?

We describe a 65-year-old man with a six year history of clinical, histological and immunological features of mixture of epidermolysis bullosa acquisita and anti-p200 pem&not ; phigoid. Indirect immunofluorescence of 1M NaCl split human skin detected IgG anti&not ; bodies reactive with dermal side. By immunoblotting of human dermal extracts, the patient sera reacted with both the 290 kDa type VII collagen and the p200. Previous treatment with corticosteroids in combination with dapsone, cyclosporine, or mycophenolate mophetil, resulted in moderate response but in numerous side-effects. Therefore, high-dose intravenous immunoglobulin therapy using commercially available preparation was employed. The therapy was well tolerated without side-effects by our patient. The result was promising. To date, one therapy course has been employed due to financial restrictions. We believe that repeated courses of high-dose intravenous immunoglobulins would reduce atrophic scarring and maintain the motility of palms and soles, as the symptom of the disease undisputedly leads to dermatogenic contractures and therefore is disabling the patient through life.

Epidermolysis abullosa acquisita anti-p200 pemphogoid treatment

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