engleski

Mixed disease of bullous pemphigoid and IgA pemphigus foliaceus

A 63-year-old male patient was admitted to our Department with clinical picture of bullous pemphigoid with blisters, erosions and crusts on the erythematous skin of his face and body. Histology revealed subepidermal separation. On direct immunofluorescence, C3 was detected along the basement membrane zone (BMZ). Standard indirect immunofluorescence (IIF) using normal human skin section showed IgG anti-BMZ antibodies at a titer >160 and IgA anti-cell surface antibodies at titer 10, stronger in the upper epidermis. On IIF on 1M NaCl- split human skin section, IgG was located on the epidermal side of the split. On immunoblotting of normal human epidermal extracts, the patient IgG antibodies reacted with BP230 and BP180, while IgA antibodies were negative. On immunoblotting of the BP180 NC16a domain recombinant protein, IgG antibodies in patient serum strongly reacted with this recombinant protein. cDNA transfection of desmocollin 1-3 for IgA was negative. ELISA of recombinant baculoproteins of Dsg1 and Dsg3 for IgG was negative. However, ELISA of recombinant baculoprotein of Dsg1 and Dsg3 for IgA detected anti-Dsg1 IgA antibodies in the patient serum. ELISA of recombinant proteins of BP180 and BP230 for IgG was also strongly positive. Therefore, all biochemical results suggested it to be a case is mixed disease of bullous pemphigoid and IgA pemphigus foliaceus.

bullous pemphigoid; IgA pemphigus

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