Epidermolysis bullosa dystrophica with renal failure due to secondary amyloidosis
Makaleler > Tam Makale · 2007
Yazarlar: FATMA MUTLUBAŞ, SEVGİ MİR, Süleyman Caner KABASAKAL, ONDER YAVASCAN, Banu SARSIK KUMBARACI
Indian Journal of Nephrology, ESCI İndekslerine Giren Dergi, Uluslararası, Scopus, 2007, cilt 17, ss 178-178
Özet
Epidermolysis bullosa dystrophica is a rare hereditary disorder with multiple bullous lesions, erosions and repeated infections. Although skin lesions can be treated with dermatologic care, the patient can also be complicated by life-threatening clinics such as nephropathy, systemic amyloidosis and skin cancers by years. A 16-year-old male who had been followed-up since birth with epidermolysis bullosa dystrophica referred to the nephrology department for nephrotic proteinuria and deep anemia. He went on renal biopsy that demonstrated AA amyloidosis. Although effective colchicine treatment was given, end stage renal disease developed after 6 months that resulted in the death of the patient. Epidermolysis bullosa causes a chronic inflammatory process that progresses with time and can be fatal. Colchicine treatment must be given before the onset of amyloidosis.