Renal Amyloidosis Secondary to Dystrophic Epidermolysis Bullosa: A Case Report and Review of Literature

Dystrophic epidermolysis bullosa (DEB) is a rare and severe hereditary dermatosis, associated with collagen VII deficiency. A chronic inflammatory syndrome secondary to recurrent cutaneous infections may be responsible for amyloid deposition in this patient population, causing renal amyloidosis. Amy...

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Veröffentlicht in:Turkish Journal of Nephrology 2020-10, Vol.29 (4), p.322-325
Hauptverfasser: Barutcu Atas, Dilek, Aykent, Mahmut Basar, Arikan, Izzet Hakki, Asicioglu, Ebru, Velioglu, Arzu, Filinte, Deniz, Koc, Mehmet, Serhan Tuglular, Zubeyde, Ozener, Ishak Cetin
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Sprache:eng
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Zusammenfassung:Dystrophic epidermolysis bullosa (DEB) is a rare and severe hereditary dermatosis, associated with collagen VII deficiency. A chronic inflammatory syndrome secondary to recurrent cutaneous infections may be responsible for amyloid deposition in this patient population, causing renal amyloidosis. Amyloidosis should be included in the differential diagnosis of DEB patients presenting with edema and proteinuria. Herein, we report a case of DEB complicated by squamous cell carcinoma and amyloid A amyloidosis of the kidneys confirmed with renal biopsy. Keywords: Amyloidosis, dystrophic epidermolysis bullosa, nephrotic syndrome
ISSN:2667-4440
2667-4440
DOI:10.5152/turkjnephrol.2020.4195