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IntroductionRecessive dystrophic epidermolysis bullosa is a rare genetic condition characterized by fragile skin and mucous membranes, caused by mutations in the COL7A1 gene. AA amyloidosis is a rare complication of this genodermatosis.ObservationsTwo patients with recessive dystrophic epidermolysis bullosa, severe generalized in the first case, and intermediate generalized in the second, developed nephrotic syndrome at the ages of 38 years and 28 years respectively. The diagnosis of secondary renal amyloidosis was confirmed by renal biopsy in the first case, and by minor salivary gland biopsy in the second. Death occurred 2 months after diagnosis in both cases.ConclusionRenal involvement is relatively common in AA amyloidosis in patients with recessive dystrophic epidermolysis bullosa. Nephrotic syndrome and rapid decline in renal function are characteristic features. The prognosis is poor due to the underlying conditions and the lack of etiologic treatment options.