Sánchez-Sustaita JR, Acosta-García N, Castillo-Guitarrero S, Uribe-Urbe NO, Bautista-Martínez B, Sánchez-Benítez FA

Language: Spanish
References: 1
Page: 180-182
PDF size: 878.37 Kb.

ABSTRACT

Introduction: dystrophic epidermolysis bullosa (DED) is a severe genodermatosis associated with systemic complications, including chronic kidney disease (CKD) secondary to various glomerulopathies. In advanced cases, kidney transplantation represents a viable therapeutic option, although it carries a risk of immunological complications. Clinical case: a 41-year-old man with congenital DED and CKD of 17 years' duration, initially treated with hemodialysis and subsequently transplanted in 2010. During transplant follow-up, nephrotic proteinuria (3.74 g/24 h) without clinical nephrotic syndrome was documented, with impaired baseline renal function (Cr 2.03 mg/dL). A renal graft biopsy was performed, revealing active chronic rejection (i-IFTA 2, i2, t1, ptc1), chronic glomerulopathy (cg2), acute tubular injury, and IFTA 30%, 90% diffuse podocyte fusion was observed under electron microscopy. Treatment with methylprednisolone and rituximab was initiated, with partial improvement in proteinuria. Conclusions: glomerulopathies in patients with DED are an emerging and probably underdiagnosed entity. This case demonstrates a prolonged post-transplant evolution with late onset of podocytopathy and active chronic rejection, highlighting the diagnostic value of biopsy with electron microscopy. Immunosuppressive management must be personalized, due to the immunological and infectious vulnerability of these patients.

REFERENCES

1. Cicek N, Yildiz N, Asadov R, Yucelten AD, Tugtepe H, Alpay H. Kidney and Urinary Tract Involvement in Epidermolysis Bullosa: Is Routine Follow-Up Necessary? Dermatol Pract Concept. 2021; 11 (3): e2021051. doi: 10.5826/dpc.1103a51