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Kidney Week

Abstract: SA-PO946

Bullous Pemphigoid and Renal Transplant Rejection: More Than a Mere Coincidence?

Session Information

Category: Nephrology Education

  • 1302 Fellows and Residents Case Reports

Authors

  • Koratala, Abhilash, University of Florida, Gainesville, Florida, United States
  • Clapp, William L., University of Florida, Gainesville, Florida, United States
  • Olaoye, Olanrewaju Adebayo, University of Florida, Division of Nephrology, Gainesville, Florida, United States
  • Santos, Alfonso, University of Florida, Gainesville, Florida, United States
Background

Bullous pemphigoid (BP) is an autoimmune blistering disease characterized by subepithelial blister formation and deposition of immunoglobulins and complement in the epidermal and/or mucosal basement membrane zone. There are few case reports on the association between BP and allograft rejection, and membranous nephropathy (MN). We report a unique case of BP associated with both acute cellular rejection (ACR) and de novo MN in a renal transplant recipient.

Methods

A 63-year-old man with a history of ESRD secondary to MN, status post living related kidney transplant 5 years ago who has been compliant with his immunosuppressant regimen was admitted for worsening skin rash that started 3 weeks ago and acute kidney injury. Skin exam revealed multiple scattered bullae with clear fluid and erosions with a collarette of scale on the neck, chest, back and limbs. His serum creatinine (Scr) was 7.3 mg/dL at presentation (baseline ~1.5). Urine exam showed 45/hpf RBC, 1/hpf WBC and an albumin-creatinine ratio of ~0.5 g/g. BK virus, CMV, Herpes simplex and Varicella zoster serologies were negative. Skin biopsy revealed BP. His Scr minimally improved with supportive measures and allograft biopsy showed Banff 2A ACR with de novo MN and ~50% interstitial fibrosis and tubular atrophy (IFTA). He was treated with pulse corticosteroid and antithymocyte globulin but his Scr remained unimproved after 1 month. Further immunosuppression was not attempted due to the severity of IFTA on repeat biopsy. His skin lesions eventually improved with high-dose steroid therapy.

Conclusion

Though the possibility of multiple distinct autoimmune processes cannot be excluded, allograft rejection-induced immune stimulation or anti-basement-zone antibody interactions are possible unifying mechanisms for the simultaneous skin and renal involvement. Whether the diagnosis of BP in a renal transplant recipient warrants kidney biopsy remains unanswered.