New diagnosis of bullous pemphigoid after withdrawal of immunosuppressive therapy in a failed renal transplant recipient on hemodialysis

Abstract

The occurrence of the autoimmune blistering disease, bullous pemphigoid (BP), in patients with failed renal allograft is rare and the few reported cases suggest various provocative factors without reaching a consensus. Here we report the case of a patient presenting with bullous lesions soon after the complete discontinuation of immunosuppressant therapy following renal allograft failure. Skin biopsy confirmed the diagnosis of BP. Administration of systemic corticosteroid controlled the occurrence of BP lesions in our patient. Increased clinical suspicion is warranted in cases of patients with renal transplant failure, since withdrawal of the immunosuppressant therapy could lead to unmasking the underlying autoimmune skin disease.

Pemphigoid diseases in patients with end-stage kidney diseases: pathogenesis and treatment

“…After tapering IS, a significant number of autoantibodies may be generated and contribute to pemphigoid diseases. This is evidenced by nine patients who developed pemphigoid diseases after decreasing or withdrawing of IS ( 35 – 43 ). Resolution of BP after restarting IS therapy has been observed.…”

“…In the realm of dermatology, the effectiveness of corticosteroids in treating pemphigoid diseases is well-established and corticosteroids remain a mainstay treatment. Studies have shown the effectiveness of corticosteroids in managing BP, even in complex cases like post-transplant recipients or in patients experiencing kidney transplant rejection ( 35 , 41 ). Among 59 reported cases using corticosteroids, 14 (23.73%) patients were recalcitrant ( 23 , 25 , 37 , 44 , 46 , 48 , 50 , 52 , 62 , 67 – 70 ).…”

“…Notably, MMF is a potent IS commonly used in organ transplant recipients ( 77 ). It has been used as an IS drug in nine patients with ESKD ( 16 , 23 , 35 , 37 – 39 , 41 , 43 , 51 ). However, the onset of BP has been observed in six cases after discontinuing MMF and other IS drugs.…”

“…Additionally, four patients with ESKD used AZA (50-100 mg/day for 2-4 weeks) in combination with other drugs to treat BP, with two achieving resolutions ( 41 , 52 , 82 ). The utilization of AZA was successful to reduce the reliance on corticosteroids in one patient ( 35 ). Therefore, AZA may be a safe and effective strategy to treat pemphigoid diseases in conjunction with renal allograft rejection, but further research is needed to validate its efficacy.…”

Bullous pemphigoid with prominent mucosal involvement in the setting of renal allograft rejection

“…There have also been reports of BP after renal allograft rejection. 1 , 2 , 3 , 4 , 5 , 6 , 7 , 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 , 17 Here, we present 2 cases of renal allograft rejection associated BP involving oral mucosal lesions. Our literature review suggests that mucosal involvement may be relatively common in BP associated with renal allograft rejection.…”