The skin-kidney connection: bullous pemphigoid associated with acute allograft rejection and membranous nephropathy.

Bullous pemphigoid has been linked to allograft rejection, as well as membranous nephropathy in renal transplant recipients. Although there is a possibility of multiple distinct autoimmune processes, immune stimulation induced by allograft rejection or antibasement zone antibody interactions are possible mechanisms for the simultaneous skin and renal involvement.

There are a few case reports on the association between bullous pemphigoid and renal allograft rejection, as well as membranous nephropathy 1, 2, 3. Herein, we present a unique case of bullous pemphigoid associated with both acute cellular rejection and de novo membranous nephropathy in a renal transplant recipient.

A 63‐year‐old man with a history of end‐stage renal disease, status post‐living‐related kidney transplant 5 years ago, was admitted to the hospital for worsening skin rash that started 3 weeks before and acute kidney injury. He has been compliant with his immunosuppressant therapy. Physical examination revealed multiple scattered bullae with clear fluid and erosions with a collarette of scale on the neck, around nipples, back, and limbs (Fig. 1). His serum creatinine at presentation was 7.3 mg/dL [ref: 0.5–1.1] with a urine albumin/creatinine ratio of ~550 mg/g [ref: <30]. The baseline serum creatinine 4 months ago was ~1.5 mg/dL. Serologic tests for BK virus, cytomegalovirus, Herpes simplex, and Varicella zoster were negative. Skin biopsy was consistent with bullous pemphigoid. Renal allograft biopsy demonstrated acute cellular rejection with membranous nephropathy (Fig. 2), and approximately 50 percent interstitial fibrosis and tubular atrophy. His renal function did not show significant improvement after 1 month, despite treatment with pulse corticosteroids and antithymocyte globulin. Further immunosuppression was not attempted due to the severity of interstitial fibrosis and tubular atrophy on the repeat renal biopsy. His skin lesions improved with high‐dose corticosteroid therapy.

Figure 1

Lesions of bullous pemphigoid on patient's (A) upper extremity and (B) the chest.

Figure 2

Renal allograft biopsy demonstrating (A) interstitial inflammatory infiltrate and vasculitis suggestive of acute cellular rejection [PAS 40×], (B) focal thickening of the peripheral capillary loops [PAS 63×], (C) granular IgG staining of the peripheral capillary loops [Immunofluorescence 63×], (D) multifocal intramembranous electron‐dense deposits (arrows) and thickened glomerular capillary walls [Electron Microscopy].

Informed Consent

Informed consent has been obtained for the publication of this clinical image.

Authorship

All authors: made substantial contribution to the preparation of this manuscript and approved the final version for submission. AK: procured the clinical images and drafted the manuscript. WLC: provided the pathology images and relevant input. OAO: drafted the manuscript. AHS: reviewed and revised the manuscript for critically important intellectual content as the attending physician on the case.

Conflict of Interest

The authors have declared that no conflict of interest exists.