Pemphigus vulgaris and focal segmental glomerulosclerosis (FSGS): First reported case and a review of the literature

• Published in: Clinical case reports Vol. 11; no. 7; pp. e7716 - n/a
• Main Authors: Sadeghzadeh‐Bazargan, Afsaneh, Amouzegar, Atefeh, Abolhasani, Maryam, Dehghani, Abbas, Goodarzi, Azadeh, Rahimi, Seyyedeh Tahereh
• Format: Journal Article
• Language: English
• Published: England John Wiley & Sons, Inc 01.07.2023; John Wiley and Sons Inc; Wiley
• Subjects: Antibodies; Autoimmune diseases; Biopsy; Case Report; Case reports; Cell adhesion & migration; Creatinine; Edema; Editing; focal segmental glomerulosclerosis; FSGS; Hematuria; Histopathology; immunobullous disease; Kidney diseases; Literature reviews; MCD; Medical research; minimal change disease; Nephrology; nephropathy; nephrotic syndrome; Pathogenesis; Pathology and Laboratory Medicine; Patients; pemphigus vulgaris; renal disease; review; Skin diseases; Steroids; Transplantation; Urology/Andrology; Writing; immunobullous disease; pemphigus vulgaris; PV; review; FSGS; case report; nephropathy; minimal change disease; focal segmental glomerulosclerosis; nephrotic syndrome; renal disease; MCD
• ISSN: 2050-0904; 2050-0904
• DOI: 10.1002/ccr3.7716

Key Clinical Message There may be a connection between pemphigus vulgaris and nephrotic syndrome, as evidenced by the occurrence of focal segmental glomerulosclerosis in our pemphigus vulgaris patient and reviewing relevant literature. Therefore, if a patient with pemphigus vulgaris presents with bilateral lower extremity edema or proteinuria detected during urinalysis, it could indicate involvement of the kidneys. Pemphigus vulgaris is a type of autoimmune blistering disease characterized by the presence of IgG autoantibodies against desmogleins 3 and 1. It is important to evaluate potential autoimmune associations in patients with pemphigus vulgaris so that appropriate laboratory and physical examinations can be performed to monitor for any increased risk of other autoimmune disorders. This case report describes a 55‐year‐old woman who presented with oral and axillary erosions, which were diagnosed as pemphigus vulgaris based on skin histopathology and immunofluorescence. During follow‐up, the patient was found to have proteinuria, which led to referral to a nephrologist. The patient was diagnosed with nephrotic syndrome and minimal change disease after a biopsy. Despite treatment, the patient's proteinuria persisted and serum creatinine levels increased, leading to a second biopsy which confirmed the diagnosis of focal segmental glomerulosclerosis. This study reports on the first case of pemphigus vulgaris with focal segmental glomerulosclerosis and reviews the literature on the co‐occurrence of acquired immunobullous diseases and nephrotic syndrome of any kind. Normal glomerulus with a delicate basement membrane.