title: IgA nephropathy in a patient with dominant dystrophic epidermolysis bullosa.
abstract: Dystrophic epidermolysis bullosa (DEB) is a rare and severe hereditary dermatosis. On the other hand, IgA nephropathy is the most common form of glomerulonephritis in childhood and adults, and clinically characterized by microhematuria and proteinuria and histologically by deposition of immunoglobulin A in mesangial lesions. Several renal complications of recessive DEB including IgA nephropathy and amyloidosis have been reported. However, there have been no reports on dominant DEB associated with IgA nephropathy. We report here for the first time a 17-year-old girl with dominant DEB associated with IgA nephropathy. The patient has suffered from episodes of urinary, upper airway, and skin infections. At 17 years of age, proteinuria and hematuria were detected, with a high value of serum IgA. Renal biopsy was performed, and immunofluorescence microscopic examination revealed segmental deposits of IgA in mesangial lesions, with many glomeruli exhibiting diffuse segmental mesangial-proliferative glomerulonephritis. We diagnosed dominant DEB associated with IgA nephropathy on the basis of proteinuria, hematuria, and deposits of IgA in mesangial lesions on immunofluorescence microscopic examination, and diffuse segmental mesangial-proliferative glomerulonephritis. These findings suggest that repeated skin infections might have contributed to the pathogenesis of IgA nephropathy in this patient.
authors.affiliation: Department of Pediatrics, Fukushima Medical University School of Medicine, Fukushima, Japan. tomo@fmu.ac.jp
authors.forename: Yukihiko
authors.initials: Y
authors.lastname: Kawasaki
authors.forename: Masato
authors.initials: M
authors.lastname: Isome
authors.forename: Kei
authors.initials: K
authors.lastname: Takano
authors.forename: Kazuhide
authors.initials: K
authors.lastname: Suyama
authors.forename: Tomoko
authors.initials: T
authors.lastname: Imaizumi
authors.forename: Hiromi
authors.initials: H
authors.lastname: Matsuura
authors.forename: Kiyoko
authors.initials: K
authors.lastname: Ichii
authors.forename: Koichi
authors.initials: K
authors.lastname: Hashimoto
authors.forename: Mitsuaki
authors.initials: M
authors.lastname: Hosoya
mesh_terms: D000293:Adolescent; D001706:Biopsy; D003872:Dermatitis / Q000150:complications; D016108:Epidermolysis Bullosa Dystrophica / Q000150:complications*; D005260:Female; D005922:Glomerulonephritis, IGA / Q000209:etiology* / Q000473:pathology; D006801:Humans; D011507:Proteinuria / Q000150:complications / Q000473:pathology; D012141:Respiratory Tract Infections / Q000150:complications; D014552:Urinary Tract Infections / Q000150:complications