title: Immunoglobulin A nephropathy in association with generalized inflammatory peeling skin syndrome.
abstract: We describe an 8-year-old girl born to second-degree consanguineous parents with complaints of recurrent episodes of hematuria for 6 months. She had generalized peeling of the skin since birth and recurrent purulent cutaneous infections. The clinical presentation and histopathology of the skin biopsy specimen were consistent with the inflammatory variant of peeling skin syndrome (PSS). She also had a single ventricle with pulmonary stenosis, for which a bidirectional Glenn shunt had been placed. The renal biopsy specimen showed immunoglobulin A (IgA) nephropathy. She responded well to enalapril and steroids, with a decrease in proteinuria. IgA nephropathy has not been previously reported in PSS. Complications such as IgA nephropathy in children with PSS would help to further delineate the diverse clinical presentations and the clinical course of this rare dermatosis. We discuss the mechanisms that could explain this hitherto unreported association.
authors.affiliation: Department of Pediatrics, Jawaharlal Institute of Postgraduate Medical Education and Research, Pondicherry, India.
authors.forename: Rangan
authors.initials: R
authors.lastname: Srinivasaraghavan
authors.forename: Sriram
authors.initials: S
authors.lastname: Krishnamurthy
authors.forename: Rumesh
authors.initials: R
authors.lastname: Chandar
authors.forename: Subramanian
authors.initials: S
authors.lastname: Mahadevan
authors.forename: Laxmisha
authors.initials: L
authors.lastname: Chandrashekar
authors.forename: Nachiappa Ganesh
authors.initials: NG
authors.lastname: Rajesh
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use*; D001707:Biopsy, Needle; D002648:Child; D003873:Dermatitis, Exfoliative / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D003879:Dermatologic Agents / Q000627:therapeutic use; D004656:Enalapril / Q000627:therapeutic use*; D005260:Female; D005922:Glomerulonephritis, IGA / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D006417:Hematuria / Q000175:diagnosis / Q000209:etiology; D006801:Humans; D007150:Immunohistochemistry; D011379:Prognosis; D035583:Rare Diseases; D018570:Risk Assessment; D012720:Severity of Illness Index; D012873:Skin Diseases, Genetic / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D016896:Treatment Outcome
chemical_list: D000305:Adrenal Cortex Hormones; D003879:Dermatologic Agents; D004656:Enalapril