Immunoglobulin A Nephropathy in Association with Generalized Inflammatory Peeling Skin Syndrome |
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| Authors: | Rangan Srinivasaraghavan M.D. Sriram Krishnamurthy M.D. Rumesh Chandar M.B.B.S Subramanian Mahadevan M.D. Ph.D. Laxmisha Chandrashekar M.D. Nachiappa Ganesh Rajesh M.D. |
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| Affiliation: | 1. Department of Pediatrics, Jawaharlal Institute of Postgraduate Medical Education and Research, Pondicherry, India;2. Department of Dermatology, Jawaharlal Institute of Postgraduate Medical Education and Research, Pondicherry, India;3. Department of Pathology, Jawaharlal Institute of Postgraduate Medical Education and Research, Pondicherry, India |
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| 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. |
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