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Case 2

Case 2. M-36,Erythematous rash on abdomen H/o Alport’s syndrome Failed renal transplant on hemodialysis. Clinical presentation. Alport’s syndrome diagnosed in 1973 –progressed to ESRF Cadaveric renal transplant in 1992 CAPD commenced in 2005

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Case 2

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  1. Case 2 M-36,Erythematous rash on abdomen H/o Alport’s syndrome Failed renal transplant on hemodialysis

  2. Clinical presentation • Alport’s syndrome diagnosed in 1973 –progressed to ESRF • Cadaveric renal transplant in 1992 • CAPD commenced in 2005 • Peritonitis and subacute bowel obstruction necessitated a • 2nd Cadaveric renal transplant in 2010 • PD port removed in 2011

  3. Presenting Symptoms • Generalised abdominal pain • Weight loss • 20x15cm erythema in periumbilical area • All other investigations negative • No response to I/V antibiotics • Biopsy performed

  4. REACTIVE ANGIOENDOTHELIOMATOSIS

  5. RAE • Clinically- • Erythematous violaceous papules • Macules, nodules • Cellulitis like areas • Seen in abdominal areas, thighs and legs • Also seen in other sites

  6. RAE • Represents a hypersensitivity reaction • Different stimuli- cryoproteins and microorganisms • Vessel occlusion—hypoxaemia—endothelial proliferation • Reported in association with bacterial endocarditis and other renal diseases.

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