Case Report
A purple rash
Henry G Cheng, Caitlin Gomez, Sarah Khan, Soma Wali
Lancet 2011; 378: 1526 Department of Internal Medicine, UCLA Medical Center/UCLA-Olive View, Los Angeles, CA, USA (H G Cheng BA, C Gomez MD, S Khan MD, S Wali MD) Correspondence to: Mr Henry G Cheng, Department of Internal Medicine, UCLA David Geen School of Medicine, Box 951720, 12-159 CHS, Los Angeles, CA 90095-1720, USA hcheng@[Link]
In July, 2010, a 60-year-old white woman with uncontrolled type 2 diabetes mellitus presented with pain and swelling of the hands, wrists, and feet, and a purpuric rash on both heels. She had a 10-year history of diabetes, hypertension, and hyperlipidaemia. Before admission, her baseline creatinine concentration was 126 mol/L (normal <88 mol/L for women), GFR was 40 mL/min, 2+ urine protein (10 g/L ), and haemoglobin A11c (HbA1c ) was 68 mmol/mol. Her medications included: atorvastatin, metoprolol, benazepril, furosemide, glargine, and glipizide. While our patient was in the emergency department, a petechial rash developed on her forearms and feet, and a vasculitic rash with tender purpuric lesions on her inner thighs. She also reported new-onset cokecoloured urine. There was marked symmetrical polyarticular synovitis in both hands, and oedema with tenderness to palpation in both feet. Initial laboratory tests showed a creatinine concentration of 178 mol/L, GFR of 30 mL/min, ESR of 62 mm/h (normal <30 mm/h), C-reactive protein of 229 mg/L (normal <10 mg/L), and HbA1c of 112 mmol/mol (normal <42 mmol/mol). Urinalysis showed copious red and white blood cells. A vasculitic aetiology was high on our dierential diagnosis list. We suspected Henoch-Schnlein purpura when her rash progressed to a conuent, purpuric appearance and spread to the buttocks. Extensive autoimmune and infectious disease investigations showed a raised titre of anti-streptolysin O. Biopsy samples of the inner thigh lesions showed leukocytoclastic vasculitis with focal deposits of IgG, IgA, IgM, and C3. A renal biopsy sample showed diabetic glomerulosclerosis, IgA nephropathy, arteriolar nephrosclerosis, mild intimal brosis, basement membrane thickening, and podocyte eacement. On the basis of these ndings, our patient was treated with methylprednisolone, but with little improvement of her rash and joint swelling. On hospital
day 5, mycophenolate mofetil and intravenous immunoglobulin were added and the rash and joint swelling resolved, but her creatinine concentration was 394 mol/L and GFR was 16 mL/min. Despite high-dose triple therapy, her renal function worsened and haemodialysis was started. After discharge, she had haemodialysis three times per week. At last follow-up, in May, 2011, her condition was stable. 5080% of adults with Henoch-Schnlein purpura develop nephritis, of whom 30% progress to chronic renal insuciency.1 To our knowledge, this is the rst described case of refractory Henoch-Schnlein purpura nephritis in an elderly diabetic patient treated with a novel triple combination therapy of methylprednisolone, mycophenolate mofetil, and immunoglobulin therapy chosen because our patients renal function was deteriorating rapidly. Studies have shown eective treatment of steroid-resistant Henoch-Schnlein purpura with cyclophosphamide, mycophenolate mofetil, or plasmapheresis.25 In a case series, mycophenolate mofetil (an immunosuppressant used in renal transplantation), showed promise in treating refractory Henoch-Schnlein purpura.5 No large studies have demonstrated an eective treatment for the prevention of renal failure in patients with this disorder. Despite combination treatment, our patient is likely to have had underlying diabetic nephropathy compounding the renal injury from IgA deposition. Whether she would have beneted from plasmapheresis is unclear, but it might be considered for similar cases. Cutaneous and joint manifestations seem to respond well to steroid and immunosuppressant therapy but prevention of renal insuciency remains dicult. In adult HenochSchnlein purpura, practitioners should be aware of the high incidence of renal involvement and its devastating outcomes if not managed aggressively.
Contributors All authors looked after the patient and wrote the report. References 1 Pillebout E, Thervet E, Hill G, Alberti C, Vanhille P, Nochy D. Henoch-Schonlein purpura in adults: outcome and prognostic factors. J Am Soc Nephrol 2002; 13: 127178. 2 Donghi D, Schanz U, Sahrbacher U, et al. Life-threatening or organ-impairing Henoch-Schonlein purpura: plasmapheresis may save lives and limit organ damage. Dermatology 2009; 219: 16770. 3 Chen TCF, Lee C, Huang SC, Chen JB, Hsu KT. Successful treatment of crescentic glomerulonephritis associated with adult onset Henoch-Schonlein purpura by double-ltration plasmapheresis. Clin Nephrol 2004; 61: 21316. 4 Pillebout E, Alberti C, Guillevin L, Ouslimani A, Thervet E. Addition of cyclophosphamide to steroids provides no benet compared with steroids alone in treating adult patients with severe Henoch-Schonlein purpura. Kidney Int 2010; 78: 495502. 5 Nikibakhsh AA, Mahmoodzadeh H, Karamyyar M, et al. Treatment of complicated Henoch-Schonlein purpura with mycophenolate mofetil: a retrospective case series report. Int J Rheumatol 2010; 2010: 254316.
Figure: Purpuric lesions of the left inner thigh
1526
[Link] Vol 378 October 22, 2011