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A Case of Henoch-Schönlein Purpura with Fulminant Complications and Its Long-term Outcome

  • Lee, Dong Hyun (Department of Pediatrics, Ajou University School of Medicine) ;
  • LEE, Eun-So (Department of Dermatology, Ajou University School of Medicine) ;
  • Hong, Jeong (Department of Pediatric Surgery, Ajou University School of Medicine) ;
  • Park, Kwang-Hwa ;
  • Pai, Ki Soo (Department of Pediatrics, Ajou University School of Medicine)
  • 투고 : 2019.10.02
  • 심사 : 2019.10.23
  • 발행 : 2019.10.31

초록

Henoch-$Sch{\ddot{o}}nlein$ purpura (HSP) is a systemic vasculitis characterized by purpura, arthritis, abdominal pain, and nephritis. Gastrointestinal involvement can manifest as pain, intussusception, intestinal bleeding, and intestinal perforation. We report a case of fulminant HSP at an age of eight in 1994, with multiple complications of intra-thoracic bleeding, massive intestinal perforation, nephritis, and various skin rashes. The brisk bleeding findings of intestinal on Technetium-99m-labeled red blood cell scan ($^{99m}Tc$ RBC scan) were well matched to those of the emergency laparotomy and the resected intestine. The patient's abdominal conditions improved gradually but nodular skin eruptions developed newly apart from improving preexisting lower limb rashes and the urine findings continued abnormal, so skin and kidney biopsy were done for the diagnosis. After cyclosporine therapy, skin eruptions and urine findings returned to normal gradually. On a follow-up after 25 years in 2019, the patient is 33-year-old, healthy without any abnormality on blood chemistries and urine examination.

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참고문헌

  1. Trnka P. Henoch-Schonlein purpura in children. J Paediatr Child Health 2013;49:995-1003. https://doi.org/10.1111/jpc.12403
  2. Choong CK, Beasley SW. Intra-abdominal manifestations of Henoch-Schonlein purpura. J Paediatr Child Health 1998;34:405-9. https://doi.org/10.1046/j.1440-1754.1998.00263.x
  3. Lerkvaleekul B, Treepongkaruna S, Saisawat P, Thanachatchairattana P, Angkathunyakul N, Ruangwattanapaisarn N, et al. Henoch-Schonlein purpura from vasculitis to intestinal perforation: a case report and literature review. World J Gastroenterol 2016;22:6089-94. https://doi.org/10.3748/wjg.v22.i26.6089
  4. Gardner-Medwin JM, Dolezalova P, Cummins C, Southwood TR. Incidence of Henoch-Schonlein purpura, Kawasaki disease, and rare vasculitides in children of different ethnic origins. Lancet 2002;360:1197-202. https://doi.org/10.1016/S0140-6736(02)11279-7
  5. Ozen S, Ruperto N, Dillon MJ, Bagga A, Barron K, Davin JC, et al. EULAR/PReS endorsed consensus criteria for the classification of childhood vasculitides. Ann Rheum Dis 2006;65:936-41. https://doi.org/10.1136/ard.2005.046300
  6. Weiss PF, Klink AJ, Luan X, Feudtner C. Temporal association of streptococcus, staphylococcus, and parainfluenza pediatric hospitalizations and hospitalized cases of Henoch-Schonlein purpura. J Rheumatol 2010;37:2587-94. https://doi.org/10.3899/jrheum.100364
  7. Trapani S, Micheli A, Grisolia F, Resti M, Chiappini E, Falcini F, et al. Henoch-Schonlein purpura in childhood: epidemiological and clinical analysis of 150 cases over a 5-year period and review of literature. Semin Arthritis Rheum 2005;35:143-53. https://doi.org/10.1016/j.semarthrit.2005.08.007
  8. Saulsbury FT. Henoch-Schonlein purpura in children. Report of 100 patients and review of the literature. Medicine (Baltimore) 1999;78:395-409. https://doi.org/10.1097/00005792-199911000-00005
  9. Pabunruang W, Treepongkaruna S, Tangnararatchakit K, Chunharas A, Phuapradit P. Henoch-Schonlein purpura: clinical manifestations and long-term outcomes in Thai children. J Med Assoc Thai 2002;85:S1213-8.
  10. Shim JO, Han K, Park S, Kim GH, Ko JS, Chung JY. Ten-year Nationwide Populationbased Survey on the Characteristics of Children with Henoch-Schonlein Purpura in Korea. J. Korean Med Sci 2018;33:1-10. https://doi.org/10.3904/kjim.2017.377
  11. Katz S, Borst M, Seekri I, Grosfeld JL. Surgical evaluation of Henoch-Schonlein purpura. Experience with 110 children. Arch Surg 1991;126:849-53; discussion 53-4. https://doi.org/10.1001/archsurg.1991.01410310059008
  12. Kaku Y, Nohara K, Honda S. Renal involvement in Henoch-Schonlein purpura: a multivariate analysis of prognostic factors. Kidney Int 1998;53:1755-9. https://doi.org/10.1046/j.1523-1755.1998.00915.x
  13. Seo MK, Hong J, Yim HE, Pai KS. A Patient with Henoch-Schonlein Purpura with Intussusception and intractable Nephritis. Child Kidney Dis 2016;20:92-6. https://doi.org/10.3339/jkspn.2016.20.2.92
  14. Hahn D, Hodson EM, Willis NS, Craig JC. Interventions for preventing and treating kidney disease in Henoch-Schonlein Purpura (HSP). Cochrane Database Syst Rev 2015;(8):CD005128.
  15. Jauhola O, Ronkainen J, Koskimies O, Ala-Houhala M, Arikoski P, Holtta T, Jahnukainen T, Rajantie J, Ormala T, Nuutinen M. Clinical course of extrarenal symptoms in Henoch-Schonlein purpura: a 6-month prospective study. Arch Dis Child 2010;95:871-6. https://doi.org/10.1136/adc.2009.167874
  16. Rajagopala S, Shobha V, Devaraj U, D'Souza G, Garg I. Pulmonary hemorrhage in Henoch-Schonlein purpura: case report and systematic review of the english literature. Semin Arthritis Rheum 2013;42:391-400. https://doi.org/10.1016/j.semarthrit.2012.07.004