J Korean Rheum Assoc.  2009 Mar;16(1):38-42. 10.4078/jkra.2009.16.1.38.

A Case of Henoch-Schonlein Purpura Associated with Idiopathic Thrombocytopenic Purpura

• Sung JM
• Suh CH
• Kim HA
• Kim MJ
• Lee JW

• KMID: 2202139
• DOI: http://doi.org/10.4078/jkra.2009.16.1.38

Abstract

Henoch-Schonlein purpura (HSP) is rarely associated with other autoimmune diseases. We experienced a case of HSP that was associated with idiopathic thrombocytopenic purpura (ITP). A 65-year old male patient complained of fever, purpura, generalized edema and arthralgia. He was diagnosed with HSP and his condition was improved with high-dose corticosteroid treatment. In 12 weeks, the symptoms of HSP were improving, however a thrombocytopenia developed. There was no reasonable cause for thrombocytopenia on the evaluation. He was diagnosed with ITP after bone marrow biopsy. Due to the refractory thrombocytopenia, which didn't respond to other medical treatments, we then performed splenectomy and the patient showed a good response. We report here on the first case of HSP and ITP, and we include a review of the related literature.

Keyword

Henoch-Schonlein purpura; Idiopathic thrombocytopenic purpura; Splenectomy

MeSH Terms

Arthralgia
Autoimmune Diseases
Biopsy
Bone Marrow
Edema
Fever
Humans
Male
Purpura
Purpura, Schoenlein-Henoch
Purpura, Thrombocytopenic, Idiopathic
Splenectomy
Thrombocytopenia

Figure

• 

  Fig. 1. (A) The sigmoidoscopic view shows the erythematous erosions in the sigmoid colon. (B) Leukoclastic vasculitis was noted with hemorrhage of the lamina propria.

• 

  Fig. 2. The renal biopsy revealed slight expansion of mesangial matrix with mild mesangial cell proliferation.

Reference

References

1. Saulsbury FT. Henoch-Schönlein purpura in children: report of 100 patients and review the literature. Medicine. 1999; 78:395–409.

2. Tancrede-Bohin E, Ochonisky S, Vignon-Pennamen MD, Flaguel B, Morel P, Rybojad M. Schönlein-Henoch purpura in adult patients. Predictive factors for IgA glomerulonephritis in a retrospective study of 57 cases. Arch Dermatol. 1997; 133:438–42.

3. Kwon EH, Kim SJ, Na MA, Jung YS, Lee DW, Lee SB, et al. A clinicopathological study of the adult Henoch-Schönlein Purpura. Korean J Intern Med. 2003; 65:323–34.

4. Cheon HW, Rho YA, Kwak HJ, Yoo KH, Kim SK. Clinical Study on Henoch-Schönlein Purpura. Korean J Nephrol. 1994; 13:560–8.

5. Blanco R, Martinez-Taboada VM, Rodriguez-Valverde V, Garcia-Fuentes M, Gonzalez-Gay MA. Henoch-Schönlein purpura in adulthood and childhood: two different expressions of the same syndrome. Arthritis Rheum. 1997; 40:859–64.

6. Choong CK, Beasley SW. Intraabdominal manifestations of Henoch-Schönlein purpura. J Paediatr Child Health. 1998; 34:405–9.

7. Stewart M, Savage JM, Bell B, McCord B. Long-term renal prognosis of Henoch-Schönlein purpura in an unselected childhood population. Eur J Pediatr. 1988; 147:113–5.

8. Lee YK, Jo SK, Shin JH, Kim YS, Cha DR, Cho WY, et al. One Case of Henoch-Schönlein Purpura associated with Thrombotic Thrombocytopenic Purpura. Koeran J Nephrol. 1997; 3:591–5.

9. Kaneko K, Igarashi J, Suzuki Y, Niijima S, Ishimoto K, Yabuta K. Carbamazepine-induced thrombocytopenia and leucopenia complicated by Henoch-Schönlein purpura symptoms. Eur J Pediatr. 1993; 152:769–70.

10. Krause I, Garty BZ, Davidovits M, Cleper R, Tamary H, Rosenmann E, et al. Low serum C3, leukopenia, and thrombocytopenia: unusual features of Henoch-Schonlein purpura. Eur J Pediatr. 1999; 158:906–9.

11. Weiss JH, Bhathena DB, Curtis JJ, Lucas BA, Luke RG. A possible relationship between Henoch-Schön-lein syndrome and IgA nephropathy (Berger's disease). An illustrative case. Nephron. 1978; 22:582–91.

12. Morino M, Inami K, Shibuya A, Sasaki N. IgA nephropathy and idiopathic thrombocytopenic purpura with splenectomy: a case report. Pediatr Nephrol. 1994; 8:345–6.

13. Watanabe M, Fujimoto T, Iwano M, Shiiki H, Nakamura S. Report of a patient of primary Sjögren syndrome, IgA nephropathy and chronic idiopathic thrombocytopenic purpura. Nihon Rinsho Meneki Gakkai Kaishi. 2002; 25:191–8.

14. Ryu KH, Chae YT, Ko EH, Moon JH, Hong SK, Shim JY, et al. A Case of the Ankylosing Spondylitis presented with Henoch-Schönlein Purpura. Korean J Intern Med. 2005; 69:953–63.

15. Kim SH, Chung JR, Kang EH, Im CH, Lee EY, Lee YJ, et al. A Case of Henoch-Schönlein Purpura in a Patient with Rheumatoid Arthritis and Chronic Lymphocytic Thyroiditis. J Korean Rheum Assoc. 2007; 14:171–5.