Henoch-Schönlein Purpura (IgA Vasculitis) in Adults

  • Ana Isabel Gouveia Interna do Internato Complementar de Dermatologia e Venereologia/Resident, Dermatology and Venereology, Clínica Universitária de Dermatologia, Hospital de Santa Maria (CHLN), Lisboa, Portugal
  • Leonor Lopes Interna do Internato Complementar de Dermatologia e Venereologia/Resident, Dermatology and Venereology, Clínica Universitária de Dermatologia, Hospital de Santa Maria (CHLN), Lisboa, Portugal
  • João Pedro Freitas Assistente Hospitalar Graduado de Dermatologia e Venereologia/Graduated Consultant, Dermatology and Venereology, Clínica Universitária de Dermatologia, Hospital de Santa Maria (CHLN), Lisboa, Portugal
Keywords: Adult, Immunoglobulin A, Purpura, Schoenlein-Henoch, Vasculitis, Leukocytoclastic, Cutaneous

Abstract

Henoch-Schönlein purpura is a small vessel vasculitis mediated by the deposition of IgA immune complexes. It is characterized by palpable purpura, arthralgia / arthritis, abdominal and renal involvement. Its etiology remains unknown, but various stimuli have been proposed to trigger this pathology, namely infectious agents, drugs and tumor antigens. There is also a possible association with malignancy and auto-immune diseases. Henoch-Schönlein purpura occurs mostly in children and is rare in adults. In the pediatric population the disease is generally self-limited, whereas in adults it is associated with a worse prognosis and a greater number of complications. This article reviews the main aspects of adult Henoch-Schönlein purpura, highlighting its particularities and specificities.

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References

Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 2013; 65:1-11.

Jithpratuck W, Elshenawy Y, Saleh H, Youngberg G, Chi DS, Krishnaswany G. The clinical implications of adult-

-onset Henoch-Schönelin purpura. Clin Mol Allergy. 2011; 9:9.

Fervenza FC. Henoch-Schönlein purpura nephritis. Int J Dermatol. 2003; 42:170-7.

Yang YH, Yu HH, Chiang BL. The diagnosis and classification of Henoch-Schönlein purpura: an updated review.

Autoimmun Rev. 2014; 13:355-8.

Zurada JM, Ward KM, Grossman ME. Henoch-Schönlein purpura associated with malignancy in adults. J Am Acad Dermatol. 2006; 55:S65-70.

Mitsui H, Shibagaki N, Kawamura T, Matsue H, Shimada S. A clinical study of Henoch-Schönlein purpura associated with malignancy. J Eur Acad Dermatology Venereol. 2009; 23:394–401.

Nishiya K, Oosaki F, Nakamura T, Hashimoto K, Kariya K, Seike M, et al. Rheumatoid arthritis associated with

Henoch-Schönlein purpura. Clin Exp Rheumatol. 2000; 18:653-4.

Bernardino V, Mendes-Bastos P, Rodrigues A, Riso N. IgA vasculitis (formerly Henoch-Schönlein purpura) in an

adult with systemic lupus erythematosus. BMJ Case Rep. 2015; 9.

Lu S, Liu D, Xiao J, Yuan W, Zhang X, Zhang J, et al. Comparison between adults and children with Henoch Schönlein purpura nephritis. Pediatr Nephrol. 2015; 30:791-6.

Wu SH, Liao PY, Chen XQ, Yin PL, Dong L. Add-on therapy with montelukast in the treatment of Henoch-Schönlein purpura. Pediatr Int. 2014; 56:315-22.

Pillebout E, Verine J. Purpura rhumatoïde de l’adulte. Rev Med Interne. 2014; 35:372-81.

Duffy TN, Genta M, Moll S, Martin PY, Gabay C. Henoch-Schönlein purpura following etanercept treatment

of rheumatoid arthritis. Clin Exp Rheumatol. 2006; 24:S106.

Rahman FZ, Takhar GK, Roy O, Shepherd A, Bloom SL, McCartney SA. Henoch-Schönlein purpura complicating adalimumab therapy for Crohn’s disease. World J Gastrointest Pharmacol Ther. 2010; 1:119-22.

Song Y, Shi YH, He C, Liu CQ, Wang JS, Zhao YJ, et al. Severe Henöch-Schonlein purpura with infliximab for ulcerative colitis. World J Gastroenterol. 2015; 21:6082-7.

Magro CM, Crowson AN. A clinical and histologic study of 37 cases of immunoglobulin A-associated vasculitis.

Am J Dermatopathol. 1999; 21:234-40.

Hughes RA, Bottomley DM, Keat AC, Drury A. Henoch-Schönlein purpura occurring in association with carcinoma of the breast. Eur J Med. 1993; 2:310-2.

López-Mejías R, Genre F, Pérez BS, Castañeda S, Ortego-Centeno N, Llorca J, et al. HLA-DRB1 association with Henoch-Schönlein purpura. Arthritis Rheumatol. 2014 [in press]

López-Mejías R, Genre F, Pérez BS, Castañeda S, Ortego-Centeno N, Llorca J, et al. Association of HLA-B*41:02 with Henoch-Schönlein purpura (IgA Vasculitis) in Spanish individuals irrespective of the HLA-DRB1 status. Arthritis Res Ther. 2015; 17:102.

Pillebout E, Thervet E, Hill G, Alberti C, Vanhille P, Nochy D. Henoch-Schönlein purpura in adults: outcome and prognostic factors. J Am Soc Nephrol. 2002; 13:1271-8.

Amoli MM, Thomson W, Hajeer AH, Calviño MC, Garcia-Porrua C, Ollier WE, et al. Interleukin 8 gene polymorphism is associated with increased risk of nephritis in cutaneous vasculitis. J Rheumatol. 2002; 29:2367-70.

Hara Y, Tajiri T, Matsuura K, Hasegawa A. Acute scrotum caused by Henoch-Schönlein purpura. Int J Urol. 2004; 11:578-80.

Modi S, Mohan M, Jennings A. Acute Scrotal Swelling in Henoch-Schönlein purpura: Case Report and Review of the Literature. Urol Case Rep. 2016; 6:9-11.

Nadrous HF, Yu AC, Specks U, Ryu JH. Pulmonar involvement in Henoch-Schönlein purpura. Mayo Clin Proc.

; 79:1151-7.

Fielding RE, Hawkins CP, Hand MF, Heath PD, Davies SJ. Seizures complicating adult Henoch-Schönlein purpura. Nephrol Dial Transplant. 1998; 13:761-2.

Lutz HH, Ackermann T, Krombach GA, Gröne HJ, Rauen T, Floege J, et al. Henoch-Schönlein purpura complicated by cardiac involvement: case report and review of the literature. Am J Kidney Dis. 2009; 54:e9-15.

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

De Castro SM, Joosse P, Unlü C, Steller EP. Henoch-Schönlein disease localized in the appendix. Indian J

Pediatr. 2013; 80:1047-9.

Linskey KR, Kroshinsky D, Mihm MC Jr, Hoang MP. Immunoglobulin-A-associated small-vessel vasculitis: a 10- year experience at the Massachusetts General Hospital. J Am Acad Dermatol. 2012; 66:813-22.

Takeuchi S, Soma Y, Kawakami T. IgM in lesional skin of adults with Henoch-Schönlein purpura is an indication of renal involvement. J Am Acad Dermatol. 2010; 63:1026-9.

Ozen S, Pistorio A, Iusan SM, Bakkaloglu A, Herlin T, Brik R, et al. EULAR/PRINTO/PRES criteria for Henoch-Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: Final classification criteria. Ann Rheum Dis. 2010; 69:798-806.

Audemard-Verger A, Pillebout E, Guillevin L, Thervet E, Terrier B. IgA vasculitis (Henoch-Shönlein purpura) in

adults: Diagnostic and therapeutic aspects. Autoimmun Rev. 2015; 14:579-85.

Sarma PS. Dapsone in Henoch-Schönlein purpura. Postgrad Med J. 1994; 70:464-5.

Ronkainen J, Koskimies O, Ala-Houhala M, Antikainen M, Merenmies J, Rajantie J, et al. Early prednisone

therapy in Henoch-Schönlein purpura: a randomized, double-blind, placebo-controlled trial. J Pediatr. 2006;

:241-7.

Foster BJ, Bernard C, Drummond KN, Sharma AK. Effective therapy for severe Henoch-Schönlein purpura nephritis with prednisone and azathioprine: a clinical and histopathologic study. J Pediatr. 2000; 136:370-5.

Ohtsuka T. Successful oral cyclosporin therapy for Henoch-Schönlein purpura nephropathy. J Dermatol.

; 36:314-6.

Han F, Chen LL, Ren PP, Le JY, Choong PJ, Wang HJ, et al. Mycophenolate mofetil plus prednisone for inducing

remission of Henoch-Schönlein purpura nephritis: a retrospective study. J Zhejiang Univ Sci B. 2015; 16:772-9.

Pillebout E, Alberti C, Guillevin L, Ouslimani A, Thervet E; CESAR study group. Addition of cyclophosphamide

to steroids provides no benefit compared with steroids alone in treating adult patients with severe Henoch

Schönlein purpura. Kidney Int. 2010; 78:495-502.

Rech J, Fuchs F, Kallert S, Hueber AJ, Requadt C, Manger B, et al. Plasmapheresis therapy in an elderly patient with rapidly progressive Henoch-Schönlein purpura with disseminated organ involvement. Clin Rheumatol. 2007; 26:112-4.

Pindi Sala T, Michot J, Snanoudj R, Dollat M, Estève E, Marie B, et al. Successful outcome of a corticodependent Henoch-Schönlein purpura adult with rituximab. Case Rep Med. 2014; 2014:619218.

Jauhola O, Ronkainen J, Koskimies O, Ala-Houhala M, Arikoski P, Hölttä T, et al. Outcome of Henoch-Schönlein purpura 8 years after treatment with a placebo or prednisone at disease onset. Pediatr Nephrol. 2012; 27:933-9.

Published
2016-10-15
How to Cite
Gouveia, A. I., Lopes, L., & Freitas, J. P. (2016). Henoch-Schönlein Purpura (IgA Vasculitis) in Adults. Journal of the Portuguese Society of Dermatology and Venereology, 74(3), 265-271. https://doi.org/10.29021/spdv.74.3.596
Section
Case Reports