Diagnosis
Henoch-Schönlein purpura
A benign self-limited small-vessel vasculitis characterized by purpura, arthritis, abdominal pain, and hematuria.
Also known as: IgA vasculitis
Etiology
Cause [aqs]
Genetic susceptibility (association with specific HLA-subgroups)
Triggered by:
Infection: Group A streptococcus, coxsackie virus, hepatitis virus A/B, mycoplasma, parvovirus B19, subacute bacterial endocarditis, helicobacter pylori, yersinia, shigella, salmonella, brucellosis, legionella, campylobacter, varicella, parainfluenza virus, influenza virus, respiratory syncytial virus, rotavirus, adenoviruses, CMV, EBV)
Vaccination
Foods
Insect bites
Drugs acting as the antigen and causing an autoimmune reaction
Pathophysiology [aqs]
Mucous membranes of the salivary glands, lungs, and GI tract produce IgA
Antigenic exposure (infection, drugs) --> triggers the formation of IgA-antibody immune complexes
IgA complexes are deposited in the small vessels (usually capillaries) of the skin, joints, kidneys, and GI tract
Proinflammatory mediators --> complement system activation:
Purpura and petechiae
Gastrointestinal hemorrhage
IgA nephropathy
Complications [qgi][aqs]
Renal Failure
Proteinuria
Hematuria
Nephrotic syndrome
Intussusception
Gastrointestinal bleeding
Bowel infarction
Bowel perforation
CNS bleeding
Seizures
Neuropathy
Pleural effusion
Pulmonary hemorrhage
Testicular torsion
Epidemiology
Incidence per 100.000 [2is][syp][m8r][qgi][cwt][e98]
Symptoms & findings
Symptoms
Anorexia, Aphasia, Arthralgia, Ataxia, Decreased consciousness, Diarrhea, Dysmenorrhea, Edema, Fatigue, Fever, Headache, Hematemesis, Hematochezia, Hematuria, Hepatomegaly, Melena, Nausea, Paresis, Seizure, Vomiting
Clinical findings
ANA, Bowel wall thickening, Elevated Creatinine, Elevated D-dimer, Elevated IgA, Elevated Sedimentation Rate, Eosinophilia, Leukocytosis, Proteinuria, Rheumatoid factor, Thrombocytosis
Anamneses
None listed.
Localized findings
Approach
Blood test
Urine analysis
Skin biopsy
Kidney biopsy
Abdominal x-ray
Ultrasound abdomen
Ultrasound testis
MR caput
Diagnostic criteria [gn5][2is]
Presence of purpura or petechiae with lower limb predominance plus at least one of the flowing four features:
Abdominal pain
Arthritis or arthralgia
Leukocytoclastic vasculitis or proliferativeglomerulonephritis with predominant deposition of IgA on histology
Renal involvement (haematuria, red blood cell casts or proteinuria)
Treatment
Henoch-Schönlein purpura spontaneously resolves in 94 percent of children and 89 percent of adults. [qgi]
Supportive treatment is the primary intervention
Rehydration with intravenous fluids
Pain management
Wound care for ulcerative skin lesions
Immunsuppresion:
Oral prednisone (1-2 mg/kg daily for two weeks) if abdominal and joint symptoms
High-dose steroids + immunosuppressants if severe renal involvement [fzw][qeu]
Plasmapheresis
High-dose intravenous pulse methylprednisolone
Azathioprine
Cyclophosphamide
Mycophenolate mofetil
Cyclosporine A
Rituximab
Dapsone
Proteinuria --> Angiotensin-converting enzyme inhibitors [aqs]
Differential diagnoses
Acute hemorrhagic edema of infancy, Acute pancreatitis, Appendicitis, Crohn disease, Disseminated intravascular coagulation, Encephalitis, Endocarditis, Epididymitis, Gastrointestinal obstruction, Glomerulonephritis, Hemolytic uremic syndrome, IgA nephropathy, Immune thrombocytopenic purpura, Intussusception, Meningitis, Polyarteritis nodosa, Renal failure, Rheumatic fever, Rocky mountain spotted fever, Systemic lupus erythematosus, Testicular torsion, Thrombotic thrombocytopenic purpura, Ulcerative colitis, Wegener's granulomatosis
References
[1] Roache-Robinson P, Killeen RB, Hotwagner DT. IgA Vasculitis (Henoch-Schönlein Purpura) [Updated 2023 Sep 26]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2025 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK537252/
[2] Reamy BV, Williams PM, Lindsay TJ. Henoch-Schönlein purpura. Am Fam Physician. 2009 Oct 1;80(7):697-704.
[3] Trnka P. Henoch-Schönlein purpura in children. J Paediatr Child Health. 2013 Dec;49(12):995-1003.
[4] Dolezalová P, Telekesová P, Nemcová D, Hoza J. Incidence of vasculitis in children in the Czech Republic: 2-year prospective epidemiology survey. J Rheumatol. 2004 Nov;31(11):2295-9.
[5] Lei WT, Tsai PL, Chu SH, Kao YH, Lin CY, Fang LC, Shyur SD, Lin YW, Wu SI. Incidence and risk factors for recurrent Henoch-Schönlein purpura in children from a 16-year nationwide database. Pediatr Rheumatol Online J. 2018 Apr 16;16(1):25.
[6] Gardner-Medwin JM, Dolezalova P, Cummins C, Southwood TR. Incidence of Henoch-Schönlein purpura, Kawasaki disease, and rare vasculitides in children of different ethnic origins. Lancet. 2002 Oct 19;360(9341):1197-202.
[7] Calviño MC, Llorca J, García-Porrúa C, Fernández-Iglesias JL, Rodriguez-Ledo P, González-Gay MA. Henoch-Schönlein purpura in children from northwestern Spain: a 20-year epidemiologic and clinical study. Medicine. 2001;80(5):279-290.
[8] Ozen S, Pistorio A, Iusan SM, Bakkaloglu A, Herlin T, Brik R, Buoncompagni A, Lazar C, Bilge I, Uziel Y, Rigante D, Cantarini L, Hilario MO, Silva CA, Alegria M, Norambuena X, Belot A, Berkun Y, Estrella AI, Olivieri AN, Alpigiani MG, Rumba I, Sztajnbok F, Tambic-Bukovac L, Breda L, Al-Mayouf S, Mihaylova D, Chasnyk V, Sengler C, Klein-Gitelman M, Djeddi D, Nuno L, Pruunsild C, Brunner J, Kondi A, Pagava K, Pederzoli S, Martini A, Ruperto N; Paediatric Rheumatology International Trials Organisation (PRINTO). 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 May;69(5):798-806.
[9] Saulsbury FT. Henoch-Schönlein purpura in children. Report of 100 patients and review of the literature. Medicine (Baltimore). 1999 Nov;78(6):395-409.
[10] Hetland LE, Susrud KS, Lindahl KH, Bygum A. Henoch-Schönlein Purpura: A Literature Review. Acta Derm Venereol. 2017 Nov 15;97(10):1160-1166.
[11] http://emedicine.medscape.com/article/984105 (2014-01-02); [Medscape]
[12] http://emedicine.medscape.com/article/780452 (2014-01-02); [Medscape]
[13] Penny K, Fleming M, Kazmierczak D, Thomas A. An epidemiological study of Henoch-Schönlein purpura. Paediatr Nurs. 2010 Dec;22(10):30-5.