Pediatric Henoch-Schonlein Purpura Clinical Presentation

  • Author: Noah S Scheinfeld, MD, JD, FAAD; Chief Editor: Craig B Langman, MD   more...
 
Updated: Nov 23, 2010
 

History

Prais et al studied 267 children (56.7% males) who were hospitalized secondary to Henoch-Schoenlein purpura (HSP); 7 of the children (2.7%) had Henoch-Schoenlein purpura that resulted in hospitalization 2 or more times.[13] No specific risk factor for recurrence was determined. The mean age for the first recurrence in that subgroup was 3 years and 8 months (range, 10 mo to 7.4 y), and the mean age for the second recurrence was 5.03 years (2.2-10 y), with a mean lag time of 13.5 months ± 2.8 months (range, 2-26 mo). The duration of the recurrent clinical symptoms was 9-30 days. Resolution took more than 2 weeks in 72% of patients.

Associated conditions that precede Henoch-Schoenlein purpura include those listed in Causes.

Scrotal involvement is not uncommon in Henoch-Schoenlein purpura and may mimic testicular torsion, which must be excluded. Male patients may have associated inflammation and hemorrhage of the testes, appendix testes, spermatic cord, epididymis, or scrotal wall. True torsion is rare. Ha and Lee reported that neurologic symptoms, localized edema, and high serum C3 levels have a significant relationship with scrotal involvement in male patients with Henoch-Schoenlein purpura.[14]

GI symptoms can accompany the onset of Henoch-Schoenlein purpura or may develop later in the course of disease. Abdominal pain occurs in 35-85% of patients and is the third most common presenting symptom in Henoch-Schoenlein purpura. GI problems usually follow the onset of rash and joint pain. Multiple and recurrent intestinal perforations are an unusual complication of Henoch-Schoenlein purpura. In addition to abdominal pain, GI findings can include the following:

  • Nausea
  • Vomiting
  • Diarrhea with gross or occult blood
  • Hematemesis
  • Intussusception: This occurs in 2-3% of patients, and the lead point can be a submucosal hematoma.
  • Bowel infarction with or without perforation
  • Ileal stricture
  • Ileus with massive GI bleed

Arthralgias occur in 60-84% of patients with Henoch-Schoenlein purpura and most commonly affect the knees, ankles, and, less frequently, the wrists and fingers. True arthritis does not occur, and joint effusions are rare. Henoch-Schoenlein purpura leaves no permanent joint deformities.

In women, gynecologic symptoms can include painful menstruation.

Henoch-Schoenlein purpura can be accompanied by neurologic manifestations, particularly headaches. Ozkaya et al reported cerebral vasculitis in a child with Henoch-Schoenlein purpura and familial Mediterranean fever.[15]

In rare cases, Henoch-Schoenlein purpura can be associated with seizures, paresis, or coma. Other manifestations include altered mental status, apathy, hyperactivity, irritability, mood lability, somnolence, seizures (partial, complex partial, generalized, status epilepticus), and focal deficits (eg, aphasia, ataxia, chorea, cortical blindness, hemiparesis, paraparesis, quadriparesis).

Polyradiculoneuropathies (eg, brachial plexus neuropathy, Guillain-Barré syndrome) and mononeuropathies (eg, facial nerve, femoral nerve, peroneal nerve, sciatic nerve, ulnar nerve) may also occur.

The liver and gallbladder can be involved in Henoch-Schoenlein purpura. Hepatomegaly, hydrops of the gallbladder, and cholecystitis may ensue. These may contribute to a patient's abdominal pain. Acute appendicitis has been noted in patients with Henoch-Schoenlein purpura.

Skin involvement is usually purpura. Chan et al noted a case of Henoch-Schoenlein purpura presented as painful bullae on both legs.[16]

Acute hemorrhagic edema of infancy (AHEI) usually occurs in infants aged 4-24 months. AHEI often occurs after drug ingestion or infection. The onset of AHEI is dramatic, with acute palpable purpura, ecchymoses, and tender edema of the limbs and face. Fever, if present, remains mild. Infants remain hemodynamically stable. Dermatologic findings are notable for a cockade (medallionlike), rosette-shaped pattern of macular-papular-hemorrhagic lesions on the face, auricles, and extremities. The lesions usually appear in successive crops. The cockades display variable stages of evolution at any given time.

Subcutaneous edema is most common in infants. Urticaria, petechiae, and ear lobe necrosis are additional rare skin manifestations of AHEI. Visceral involvement is rare.

Renal pathology is the most important cause of morbidity and mortality in patients with Henoch-Schoenlein purpura. Renal involvement may precede skin manifestations (1-4% of patients) but is usually evident during the acute phase of the disease. In most cases, the severity of nephritis is not related to the extent of other Henoch-Schoenlein purpura manifestations.

Hematuria, usually microscopic, can be accompanied by mild-to-moderate proteinuria (< 2 g/d). Oliguria, hypertension, and azotemia are rarely present. Nephrotic syndrome (urinary protein excretion >40 mg/m2/h) can also occur. In most cases, histologic examination of the kidneys reveals mesangial proliferation that can be diffuse or focal and segmental. Resolution of the renal involvement is the focus in these patients.

Patients who present with hematuria and persistent proteinuria have an approximate 15% risk of developing renal failure. The risk may increase to 50% in patients with a nephrotic-nephritic syndrome.

Urinary complications include bladder-wall hematoma, calcified ureter, hydronephrosis, and urethritis.

Hemoptysis and hemarthroses can develop in patients who have bleeding abnormalities such as coagulopathy, factor VIII deficiency, vitamin K deficiency, or hypoprothrombinemia. They also probably include factor V Leiden, protein C deficiency, and protein S deficiency, but this has not been documented.

Next

Physical

Purpura of the skin is the most prominent physical finding in Henoch-Schoenlein purpura, but renal, GI, and joint manifestations are commonly present. Other manifestations have also been reported.

Henoch-Schoenlein purpura begins with a symmetrical erythematous macular rash on the lower extremities that quickly evolves into purpura. The rash may initially be confined to malleolar skin but usually extends to the dorsal surface of the legs, the buttocks, and the ulnar side of the arms. Within 12-24 hours, the macules evolve into purpuric lesions that are dusky red and have a diameter of 0.5-2 cm. The lesions may coalesce into larger plaques that resemble ecchymoses. Several cases of Henoch-Schoenlein purpura have been observed after varicella infections.

In children younger than 2 years, the clinical picture may be dominated by edema of the scalp, periorbital area, hands, and feet. This presentation is termed AHEI. The severity of edema is correlated with the severity of the vasculitis and not with the degree of proteinuria. However, the edema has been attributed to the enteric loss of protein.

Overview of renal findings

The most serious complication of Henoch-Schoenlein purpura is renal involvement, which occurs in 50% of older children but is serious in only approximately 10% of patients. In 80% of patients, renal involvement becomes apparent within the first 4 weeks of illness. Overall, 2-5% of patients progress to end-stage renal failure (ESRF). In one series, acute glomerular lesions, including mesangial hypercellularity, endocapillary proliferation, necrosis, cellular crescents, and leukocyte infiltration, were observed in 41%, 12%, 50%, 29%, and 32% of patients, respectively.[17] Only glomerular necrotizing lesions and cellular crescents correlated with the renal survival rate and were associated with clinically significant proteinuria and development of hypertension.

In a prospective study of 223 children with Henoch-Schoenlein purpura, Jauhola et al reported that 46% developed renal manifestations. The authors recommend that children with renal involvement be followed for more than 6 months. In addition, treatment with prednisone did not affect the renal manifestations.[18]

Henoch-Schoenlein purpura and IgA nephropathy

The relationship of Henoch-Schoenlein purpura and IgA nephropathy requires further definition. Whether they are the same or distinct entities remains unclear. Evidence of both their commonality and distinctiveness is presented herein.

The occurrence of extrarenal manifestations in IgA nephropathy is similar to those observed in Henoch-Schoenlein purpura.

IgA nephropathy has developed in patients with a history of Henoch-Schoenlein purpura. Henoch-Schoenlein purpura and IgA nephropathy have occurred in the same families.

In a survey of 40 families in which 2 or more members had IgA nephropathy, 5 presented with Henoch-Schoenlein purpura.[19]

Patients with Henoch-Schoenlein purpura who undergo renal transplantation develop IgA deposits in the graft.

The prevalence of both conditions is high in certain geographic areas.

Similar changes in the IgA system (ie, high IgA, IgA-1C, IgA1-IC, IgA-fibronectin aggregates, aberrantly glycosylated IgA in the circulation) occur in both diseases.[20, 21, 22]

Cystic changes in the ovaries of a prepubertal girl with Henoch-Schonlein purpura has been recorded.

However, data indicate that Henoch-Schoenlein purpura and IgA nephropathy are distinct diseases. Zhou et al examined 31 children aged 3-15 years with IgA nephropathy and 120 children aged 4-15 years with Henoch-Schoenlein purpura, noting their clinical manifestations, blood biochemistries, serum immunology, and follow-up data.[23] Renal pathologic findings on light microscopy, immunofluorescence study, and electron microscopy were analyzed and compared between 31 children with IgA nephropathy and 32 children with Henoch-Schoenlein purpura.

The age of onset was more than 12 years in 25.8% of the children with IgA nephropathy. However, the age of onset was more than 12 years in only 10% of those with Henoch-Schoenlein purpura. Clinical patterns of IgA nephropathy and Henoch-Schoenlein purpura were similar, but extrarenal manifestations were observed most often in patients with Henoch-Schoenlein purpura. All patients with Henoch-Schoenlein purpura had skin purpura, 59% of patients had GI symptoms, and 47% of patients had arthralgia. Abdominal pain occurred in only 3.2% of children with IgA nephropathy. In patients with IgA nephrology and in patients with Henoch-Schoenlein purpura, renal pathology revealed global sclerosis in 35.5% and 3.1%, mesangial sclerosis in 41.9% and 6.3%, endothelial proliferation in 29% and 65.6%, and thin basement-membrane nephropathy in 6.5% and 0%, respectively.

Electronically dense deposits in Henoch-Schoenlein purpura were sparse, loose, and widely spread in the glomerular mesangium, in the subendothelial area, and even in the intrabasement membranes, whereas the deposits were dense, lumpy, and mostly limited in mesangium and paramesangium in IgA nephropathy. Immunoglobulin G (IgG) was found in glomerular immune deposits in 71.9% of patients with Henoch-Schoenlein purpura but in only 19.4% of patients with IgA nephropathy. No IgG deposit was observed in 81.6% of those with IgA nephropathy; most had IgA and immunoglobulin M (IgM) and/or C3 deposit. Predominant IgG deposits were found in 12.5% of patients Henoch-Schoenlein purpura, with relatively weak IgA deposits. Moreover, 6.3% of patients with Henoch-Schoenlein purpura had linear IgG deposits in the glomerular capillary wall, which was not found in IgA nephropathy.

The follow-up data at an average of 20 months showed complete remission in 72.5% of patients with Henoch-Schoenlein purpura; the rate was 19.4% of those with IgA nephropathy after 34 months follow-up. Moreover, 64.5% of patients with IgA nephropathy had consistent hematuria and proteinuria, and 16.1% had active nephritides. Therefore, the difference was significant (P < .05).

Zhou et al found important clinicopathologic differences between Henoch-Schoenlein purpura and IgA nephropathy, which does not support the one-disease hypothesis.

Overview of GI findings

Abdominal pain and bloody diarrhea may precede the typical purpuric rash of Henoch-Schoenlein purpura in 14-36% of patients, complicating the initial diagnosis and even resulting in unnecessary laparotomy. GI manifestations occur in about 50% of cases and usually consist of colicky abdominal pain, melena, or bloody diarrhea. Hematemesis occurs less frequently. Intussusception should be suspected in patients Henoch-Schoenlein purpura with abdominal pain and/or melena. Barium enema is frequently therapeutic.

Overview of joint findings

Arthralgias occur in 60-84% of patients with Henoch-Schoenlein purpura. The pain most commonly affects the knees and ankles and less frequently the wrists and fingers. Frank arthritis does not occur, and joint effusions are rare. Henoch-Schoenlein purpura leaves no permanent joint deformities.

Palpable purpura usually occurs first on the lower limbs and then spreads to the buttocks. Purpura is usually most prominent over the buttocks, the posterior aspects of the lower legs, and the elbows.

Palpable purpura can also be present on the forearms and pinna. Scalp edema can occur. Hemorrhagic vesicles and bullae are rare. In most patients, the skin lesions are the first sign of Henoch-Schoenlein purpura.

Hives, angioedema, and target lesions can also occur. Vesicular eruptions and swelling and tenderness of an entire limb have been noted. Erythema multiforme–like lesions can be present.

Henoch-Schoenlein purpura with hemorrhagic bullae in children has been noted.

In AHEI, dermatologic findings are notable for a cockade (medallionlike), rosette-shaped pattern on the face, auricles, and extremities. The lesions usually appear in successive crops. The cockades display variable stages of evolution at any given time and look different from the normal purpura of Henoch-Schoenlein purpura.

The subcutaneous edema of AHEI is more common in infants. Urticaria, petechiae, and necrosis of the ear lobe are additional, rare skin manifestations of AHEI. AHEI is rarely associated with visceral involvement.

Possible renal manifestations include microscopic hematuria, proteinuria, nephrotic syndrome, progressive glomerulonephritis, ESRF (rarely), and intestinal perforation. The relationship of Henoch-Schoenlein purpura and IgA nephropathy requires further definition. Whether they are the same or distinct entities remains unclear. Evidence of both their commonality and distinctiveness is presented in Overview of renal findings above.

If serial urine samples are obtained in patients with Henoch-Schoenlein purpura, microscopic hematuria is usually found and is probably present in 100% of the patients. However, frank nephritis appears in only 20-30% of unselected children. The entire clinical spectrum of glomerulonephritis may occur in Henoch-Schoenlein purpura. The most common renal manifestations are hematuria with mild-to-moderate proteinuria.

The duodenum and small intestine are the most frequently involved segments of the GI tract. Duodenal ulcers also occur. Massive GI bleeding has been reported in Henoch-Schoenlein purpura.[24] Ileal vasculitis has also been reported.

Arthritis and/or arthralgia affects 60-75% of patients and is the presenting feature in 25%. Joints may be swollen, tender, and painful. The knees and ankles are most commonly affected. On rare occasions, symptoms involve the fingers and wrists. Findings are transient but can occur again during active disease. The joints are not permanently deformed.

Vasculitis involving the myocardia can occur. Vasculitis can involve the lungs, resulting in pulmonary hemorrhage or severe bilateral pulmonary hemorrhage. Vasculitis may cause stenosing ureteritis, priapism, penile edema, or orchitis. Ha and Lee reported that neurologic symptoms, localized edema, and high serum C3 levels show a significant relation with scrotal involvement in male patients with Henoch-Schoenlein purpura.[14] Vasculitis involving the CNS and intracranial hemorrhage has been reported.

Bilateral subperiosteal orbital hematomas have been noted. Adrenal hematomas have occurred. In rare patients, acute pancreatitis is the sole presenting feature of Henoch-Schoenlein purpura.[25] A case involving the cystic changes of the ovaries and Henoch-Schoenlein purpura has been reported.[26]

Previous
Next

Causes

The conditions below may precede Henoch-Schoenlein purpura.

Infections include the following:

Vaccinations include the following:

  • Typhoid
  • Measles
  • Cholera
  • Yellow fever

Environmental exposure to allergens include the following:

  • Drugs (eg, ampicillin, erythromycin, penicillin, quinidine, quinine, cytarabine[30] )
  • Foods
  • Horse serum
  • Cold exposure
  • Insect bites

Glomerulocystic kidney disease has also been noted.

Previous
Proceed to Differential Diagnoses
 
 
Contributor Information and Disclosures
Author

Noah S Scheinfeld, MD, JD, FAAD  Assistant Clinical Professor, Department of Dermatology, Columbia University; Consulting Staff, Department of Dermatology, St Luke's Roosevelt Hospital Center, Beth Israel Medical Center, New York Eye and Ear Infirmary; Private Practice

Noah S Scheinfeld, MD, JD, FAAD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Optigenex Consulting fee Independent contractor

Coauthor(s)

Elena L Jones, MD  Clinical Assistant Professor of Dermatology, College of Physicians and Surgeons of Columbia University; Clinic Chief, Department of Dermatology, St Luke's-Roosevelt Hospital Center

Disclosure: Nothing to disclose.

Specialty Editor Board

Richard Neiberger, MD, PhD  Director of Pediatric Renal Stone Disease Clinic, Associate Professor, Department of Pediatrics, Division of Nephrology, University of Florida College of Medicine and Shands Hospital

Richard Neiberger, MD, PhD is a member of the following medical societies: American Academy of Pediatrics, American Federation for Medical Research, American Medical Association, American Society of Nephrology, American Society of Pediatric Nephrology, Christian Medical & Dental Society, Florida Medical Association, International Society for Peritoneal Dialysis, International Society of Nephrology, National Kidney Foundation, New York Academy of Sciences, Shock Society, Sigma Xi, Southern Medical Association, Southern Society for Pediatric Research, and Southwest Pediatric Nephrology Study Group

Disclosure: The Osler Institute Honoraria Speaking and teaching

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Pharmacy Editor, eMedicine

Disclosure: Nothing to disclose.

Adrian Spitzer, MD  Professor, Department of Pediatrics, Albert Einstein College of Medicine; Director of NIH Training Program, Children's Hospital at Montefiore Medical Center

Adrian Spitzer, MD is a member of the following medical societies: American Academy of Pediatrics, American Federation for Medical Research, American Pediatric Society, American Society of Nephrology, American Society of Pediatric Nephrology, International Society of Nephrology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Howard Trachtman, MD  Program Director, Pediatrics Research, Schneider Children's Hospital, Department of Pediatrics, Division of Nephrology, Professor, Albert Einstein College of Medicine

Howard Trachtman, MD is a member of the following medical societies: American Society of Hypertension, American Society of Nephrology, American Society of Pediatric Nephrology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Chief Editor

Craig B Langman, MD  The Isaac A Abt, MD, Professor of Kidney Diseases, Feinberg School of Medicine, Northwestern University; Division Head of Kidney Diseases, Children's Memorial Hospital, Chicago

Craig B Langman, MD is a member of the following medical societies: American Academy of Pediatrics, American Society of Nephrology, and International Society of Nephrology

Disclosure: Merck Grant/research funds None; NIH Grant/research funds None; Raptor Pharmaceuticals, Inc Grant/research funds None; Alexion Pharmaceuticals, Inc. Grant/research funds None

References

  1. Henoch EH. Uber ein eigenthe Form von Purpura. Berl Klin Wochenschr. 1974;11:641-3.

  2. Yilmaz D, Kavakli K, Ozkayin N. The elevated markers of hypercoagulability in children with Henoch-Schonleinpurpura. Pediatr Hematol Oncol. Jan-Feb 2005;22(1):41-8. [Medline].

  3. Aliyazicioglu Y, Ozkaya O, Yakut H, et al. Leptin levels in Henoch-Schonlein purpura. Clin Rheumatol. Mar 2007;26(3):371-5. [Medline].

  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. Oct 19 2002;360(9341):1197-202. [Medline].

  5. Haugeberg G, Bie R, Bendvold A, et al. Primary vasculitis in a Norwegian community hospital: a retrospective study. Clin Rheumatol. 1998;17(5):364-8. [Medline].

  6. Bazina M, Glavina-Durdov M, Scukanec-Spoljar M, et al. Epidemiology of renal disease in children in the region of Southern Croatia: A 10-year review of regional renal biopsy databases. Med Sci Monit. Mar 27 2007;13(4):CR172-176. [Medline].

  7. Nong BR, Huang YF, Chuang CM, Liu CC, Hsieh KS. Fifteen-year experience of children with Henoch-Schonlein purpura in southern Taiwan, 1991-2005. J Microbiol Immunol Infect. Aug 2007;40(4):371-6. [Medline].

  8. Suehiro RM, Soares BS, Eisencraft AP, Campos LM, Silva CA. Acute hemorrhagic edema of childhood. Turk J Pediatr. Apr-Jun 2007;49(2):189-92. [Medline].

  9. Pabunruang W, Treepongkaruna S, Tangnararatchakit K, et al. Henoch-Schonlein purpura: clinical manifestations and long-term outcomes in Thai children. J Med Assoc Thai. Nov 2002;85 Suppl 4:S1213-8. [Medline].

  10. Lin SJ, Huang JL. Henoch-Schonlein purpura in Chinese children and adults. Asian Pac J Allergy Immunol. Mar 1998;16(1):21-5. [Medline].

  11. Anil M, Aksu N, Kara OD, et al. Henoch-Schonlein purpura in children from western Turkey: a retrospective analysis of 430 cases. Turk J Pediat. 51;2009:429-36. [Medline].

  12. Al Sufyani MA. Acute hemorrhagic edema of infancy: unusual scarring and review of the English language literature. Int J Dermatol. Jun 2009;6:617-22. [Medline].

  13. Prais D, Amir J, Nussinovitch M. Recurrent Henoch-Schonlein purpura in children. J Clin Rheumatol. Feb 2007;13(1):25-8. [Medline].

  14. Ha TS, Lee JS. Scrotal involvement in childhood Henoch-Schonlein purpura. Acta Paediatr. Apr 2007;96(4):552-5. [Medline].

  15. Ozkaya O, Bek K, Alaca N, et al. Cerebral vasculitis in a child with Henoch-Schonlein purpura and familial Mediterranean fever. Clin Rheumatol. Oct 2007;26(10):1729-32. [Medline].

  16. Chan KH, Tang WY, Lo KK. Bullous lesions in Henoch-Schonlein purpura. Pediatr Dermatol. May-Jun 2007;24(3):325-6. [Medline].

  17. Szeto CC, Choi PC, To KF, et al. Grading of acute and chronic renal lesions in Henoch-Schönlein purpura. Mod Pathol. Jul 2001;14(7):635-40. [Medline].

  18. Jauhola O, Ronkainen J, Koskimies O, Ala-Houhala M, Arikoski P, Holtta T, et al. Renal manifestations of Henoch-Schonlein purpura in a 6-month prospective study of 223 children. Arch Dis Child. Nov 2010;95(11):877-82. [Medline].

  19. Levy M. Familial cases of Berger's disease and anaphylactoid purpura. Kidney Int. Oct 2001;60(4):1611-2. [Medline].

  20. Coppo R, Basolo B, Piccoli G, et al. IgA1 and IgA2 immune complexes in primary IgA nephropathy and Henoch-Schonlein nephritis. Clin Exp Immunol. Sep 1984;57(3):583-90. [Medline].

  21. Davin JC, Malaise M, Foidart J, Mahieu P. Anti-alpha-galactosyl antibodies and immune complexes in children with Henoch-Schonlein purpura or IgA nephropathy. Kidney Int. May 1987;31(5):1132-9. [Medline].

  22. Jennette JC, Wieslander J, Tuttle R, Falk RJ. Serum IgA-fibronectin aggregates in patients with IgA nephropathy and Henoch-Schonlein purpura: diagnostic value and pathogenic implications. The Glomerular Disease Collaborative Network. Am J Kidney Dis. Oct 1991;18(4):466-71. [Medline].

  23. Zhou JH, Huang AX, Liu TL. [A clinico-pathological study comparing Henoch-Schonlein purpura nephritis with IgA nephropathy in children]. Zhonghua Er Ke Za Zhi. Nov 2003;41(11):808-12. [Medline].

  24. Makay B, Turkyilmaz Z, Duman M, Unsal E. Mean platelet volume in Henoch-Schonlein purpura: relationship to gastrointestinal bleeding. Clin Rheumatol. Oct 2009;28(10):1225-8. [Medline].

  25. Soyer T, Egritas O, Atmaca E, Akman H, Ozturk H, Tezic T. Acute pancreatitis: a rare presenting feature of Henoch Schonlein purpura. J Paediatr Child Health. Mar 2008;3:152-3. [Medline].

  26. Nader NS, Matsumoto JM, Lteif A. Cystic changes in the ovaries of a pre-pubertal girl with Henoch-Schonlein purpura. J Pediatr Endocrinol Metab. May 2010;23(5):517-9. [Medline].

  27. Hernando-Harder AC, Booken N, Goerdt S, Singer MV, Harder H. Helicobacter pylori infection and dermatologic diseases. Eur J Dermatol. Jun 2009;[Medline].

  28. Hoshino C. Adult onset Schonlein-Henoch purpura associated with Helicobacter pylori infection. Intern Med. May 2009;10:847-51. [Medline].

  29. Mytinger JR, Patterson JW, Thibault ES, Webb J, Saulsbury FT. Henoch-Schönlein purpura associated with Helicobacter pylori infection in a child. Pediatr Dermatol. Nov-Dec 2008;6:630-2. [Medline].

  30. Aktas B, Topcuoglu P, Kurt OK, Ensari A, Demirer T. Severe henoch-schonlein purpura induced by cytarabine. Ann Pharmacother. Apr 2009;4:792-3. [Medline].

  31. Coppo R, Andrulli S, Amore A, et al. Predictors of outcome in Henoch-Schonlein nephritis in children and adults. Am J Kidney Dis. Jun 2006;47(6):993-1003. [Medline].

  32. Prenzel F, Pfaffle R, Thiele F, Schuster V. Decreased factor XIII activity during severe Henoch-Schoenlein purpura -- does it play a role?. Klin Padiatr. May-Jun 2006;218(3):174-6. [Medline].

  33. Shin JI, Lee JS. Could measurement of factor XIII level detect the vasculitic process of Henoch-Schonlein purpura without skin rash. Acta Paediatr. Apr 2008;4:395. [Medline].

  34. Nchimi A, Khamis J, Paquot I, Bury F, Magotteaux P. Significance of bowel wall abnormalities at ultrasound in Henoch-Schonlein purpura. J Pediatr Gastroenterol Nutr. Jan 2008;46(1):48-53. [Medline].

  35. 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. Jun 2010;[Medline].

  36. Chartapisak W, Opastirakul S, Hodson EM, Willis NS, Craig JC. Interventions for preventing and treating kidney disease in Henoch-Schonlein Purpura (HSP). Cochrane Database Syst Rev. 2009;Chartapisak W, Opastirakul S, Hodson EM, Willis NS, Craig JC:CD005128. [Medline].

  37. Shenoy M, Ognjanovic MV, Coulthard MG. Treating severe Henoch-Schonlein and IgA nephritis with plasmapheresis alone. Pediatr Nephrol. Aug 2007;22(8):1167-71. [Medline].

  38. 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(2):167-70. [Medline].

  39. [Guideline] Anstey AV, Wakelin S, Reynolds NJ. Guidelines for prescribing azathioprine in dermatology. Br J Dermatol. Dec 2004;151(6):1123-32. [Medline]. [Full Text].

  40. Ohtsuka T. Successful oral cyclosporin therapy for Henoch-Schonlein purpura nephropathy. J Dermatol. May 2009;36(5):314-6. [Medline].

  41. Zaffanello M, Brugnara M, Franchini M. Therapy for children with henoch-schonlein purpura nephritis: a systematic review. ScientificWorldJournal. 2007;7:20-30. [Medline].

  42. Fagbemi AA, Torrente F, Hilson AJ, Thomson MA, Heuschkel RB, Murch SH. Massive gastrointestinal haemorrhage in isolated intestinal Henoch-Schonlein purpura with response to intravenous immunoglobulin infusion. Eur J Pediatr. Sep 2007;166(9):915-9. [Medline].

  43. Faedda R, Pirisi M, Satta A, et al. Regression of Henoch-Schonlein disease with intensive immunosuppressive treatment. Clin Pharmacol Ther. Nov 1996;60(5):576-81. [Medline].

  44. Iqbal H, Evans A. Dapsone therapy for Henoch-Schonlein purpura: a case series. Arch Dis Child. Sep 2005;90(9):985-6. [Medline].

  45. Donnithorne KJ, Atkinson TP, Hinze CH, et al. Rituximab therapy for severe refractory chronic Henoch-Schonlein purpura. J Pediatr. 155;2009:136-9. [Medline].

  46. Abend NS, Licht DJ, Spencer CH. Lupus anticoagulant and thrombosis following Henoch-Schonlein purpura. Pediatr Neurol. May 2007;36(5):345-7. [Medline].

  47. Acar B, Arikan FI, Alioglu B, Oner N, Dallar Y. Successful treatment of gastrointestinal involvement in Henoch-Schonlein purpura withplasmapheresis. Pediatr Nephrol. Nov 2008;11:2103. [Medline].

  48. Amini M, Najafi I, Ganji MR, Hakemi MS, Nouri M. Foot-drop: an unusual complaint in Henoch-Schonlein purpura. Pediatr Nephrol. Jan 2009;1:219-20. [Medline].

  49. Amitai Y, Gillis D, Wasserman D, Kochman RH. Henoch-Schonlein purpura in infants. Pediatrics. Dec 1993;92(6):865-7. [Medline].

  50. Ansell BM, Falcini F. Cutaneous vasculitis in children. Clin Dermatol. Sep-Oct 1999;17(5):577-80. [Medline].

  51. Blasini W, Saini R, Vincek V. Acute hemorrhagic edema of infancy: a case report. Dermatol Online J. Jul 2007;3:27. [Medline].

  52. Cantarini L, Brogna A, Fioravanti A, Galeazzi M. Henoch-Schönlein purpura associated with pidotimod therapy. Clin Exp Rheumatol. May-Jun 2008;S152. [Medline].

  53. Cioc AM, Sedmak DD, Nuovo GJ, et al. Parvovirus B19 associated adult Henoch Schonlein purpura. J Cutan Pathol. Nov 2002;29(10):602-7. [Medline].

  54. Dubin BA, Bronson DM, Eng AM. Acute hemorrhagic edema of childhood: an unusual variant of leukocytoclastic vasculitis. J Am Acad Dermatol. Aug 1990;23(2 Pt 2):347-50. [Medline].

  55. Dudley J, Afifi E, Gardner A, et al. Polymorphism of the ACE gene in Henoch-Schonlein purpura nephritis. Pediatr Nephrol. Mar 2000;14(3):218-20. [Medline].

  56. Duzova A, Bakkaloglu A. Central nervous system involvement in pediatric rheumatic diseases: current concepts intreatment. Curr Pharm Des. 2008;13:1295-301. [Medline].

  57. Ebert EC. Gastrointestinal manifestations of Henoch-Schonlein Purpura. Dig Dis Sci. Aug 2008;8:2011-9. [Medline].

  58. Fiore E, Rizzi M, Ragazzi M, et al. Acute hemorrhagic edema of young children (cockade purpura and edema): a case series and systematic review. J Am Acad Dermatol. Oct 2008;4:684-95. [Medline].

  59. Fretzayas A, Sionti I, Moustaki M, Papadimitriou A, Nicolaidou P. Henoch-Schonlein purpura: a long-term prospective study in Greek children. J Clin Rheumatol. Dec 2008;6:324-31. [Medline].

  60. Gandy A. Henoch-Schonlein purpura. Children's Intensive Caring Web site. Available at http://www.intensivecaring.com.

  61. Garcia-Porrua C, Gonzalez-Louzao C, Llorca J, Gonzalez-Gay MA. Predictive factors for renal sequelae in adults with Henoch-Schonlein purpura. J Rheumatol. May 2001;28(5):1019-24. [Medline].

  62. GE Medical Systems. Henoch-schonlein purpura. Medcyclopaedia. Available at http://www.medcyclopaedia.com.

  63. Gibson KL, Amamoo MA, Primack WA. Corticosteroid therapy for Henoch Schönlein purpura. Pediatrics. Apr 2008;4:870-1. [Medline].

  64. Ha TS, Lee JS. Scrotal involvement in childhood Henoch-Schönlein purpura. Acta Paediatr. Apr 2007;96(4):552-5. [Medline].

  65. Hamdan JM, Barqawi MA. Henoch-Schonlein purpura in children. Influence of age on the incidence of nephritis andarthritis. Saudi Med J. Apr 2008;4:549-52. [Medline].

  66. Harada T, Machida H, Ito S, Aihara Y, Yokota S. Henoch-Schonlein purpura presenting duodenal involvement similar to superior mesenteric artery syndrome in a girl. Eur J Pediatr. May 2007;166(5):489-90. [Medline].

  67. Hung TY, Liu MC, Hsu CF, Lin YC. Facial edema as the initial presentation of Henoch-Schonlein purpura in a 5-year-old boy. Pediatr Emerg Care. Jan 2009;1:31-2. [Medline].

  68. Inoue CN, Nagasaka T, Matsutani S, et al. Efficacy of early dental and ENT therapy in preventing nephropathy in pediatric Henoch-Schonlein purpura. Clin Rheumatol. Dec 2008;12:1489-96. [Medline].

  69. Jangjoo A, Amouzeshi A, Jalali AN. Gangrenous appendicitis in a child with Henoch-Schonlein purpura. J Pediatr Surg. Nov 2008;11:e33-5. [Medline].

  70. Kaku Y, Nohara K, Honda S. Renal involvement in Henoch-Schonlein purpura: a multivariate analysis of prognostic factors. Kidney Int. Jun 1998;53(6):1755-9. [Medline].

  71. Karnsakul W, Fallon KB, Swart S. Exudative hemorrhagic duodenitis as a primary event in a child with Henoch-Schönlein purpura. Clin Gastroenterol Hepatol. Aug 2008;8:A24. [Medline].

  72. Kausar S, Yalamanchili A. Management of haemorrhagic bullous lesions in Henoch-Schonlein purpura: is there anyconsensus. J Dermatolog Treat. 2009;2:88-90. [Medline].

  73. Kawasaki Y, Suyama K, Matsumoto A, et al. Efficacy of tonsillectomy plus methylprednisolone pulse therapy for a child with Henoch-Schoenlein purpura nephritis. Tohoku J Exp Med. Mar 2007;211(3):291-5. [Medline].

  74. Kawasaki Y, Suzuki J, Murai M, et al. Plasmapheresis therapy for rapidly progressive Henoch-Schonlein nephritis. Pediatr Nephrol. Aug 2004;19(8):920-3. [Medline].

  75. Kim CJ, Chung HY, Kim SY, et al. Acute appendicitis in Henoch-Schonlein purpura: a case report. J Korean Med Sci. Oct 2005;20(5):899-900. [Medline].

  76. Korzets Z, Magen E, Tetro J, et al. [Crescentic glomerulonephritis and cerebral vasculitis in the course of Henoch-Schonlein purpura]. Harefuah. Nov 2002;141(11):960-3, 1010. [Medline].

  77. Koskimies O, Mir S, Rapola J, Vilska J. Henoch-Schonlein nephritis: long-term prognosis of unselected patients. Arch Dis Child. Jun 1981;56(6):482-4. [Medline].

  78. Kraft DM, Mckee D, Scott C. Henoch-Schonlein purpura: a review. Am Fam Physician. Aug 1998;58(2):405-8, 411. [Medline]. [Full Text].

  79. Lau KK, Wyatt RJ, Moldoveanu Z, et al. Serum levels of galactose-deficient IgA in children with IgA nephropathy and Henoch-Schonlein purpura. Pediatr Nephrol. Dec 2007;22(12):2067-72. [Medline].

  80. Legrain V, Lejean S, Taieb A, et al. Infantile acute hemorrhagic edema of the skin: study of ten cases. J Am Acad Dermatol. Jan 1991;24(1):17-22. [Medline].

  81. Maripuri S, Fervenza FC. 71-year-old man with shortness of breath and rash. Mayo Clin Proc. Dec 2008;12:1388-91. [Medline].

  82. Martinez Lopez MM, Rodriguez Arranz C, Pena Carrion A, Merino Munoz R, Garcia-Consuegra Molina J. [Henoch-Schonlein purpura. Study of factors associated with the development and course of the disease]. An Pediatr (Barc). May 2007;66(5):453-8. [Medline].

  83. Millard T, Harris A, MacDonald D. Acute infantile hemorrhagic oedema. J Am Acad Dermatol. Nov 1999;41(5 Pt 2):837-9. [Medline].

  84. Mitsui H, Shibagaki N, Kawamura T, Matsue H, Shimada S. A clinical study of Henoch-Schonlein Purpura associated with malignancy. J Eur Acad Dermatol Venereol. Apr 2009;4:394-401. [Medline].

  85. Moses S. Henoch-Schonlein purpura. Family Practice Notebook Web site. Available at http://www.fpnotebook.com/HEM33.htm. Accessed 2003.

  86. Nan DN, Fernandez-Ayala M, Garcia-Ibarbia C. Henoch-Schonlein purpura after intravesical administration of bacillus Calmette-Guerin. Scand J Infect Dis. 2005;37(8):613-615. [Medline].

  87. Oki E, Tsugawa K, Suzuki K, Ito E, Tanaka H. Leukocytapheresis for the treatment of refractory Henoch-Schonlein purpura resistant to bothprednisolone and intravenous immunoglobulin therapy. Rheumatol Int. Sep 2008;11:1181-2. [Medline].

  88. Peru H, Soylemezoglu O, Bakkaloglu SA, et al. Henoch Schonlein purpura in childhood: clinical analysis of 254 cases over a 3-year period. Clin Rheumatol. Sep 2008;9:1087-92. [Medline].

  89. Quercia O, Emiliani F, Foschi FG, Stefanini GF. Unusual reaction to hymenoptera sting: a case of Schonlein-Henoch purpura. Allergy. Mar 2007;62(3):333-4. [Medline].

  90. Rafailidis PI, Kapaskelis A, Falagas ME. Henoch-Schonlein purpura associated with Proteus mirabilis urinary tract infection. J Am Acad Dermatol. May 2008;S98-9. [Medline].

  91. Ronkainen J, Nuutinen M, Koskimies O. The adult kidney 24 years after childhood Henoch-Schonlein purpura: a retrospective cohort study. Lancet. Aug 31 2002;360(9334):666-70. [Medline].

  92. Sanders JT, Wyatt RJ. IgA nephropathy and Henoch-Schonlein purpura nephritis. Curr Opin Pediatr. Apr 2008;2:163-70. [Medline].

  93. Saraclar Y, Tinaztepe K, Adalioglu G, Tuncer A. Acute hemorrhagic edema of infancy (AHEI)--a variant of Henoch-Schonlein purpura or a distinct clinical entity?. J Allergy Clin Immunol. Oct 1990;86(4 Pt 1):473-83. [Medline].

  94. Saulsbury FT. Henoch-Schonlein purpura in children. Report of 100 patients and review of the literature. Medicine (Baltimore). Nov 1999;78(6):395-409. [Medline].

  95. Saulsbury FT. Henoch-Schonlein purpura. Pediatr Dermatol. Jan 1984;1(3):195-201. [Medline].

  96. Sexton K, McNicholas A, Galloway Y, et al. Henoch-Schonlein purpura and meningococcal B vaccination. Arch Dis Child. Mar 2009;3:224-6. [Medline].

  97. Shetty AK, Desselle BC, Ey JL, et al. Infantile Henoch-Schonlein purpura. Arch Fam Med. Jun 2000;9(6):553-6. [Medline].

  98. Shin JI, Lee JS. Comment on acute pancreatitis: a rare presenting feature of Henoch-Schönlein purpura. J Paediatr Child Health. Nov 2008;11:678-9. [Medline].

  99. Shin JI, Lee JS. Familial clusturing of Henoch-Schonlein purpura or IgA nephropathy: genetic background orenvironmental triggers. Pediatr Dermatol. Nov-Dec 2008;6:651. [Medline].

  100. Siomou E, Serbis A, Salakos C, et al. Masked severe stenosing ureteritis: a rare complication of Henoch-Schonlein purpura. Pediatr Nephrol. May 2008;5:821-5. [Medline].

  101. Snow IM. Purpura, urticaria and angioneurotic edema of the hands and feet in a nursing baby. JAMA. 1913;61:18-9.

  102. Soylemezoglu O, Ozkaya O, Erbas D, et al. Nitric oxide in Henoch-Schonlein purpura. Scand J Rheumatol. 2002;31(5):271-4. [Medline].

  103. Soylemezoglu O, Peru H, Gonen S, Cetinyurek A, Buyan N. HLA-DRB1 alleles and Henoch-Schönlein purpura: susceptibility and severity of disease. J Rheumatol. Jun 2008;6:1165-8. [Medline].

  104. Soylemezoglu O, Peru H, Gonen S, et al. CTLA-4 +49 A/G genotype and HLA-DRB1 polymorphisms in Turkish patients with Henoch-Schonlein purpura. Pediatr Nephrol. Aug 2008;23(8):1239-44. [Medline].

  105. Sugiyama H, Watanabe N, Onoda T, et al. Successful treatment of progressive Henoch-Schonlein purpura nephritis withtonsillectomy and steroid pulse therapy. Intern Med. Jun 2005;44(6):611-5. [Medline].

  106. Sunderkotter C. Vasculitis of small blood vessels--some riddles about IgA and about the complexity of transmigration. Exp Dermatol. Jan 2009;1:91-6. [Medline].

  107. Szer IS. Gastrointestinal and renal involvement in vasculitis: management strategies in Henoch-Schonlein purpura. Cleve Clin J Med. May 1999;66(5):312-7. [Medline].

  108. Szer IS. Henoch-Schonlein purpura. Curr Opin Rheumatol. Jan 1994;6(1):25-31. [Medline].

  109. Szer IS. Henoch-Schonlein purpura: when and how to treat. J Rheumatol. Sep 1996;23(9):1661-5. [Medline].

  110. Tizard EJ. Henoch-Schonlein purpura. Arch Dis Child. Apr 1999;80(4):380-3. [Medline].

  111. Topaloglu R, Bayrakci US, Cil B, Orhon D, Bakkaloglu A. Henoch-Schonlein purpura with high factor VIII levels and deep venous thrombosis: anassociation or coincidence. Rheumatol Int. Jul 2008;9:935-7. [Medline].

  112. Vila Cots J, Gimenez Llort A, Camacho Diaz JA, Vila Santandreu A. [Nephropathy in Schonlein-Henoch purpura: a retrospective study of the last 25 years]. An Pediatr (Barc). Mar 2007;66(3):290-3. [Medline].

  113. Watanabe T, Sato Y. Renal involvement and hypocomplementemia in a patient with acute hemorrhagic edema of infancy. Pediatr Nephrol. Nov 2007;22(11):1979-81. [Medline].

  114. Zaffanello M, Fanos V. Treatment-based literature of Henoch-Schonlein purpura nephritis in childhood. Pediatr Nephrol. Oct 2009;24(10):1901-11. [Medline].

  115. Zhang Y, Gu W, Mao J. Sibling cases of Henoch-Schonlein purpura in two families and review of literature. Pediatr Dermatol. May-Jun 2008;3:393-5. [Medline].

  116. Zmora O, Shin CE. Multiple surgical interventions due to recurrent intussusception in a patient with henoch-schonlein purpura: a case report. Eur J Pediatr Surg. Oct 2008;5:340-1. [Medline].

  117. Zoch-Zwierz W, Biernacka A, Tomaszewska B, et al. [Vasculitis of cerebral vessels, probable cause of neurological complications in a child with Schonlein-Henoch purpura]. Pol Merkuriusz Lek. Apr 2002;12(70):306-8. [Medline].

 
Previous
Next
 
Purpuric papules and plaques of the lower extremity characteristic of Henoch-Schönlein purpura.
Hemorrhagic macules, papules, and patches on the ankle and foot of a child with Henoch-Schönlein purpura.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.