A 6-year-old female came to a pediatric nephrologist with a 2-month history of intermittent rash. The rash was located bilaterally on the ankles and was described as initially red, papular and then became pustular with white fluid. It was not pruritic and the family denied any new contacts or ill exposures. The patient was seen locally by their regular physician who prescribed antibiotics. The rash resolved. The rash then returned and the patient was seen twice over the next month in a local emergency room. Again antibiotics were prescribed and the rash resolved. The patient returned for a third emergency room visit and the rash now was described as having a halo of bluish discoloration and was also seen on the buttocks and ankles bilaterally. The patient also had intermittent abdominal pain that lasted 1-2 days, resolved and returned over the past week. There was no change in stools or appetite. The parents report that sometime in the past month her urine had looked “different.” A urinalysis showed hematuria and proteinuria.
The past medical history was non-contributory.
The family history was negative for any gastrointestinal or nephrological problem. There was some hypertension in two grandparents.
The review of systems showed no change in stools or stooling pattern, fever, emesis, joint swelling, or difficulty ambulating.
The pertinent physical exam showed vital signs to be normal but with an elevated blood pressure of 110/65. Her weight was 23 kg but previous weights were not available. HEENT was negative except for mild periorbital edema. Abdominal examination showed a slightly protuberant abdomen without a fluid wave or masses. Extremities showed mild ankle edema with palpable ~3 mm bluish-red lesions around the ankle and intermittently up the posterior legs to the buttocks. These lesions were non-blanching nor were pruritic. The rest of the examination was normal.
The laboratory evaluation included a normal complete blood count, coagulation profile, calcium, magnesium, phosphorus, alkaline phosphatase, electrolytes, albumin, total protein, and cholesterol. Urinalysis showed 3+ proteinuria and hematuria and a low C3 of 55 mg/dL (normal 90-180 mg/dL). C4 was normal.
The diagnosis of probable Henoch-Schönlein purpura (HSP) was made and the patient was begun on a course of steroids.
The patient’s clinical course over the next 2 weeks found her to be increasing in weight and developing more edema. She was admitted to the hospital for further management and evaluation as her symptoms had not improved. She received intravenous albumin, furosemide and solumedrol, which improved her blood pressure and clinical symptoms. As this patient had been on steroids and needed hospitalization and a solumedrol pulse, she underwent renal biopsy to determine if this was HSP, another vasculitic or an infectious/post-infectious process. The results were pending.
Henoch-Schönlein purpura (HSP) is a generalized vasculitis that commonly involves the gastrointestinal tract, kidneys, skin and joints. It can also affect the lungs and central nervous system much less often. Its etiology is uncertain but is probably multifactorial with antigens, environmental and genetic factors. It is thought to be caused by an unknown antigen stimulating a rise in IgA producing and antigen-antibody complexes being deposited locally in the body and activating pathways leading to necrotizing vasculitis.
Most children with HSP are between 2-11 years old with males affected more than females in a 2:1 ratio. Most children have a preceding gastrointestinal, pharyngeal or upper respiratory tract infection. Associations with bacteria, viruses, vaccinations and drugs have been reported.
Signs and symptoms of HSP include:
- Headache, fever and malaise may occur as a prodrome.
- The rash of classic HSP is a non-blanching red-purple-rust colored macules or plaques. They may be urticarial and/or palpable. They are particularly found on the buttocks and lower extremities but can be found in other locations. It may occur in new crops or waves. It may come and go throughout the illness and it may come late in the presentation making the diagnosis more difficult.
- Migratory polyarthritis especially of the knees and ankles but other joints often occurs with pain and edema.
- Colicky abdominal pain often occurs with or without nausea and emesis. This can last for several weeks and is sometimes treated with steroids. Complications such as bowel perforation, gastrointestinal bleeding, and intussception can make the differential diagnosis of abdominal pain more difficult for patients with HSP.
- Renal involvement includes a wide range from mild hematuria and proteinuria to renal failure. Renal symptoms may persist for 6 months after the rash. Peripheral edema may also occur.
Most children have complete recovery but serious renal and gastrointestinal complications may occur.
Long term morbidity is mainly related to renal involvement.
Treatment is mainly supportive with close followup and monitoring of proteinuria and hematuria at intervals. Complications require more aggressive treatment.
Renal biopsy is sometimes necessary to more clearly differentiate other causes of acute/chronic renal failure in patients that appear to have HSP including:
- Anaphylactic purpura with nephritis
- Hemolytic-uremic syndrome
- IgA nephropathy
- Infectious glomerulonephritis – bacterial (especially post-streptococcal), viral and fungal etiologies
- Membranoproliferative glomerulonephritis
- Rheumatoid arthritis
- Systemic lupus erythematosus
- Wegner’s granulomatosis
Questions for Further Discussion
1. What other diseases should be included in the differential diagnosis of HSP?
2. What are the differences between nephrotic syndrome and nephritis?
3. List the possible gastrointestinal complications of HSP.
4. When should steroids be used for HSP?
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Haroon M. Should children with Henoch-Schonlein purpura and abdominal pain be treated with steroids? Arch Dis Child. 2005 Nov;90(11):1196-8.
Dyne PL, Sawtelle S, Kesler D. Pediatrics, Henoch-Schonlein Purpura. eMedicine. Available from the Internet at http://emedicine.medscape.com/article/804681-overview (rev. 12/20/2007, cited 1/5/09).
Sanders JT, Wyatt RJ. IgA nephropathy and Henoch-Schönlein purpura nephritis. Curr Opin Pediatr. 2008 Apr;20(2):163-70.
ACGME Competencies Highlighted by Case
1. When interacting with patients and their families, the health care professional communicates effectively and demonstrates caring and respectful behaviors.
2. Essential and accurate information about the patients’ is gathered.
3. Informed decisions about diagnostic and therapeutic interventions based on patient information and preferences, up-to-date scientific evidence, and clinical judgment is made.
4. Patient management plans are developed and carried out.
5. Patients and their families are counseled and educated.
6. Information technology to support patient care decisions and patient education is used.
7. All medical and invasive procedures considered essential for the area of practice are competently performed.
9. Patient-focused care is provided by working with health care professionals, including those from other disciplines.
10. An investigatory and analytic thinking approach to the clinical situation is demonstrated.
11. Basic and clinically supportive sciences appropriate to their discipline are known and applied.
13. Information about other populations of patients, especially the larger population from which this patient is drawn, is obtained and used.
Donna M. D’Alessandro, MD
Professor of Pediatrics, University of Iowa Children’s Hospital