Henoch-Schonlein purpura
Background
- Most common vasculitis in childhood
- Small vessel
- Most cases preceded by a URI
- Usually affects 2-11 yr
- 5% of cases associated with intussusception (abdominal vasculitis)
- Renal involvement is feared complication
- 95% recover completely after 3-4wk
Clinical Presentation
Classic
May develop over the course of days to weeks and may vary in order of presentation
- Rash: palpable purpura
- Often begins with erythematous, macular, or urticarial wheals, progressing into the typical ecchymoses, petechiae, and palpable purpura (see picture)
- Typically appears in crops, symmetrically distributed, and located primarily in gravity/pressure-dependent areas (e.g. lower extremities, buttocks)
- Acute abdominal pain
- Typically diffuse and colicky
- May have blood in the stool (massive bleeding is rare)
- Arthritis/arthralgia
- Migratory, usually involves knees/ankles
- Nephritis/renal disease
- (~50% of the time)[1]
- Hematuria +/- proteinuria
Rare manifestations
- Melena, hematemesis, hepatosplenomegaly
- Headache, seizures
- Fever
- Non-pitting edema of the extremities and face
- Nephrotic Syndrome
Differential Diagnosis
- Erythema nodosum
- Intussusception
- Rheumatic fever
- Polyarteritis nodosa
- SLE
- Rheumatoid arthritis
- Drug reaction
Pediatric Rash
- Atopic dermatitis
- Bed bugs
- Contact dermatitis
- Drug rash
- Erythema infectiosum (Fifth disease)
- Hand-foot-and-mouth disease
- Henoch-schonlein purpura (HSP)
- Herpangina
- Herpes simplex virus (HSV)
- Infectious mononucleosis
- Meningitis
- Measles
- Molluscum contagiosum
- Roseola infantum
- Rubella (German measles)
- Scabies
- Scarlet fever
- Smallpox
- Varicella (Chickenpox)
Causes of Glomerulonephritis
- Poststreptococcal glomerulonephritis
- Hemolytic-uremic syndrome
- Henoch-Schonlein purpura
- IgA nephropathy
- Lupus nephritis
- Alport syndrome
- Goodpasture syndrome
- Paraneoplastic
Diagnosis
Work-Up
- UA
- Hematuria, proteinuria
- Chemistry
- Consider stool guaiac if concern for melena
Treatment
- Supportive
- NSAIDs for pain, may worsen renal disease or GI disease
- Consider prednisone 1mg/kg/day for severe arthralgias, abdominal or scrotal disease
Disposition
- Outpt management for most w/ rheum f/u
- Recurrence rate of up to 33%
See Also
References
- ↑ Chen JY et al. Henoch-Schönlein purpura nephritis in children: incidence, pathogenesis and management. World J Pediatr. 2015 Feb;11(1):29-34. doi: 10.1007/s12519-014-0534-5. Epub 2014 Dec 29.
- ↑ Calviño, MC, Llorca, J, García-Porrúa, C, Fernández-Iglesias, JL, Rodriguez-Ledo, P, González-Gay, MA (2001) Henoch-Schönlein purpura in children from northwestern Spain: a 20-year epidemiologic and clinical study. Medicine (Baltimore) 80: pp. 279-290
- ↑ Saulsbury, FT (1999) Henoch-Schönlein purpura in children. Report of 100 patients and review of the literature. Medicine (Baltimore) 78: pp. 395-409