Updated: 7/11/2019

Henoch-Schonlein Purpura

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Snapshot
  • A 4-year-old boy presents to the emergency room with abdominal pain. His mother reports that he also had a rash for about a week on his lower extremities as well as joint pain and blood in his urine. Physical exam reveals palpable purpura on his buttocks and bilaterally on his legs and abdominal tenderness. On laboratory evaluation, there is no thrombocytopenia. The physician reassures his mother that this will resolve on its own and he will be monitored for renal involvement. 
Introduction
  • Clinical definition 
    • also known as IgA vasculitis
    • a systemic immune-mediated small vessel vasculitis often following an upper respiratory infection and characterized by IgA deposition and triad of 
      • palpable purpura
      • arthralgias
      • abdominal pain
  • Epidemiology
    • demographics
      • children < 10 years of age
      • most common childhood vasculitis
    • risk factors 
      • fall and winter months
      • family history
      • upper respiratory tract infection
        • especially with group A streptococcus or parvovirus B19
  • Etiology
    • associated with preceding upper respiratory infection
  • Pathogenesis
    • IgA immune complex deposition in the small vessels causes a vasculitis
      • deposition in the skin, gastrointestinal tract, and renal glomeruli cause physical manifestations of the disease
      • leukocytoclasis causes small vessel necrosis 
  • Associated conditions
    • IgA nephropathy (Berger disease)
  • Prognosis
    • resolves spontaneously over time
    • typically lasts 4 weeks
Presentation
  • Symptoms 
    • joint pains (arthralgias) of lower extremities
    • colicky abdominal pain
    • hematuria
    • constitutional symptoms
      • fever, malaise, or fatigue
  • Physical exam
    • symmetric palpable nontender purpura on buttocks and lower extremities
      • typically the first sign of the disease
      • may spread to upper extremities
    • abdominal tenderness on palpation
      • typically 1 week after onset of rash but can occur simultaneously
Studies
  • Labs
    • normal platelet count
      • presence of thrombocytopenia should prompt investigation into other causes of palpable purpura
    • normal coagulation profile
    • urine studies
      • proteinuria and hematuria indicates renal involvement
  • Biopsy
    • indications
      • most accurate test but usually not necessary
        • only indicated if the diagnosis is uncertain
    • findings
      • IgA deposition in small vessels seen on direct immunofluorescence
      • leukocytoclasis
  • Making the diagnosis
    • most cases are clinically diagnosed
Differential
  • Thrombotic thrombocytopenic purpura
    • distinguishing factors
      • FATRN
        • Fever
        • Anemia (microangiopathic hemolytic)
        • Thrombocytopenia
        • Renal failure
        • Neurological symptoms
      • schistocytes on peripheral blood smear
  • Polyarteritis nodosa
    • distinguishing factors
      • usually in adults
      • typically presents with ulcerative lesions but can also present with palpable purpura
      • associated with hepatitis B virus infection
      • "string of beads" (microaneurysms) on angiogram
Treatment
  • Management approach
    • as the disease resolves spontaneously over time, medical therapy is typically unnecessary, unless there is persistent nephropathy
  • Conservative
    • supportive care
      • indication
        • for all patients
      • modalities
        • hydration
        • pain management
  • Medical
    • angiotensin-converting enzyme (ACE) inhibitor
      • indication
        • for patients with persistent proteinuria
    • corticosteroids
      • indications
        • for patients with persistent proteinuria despite ACE-inhibitors
        • not recommended for prevention of renal disease
Complications
  • Persistent renal sequelae   
  • Recurrence
 

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Questions (4)
Lab Values
Blood, Plasma, Serum Reference Range
ALT 8-20 U/L
Amylase, serum 25-125 U/L
AST 8-20 U/L
Bilirubin, serum (adult) Total // Direct 0.1-1.0 mg/dL // 0.0-0.3 mg/dL
Calcium, serum (Ca2+) 8.4-10.2 mg/dL
Cholesterol, serum Rec: < 200 mg/dL
Cortisol, serum 0800 h: 5-23 μg/dL //1600 h:
3-15 μg/dL
2000 h: ≤ 50% of 0800 h
Creatine kinase, serum Male: 25-90 U/L
Female: 10-70 U/L
Creatinine, serum 0.6-1.2 mg/dL
Electrolytes, serum  
Sodium (Na+) 136-145 mEq/L
Chloride (Cl-) 95-105 mEq/L
Potassium (K+) 3.5-5.0 mEq/L
Bicarbonate (HCO3-) 22-28 mEq/L
Magnesium (Mg2+) 1.5-2.0 mEq/L
Estriol, total, serum (in pregnancy)  
24-28 wks // 32-36 wks 30-170 ng/mL // 60-280 ng/mL
28-32 wk // 36-40 wks 40-220 ng/mL // 80-350 ng/mL
Ferritin, serum Male: 15-200 ng/mL
Female: 12-150 ng/mL
Follicle-stimulating hormone, serum/plasma Male: 4-25 mIU/mL
Female: premenopause: 4-30 mIU/mL
midcycle peak: 10-90 mIU/mL
postmenopause: 40-250
pH 7.35-7.45
PCO2 33-45 mmHg
PO2 75-105 mmHg
Glucose, serum Fasting: 70-110 mg/dL
2-h postprandial:<120 mg/dL
Growth hormone - arginine stimulation Fasting: <5 ng/mL
Provocative stimuli: > 7ng/mL
Immunoglobulins, serum  
IgA 76-390 mg/dL
IgE 0-380 IU/mL
IgG 650-1500 mg/dL
IgM 40-345 mg/dL
Iron 50-170 μg/dL
Lactate dehydrogenase, serum 45-90 U/L
Luteinizing hormone, serum/plasma Male: 6-23 mIU/mL
Female: follicular phase: 5-30 mIU/mL
midcycle: 75-150 mIU/mL
postmenopause 30-200 mIU/mL
Osmolality, serum 275-295 mOsmol/kd H2O
Parathyroid hormone, serume, N-terminal 230-630 pg/mL
Phosphatase (alkaline), serum (p-NPP at 30° C) 20-70 U/L
Phosphorus (inorganic), serum 3.0-4.5 mg/dL
Prolactin, serum (hPRL) < 20 ng/mL
Proteins, serum  
Total (recumbent) 6.0-7.8 g/dL
Albumin 3.5-5.5 g/dL
Globulin 2.3-3.5 g/dL
Thyroid-stimulating hormone, serum or plasma .5-5.0 μU/mL
Thyroidal iodine (123I) uptake 8%-30% of administered dose/24h
Thyroxine (T4), serum 5-12 μg/dL
Triglycerides, serum 35-160 mg/dL
Triiodothyronine (T3), serum (RIA) 115-190 ng/dL
Triiodothyronine (T3) resin uptake 25%-35%
Urea nitrogen, serum 7-18 mg/dL
Uric acid, serum 3.0-8.2 mg/dL
Hematologic Reference Range
Bleeding time 2-7 minutes
Erythrocyte count Male: 4.3-5.9 million/mm3
Female: 3.5-5.5 million mm3
Erythrocyte sedimentation rate (Westergren) Male: 0-15 mm/h
Female: 0-20 mm/h
Hematocrit Male: 41%-53%
Female: 36%-46%
Hemoglobin A1c ≤ 6 %
Hemoglobin, blood Male: 13.5-17.5 g/dL
Female: 12.0-16.0 g/dL
Hemoglobin, plasma 1-4 mg/dL
Leukocyte count and differential  
Leukocyte count 4,500-11,000/mm3
Segmented neutrophils 54%-62%
Bands 3%-5%
Eosinophils 1%-3%
Basophils 0%-0.75%
Lymphocytes 25%-33%
Monocytes 3%-7%
Mean corpuscular hemoglobin 25.4-34.6 pg/cell
Mean corpuscular hemoglobin concentration 31%-36% Hb/cell
Mean corpuscular volume 80-100 μm3
Partial thromboplastin time (activated) 25-40 seconds
Platelet count 150,000-400,000/mm3
Prothrombin time 11-15 seconds
Reticulocyte count 0.5%-1.5% of red cells
Thrombin time < 2 seconds deviation from control
Volume  
Plasma Male: 25-43 mL/kg
Female: 28-45 mL/kg
Red cell Male: 20-36 mL/kg
Female: 19-31 mL/kg
Cerebrospinal Fluid Reference Range
Cell count 0-5/mm3
Chloride 118-132 mEq/L
Gamma globulin 3%-12% total proteins
Glucose 40-70 mg/dL
Pressure 70-180 mm H2O
Proteins, total < 40 mg/dL
Sweat Reference Range
Chloride 0-35 mmol/L
Urine  
Calcium 100-300 mg/24 h
Chloride Varies with intake
Creatinine clearance Male: 97-137 mL/min
Female: 88-128 mL/min
Estriol, total (in pregnancy)  
30 wks 6-18 mg/24 h
35 wks 9-28 mg/24 h
40 wks 13-42 mg/24 h
17-Hydroxycorticosteroids Male: 3.0-10.0 mg/24 h
Female: 2.0-8.0 mg/24 h
17-Ketosteroids, total Male: 8-20 mg/24 h
Female: 6-15 mg/24 h
Osmolality 50-1400 mOsmol/kg H2O
Oxalate 8-40 μg/mL
Potassium Varies with diet
Proteins, total < 150 mg/24 h
Sodium Varies with diet
Uric acid Varies with diet
Body Mass Index (BMI) Adult: 19-25 kg/m2
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(M2.RH.41) Approximately 1 week following an upper respiratory infection, a 5-year-old boy begins to develop abdominal pain, diffuse joint pain, and a urticarial rash on his bilateral lower extremities. He has no other complaints. Over the next 12-24 hours, the rash changes in character, and the boy is brought to his primary care physician. On exam, the pediatrician notes dark purple, non-blanching papules and plaques that are distributed symmetrically over the patient's buttocks and legs (Figure A). A CBC and CMP show no abnormalities. What is the underlying process causing this patient's dermatological findings? Review Topic

QID: 104252
FIGURES:
1

Exotoxin release

5%

(1/22)

2

Histamine release

0%

(0/22)

3

Immune-mediated platelet destruction

5%

(1/22)

4

IgA-mediated vasculitis

86%

(19/22)

5

Anti-neutrophil cytoplasmic antibodies

0%

(0/22)

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SUBMIT RESPONSE 4

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(M2.RH.188) A 6-year-old girl presents to her pediatrician with a rash and intermittent abdominal pain and swelling of her legs and arms. She has also begun to complain of pain in her knees and hands with no history of trauma. Her temperature is 97.6°F (36.4°C), blood pressure is 100/80 mmHg, pulse is 89/min, respirations are 18/min, and oxygen saturation is 98% on room air. Physical exam is notable for the finding in Figure A as well as symmetric swelling in the joints of the upper extremities. Her abdomen is currently nontender and not painful. Laboratory studies are pending, and the results of a urinalysis are notable only for positive blood and protein in the urine. Which of the following is the most likely diagnosis? Review Topic

QID: 105586
FIGURES:
1

Hemorrhagic adrenalitis

0%

(0/30)

2

Henoch-Schonlein purpura

87%

(26/30)

3

Immune thrombocytopenic purpura

7%

(2/30)

4

Kawasaki disease

3%

(1/30)

5

Rubella

0%

(0/30)

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