Purpura

Purpura represents leakage of blood from vessels into skin/mucous membranes; thus, it is nonblanching on pressure; petechiae are <3 mm, ecchymosis >3 mm in diameter. Idiopathic thrombocytopenic purpura (ITP) is the most common cause of thrombocytopenic and Henoch-Schönlein purpura (HSP) the most common form of non-thrombocytopenic purpura in children; von Willebrand disease (vWD) is the most common bleeding disorder.

Differential Diagnosis

• Vasculitis (palpable purpura)
  –HSP: Most common vasculitis, incidence: 0.01%, in 50% follows URI; other triggers are bacterial infection, drugs, vaccines, food, insect bites; lasts 1–2 weeks, age 2–8 (mean 4 years), M > F, IgA-mediated, small vessels
  –Polyarteritis nodosa (PAN), Wegener granulomatosis (WG): Rare in children

• Hematologic
  –ITP: Age 1–5 years; autoantibodies against platelets (platelets destroyed by splenic macrophages); usually 1–6 weeks after viral infection; 70–80% acute self-limited; 10–20% chronic recurrent; <1% associated with intracranial hemorrhage
  –Other causes of thrombocytopenia: Wiscott-Aldrich syndrome, aplastic anemia, leukemia, disseminated intravascular coagulation (DIC), thrombocytopenia absent radius (TAR)

• Coagulation factor deficiencies:
  –Hemophilia A/B (factors VIII/XI): A (1/7,500 male births) four times more common than B; X-linked recessive
  –vWD: Prevalence 1%, autosomal dominant, vW factor deficiency or decreased function
  –Liver disease: Decreased production of coagulation factors
  –Hemorrhagic disease of the newborn: Decreased vitamin K-dependent coagulation factors (II, VII, IX, X)

• Infections
  –Bacterial/rickettsial: Meningococcemia (MC), Group A strep (scarlet fever), Streptococcus viridans/Staphylococcus aureus (endocarditis), Gonococcus (disseminated), Leptospirosis, Rickettsia rickettsii (Rocky Mountain spotted fever), R. prowazekii (epidemic typhus), Ehrlichiosis
  –Viral: Hepatitis B, Dengue hemorrhagic fever, atypical measles

• Drugs: Coumadin, heparin, aspirin, thiazide, corticosteroids, penicillins, sulfonamides
• Others: Trauma/abuse, scurvy (vitamin C deficiency)

Workup and Diagnosis

• Determine location, duration, associated/preceding symptoms, family history of bleeding disorders, social history (for abuse), insect bites, travel history, drugs, easy bruising; meningeal signs; purpura fulminans
• HSP: Purpura mostly on buttocks and legs; arthritis, abdominal pain, ±GI hemorrhage, renal disease, self-limited; complications: intussusception, end-stage renal disease <1%; increased platelet/ESR/WBC/IgA
• ITP: Sudden petechiae/ecchymoses, epistaxis, or menorrhagia; rare splenomegaly; platelet count usually <20,000; bone marrow exam if other marrow disease is suspected
• MC: Septic shock, ±meningitis; culture and Gram stain (blood and CSF)
• Hemophilia A/B: Easy bruising, intramuscular hematomas, hemarthroses, B milder than A; mild: 5–30%, moderate: 1–5%, severe: <1% of normal factor level
• vWD: Petechiae/ecchymoses, epistaxis, menorrhagia
• Endocarditis: Janeway lesions, splinter hemorrhages
• PAN/WG: Painful skin nodules, any organ (PAN); skin, kidneys (glomerulonephritis), pulmonary hemorrhage (WG); biopsy; angiography in WG (for aneurysms)
• Atypical measles: clinical diagnosis, history of killed vaccine + exposure
• DIC: Increased PT/PTT, decreased platelets, increased FDP/D-dimers, hemolysis

Treatment

• HSP: Analgesia, hydration, treat complications
  –Corticosteroid use is controversial

• ITP with platelet count <20,000
  –IV immunoglobulin to block macrophage receptors
  –Anti-Rh immunoglobulin binds to RBCs so the spleen destroys RBCs instead of platelets, corticosteroids
  –Treat to raise platelet count and decrease risk of intracranial hemorrhage
  –Emergency: Platelet transfusion
  –Chronic: Immunosuppressant or splenectomy

• Hemophilia A: Recombinant F VIII
  –IV or intranasal DDAVP (desmopressin) releases F VIII and vWF from endothelial cells
• Hemophilia B: Recombinant or plasma-derived F IX
• DIC: Treat cause; transfuse platelets, cryoprecipitate, or fresh frozen plasma
• vWD: DDAVP or plasma-derived vWF
• PAN: Oral or IV corticosteroid

Book Source Details

• Book Title: In A Page: Pediatric Signs and Symptoms
• Author(s): Jonathan E. Teitelbaum, Kathleen O. Deantonis, Scott Kahan
• Year of Publication: 2007
• Copyright Details: In A Page: Pediatric Signs and Symptoms, Copyright © 2007 Lippincott Williams & Wilkins.

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Copyright Details: In A Page: Pediatric Signs and Symptoms, Copyright © 2008 Williams & Wilkins.

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More About This Book: Title: In A Page: Pediatric Signs and Symptoms Authors: Jonathan E. Teitelbaum, Kathleen O. Deantonis, Scott Kahan Publisher: Lippincott Williams & Wilkins Copyright: 2007 ISBN: 1-4051-0427-9

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