Hematology and Oncology Book


Henoch-Schonlein Purpura

Aka: Henoch-Schonlein Purpura, Henoch Schonlein Purpura, Henoch-Schoenlein Purpura, Henoch Schoenlein Purpura, HSP, IgA Vasculitis, Immunoglobulin A Vasculitis
  1. Epidemiology
    1. Children and young adults
      1. Most common acute Vasculitis in children
      2. Peak Incidence at 5 year old
      3. Ages 2-11 years represent 75-90% of cases
      4. Milder case occur in children under age 2 years
    2. Occurs more often in boys (2:1)
    3. Incidence: 14 cases per 100,000
    4. Occurs most frequently in spring and fall
  2. Pathophysiology
    1. Upper Respiratory Infection precedes in 60-75% cases
    2. Acute immune complex-mediated Leukocytoclastic Vasculitis
    3. Idiopathic inflammatory IgA hypersensitivity
      1. Petechiae and Purpura
        1. IgA immune complexes deposit in small vessel walls of skin
      2. Gastrointestinal Hemorrhage
        1. IgA immune complexes deposit in small vessel walls of intestinal wall
      3. Crescentic Glomerulonephritis
        1. IgA immune complexes deposit in small vessel walls of renal mesangium
  3. Associated Conditions (preceding HSP)
    1. Bacterial Infections
      1. Group A Streptococcus (most common - may be responsible for 30% of cases)
      2. Bartonella Henselae
      3. Campylobacter enteritis
      4. Salmonella
      5. Shigella
      6. Methicillin-Resistant Staphylococcus aureus (MRSA)
      7. Mycoplasma
      8. Haemophilus parainfluenza
      9. Helicobacter Pylori
    2. Viral Infections
      1. Adenovirus
      2. Coxsachie Virus
      3. Epstein-Barr Virus (Mononucleosis)
      4. Hepatitis A Virus
      5. Hepatitis BVirus
      6. Parvovirus B19
      7. Varicella Zoster Virus
    3. Vaccinations
      1. Typhoid
      2. Measles
      3. Cholera
      4. Yellow Fever
    4. Environmental exposures
      1. Allergens in drugs and foods
      2. Cold exposure
      3. Insect Bites
  4. Symptoms: Classic Triad (beyond rash, triad is not uniformly present)
    1. Palpable Purpura rash on lower extremities (gravity dependent regions)
    2. Abdominal Pain or renal involvement (Nephritis)
    3. Arthritis or Arthralgias
  5. Signs: Rash (100% of cases)
    1. Timing
      1. Rash precedes other signs and symptoms of HSP
      2. First appears as erythematous Papules
      3. Palpable Purpura rash follows
    2. Distribution
      1. Gravity and pressure dependent
      2. Typically appears on extensor surfaces of lower extremities, belt line and buttocks
      3. Can involve face and trunk
    3. Characteristics: Petechiae or palpable Purpura (primary lesion type)
      1. Non-pruritic, non-blanching hemorrhagic lesions (palpable Purpura and Petechiae)
        1. Initially they may blanch on pressure; later they do not
        2. Lesions may become hemorrhagic or necrotic
      2. Transition from purple to rust-colored and then fade over a 10 day period
    4. Characteristics: Other associated lesions
      1. Urticarial wheels
      2. Erythematous Macules
      3. Erythematous Papules
      4. Target lesions
        1. May appear similar to Erythema Multiforme
  6. Signs: Abdominal Pain (60-80% of cases)
    1. Diffuse, Colicky Abdominal Pain (may mimic Acute Abdomen)
    2. Abdominal Pain onset typically follows rash
    3. Stools may show occult or gross blood
    4. Hematuria
    5. Vomiting or Hematemesis (rarely severe) in up to 30% of patients
  7. Signs: Joint Involvement (70% of cases)
    1. Arthritis precedes rash in 25% of cases
    2. Transient Arthritis with no permanent deformity
    3. Non-Migratory polyarthritis
      1. Ankles and knees most commonly affected
      2. Elbows, hands and feet may also be affected
  8. Signs: Renal Disease (25-50% of cases)
    1. General
      1. Most serious complication of HSP
    2. Risk Factors
      1. Age over 10 years
      2. Persistent Purpura
      3. Severe Abdominal Pain
      4. Relapsing episodes
    3. Presentation
      1. Develops within 3 months of rash (typically within first month and rarely beyond 6 months)
      2. Hematuria most common presenting sign (also accompanied by red cell casts and Proteinuria)
  9. Complications (more common in adults)
    1. Cardiopulmonary conditions
      1. Myocardial Infarction
      2. Pulmonary Hemorrhage
      3. Pleural Effusion
    2. Gastrointestinal conditions
      1. Intussusception (mural hematoma is lead point) in 5% of cases
      2. Gastrointestinal Bleeding
      3. Bowel infarction
    3. Neurologic conditions
      1. Seizures
      2. Mononeuropathies
    4. Renal disorders: Crescentic glomeruloneprhitis
      1. Renal Failure
      2. Hematuria
      3. Proteinuria
    5. Male genitourinary conditions
      1. Orchitis
      2. Testicular Torsion
  10. Differential Diagnosis (based on predominant presenting symptom)
    1. Purpura
      1. Hypersensitivity Vasculitis
        1. Elevated Renal Function tests (BUN, Creatinine)
        2. Global organ involvement
      2. Meningococcal Meningitis or Septicemia
      3. Idiopathic Thrombocytopenic Purpura
      4. Child Abuse
      5. Bacterial Endocarditis
      6. Rheumatic Fever
      7. Rocky Mountain Spotted Fever
      8. Drug Reactions
      9. Polyarteritis Nodosa
      10. Leukemia
      11. Kawasaki Disease
    2. Arthritis
      1. Rheumatoid Arthritis
      2. Systemic Lupus Erythematosus
      3. Granulomatosis with Polyangiitis (previously known as Wegener's Granulomatosis)
    3. Abdominal Pain
      1. Acute Abdomen
      2. Familial Mediterranean Fever
      3. Inflammatory Bowel Disease
  11. Diagnosis: International Consensus Conference
    1. Major criteria (required)
      1. Palpable Purpura in the absence of Thrombocytopenia
    2. Minor criteria (requires 1 of the following)
      1. Diffuse Acute Abdominal Pain
      2. Biopsy showing predominant IgA deposition
      3. Arthritis or Arthralgia involving any joint
      4. Renal involvement presenting as Proteinuria or Hematuria
  12. Labs: Initial
    1. Complete Blood Count (CBC)
      1. Leukocytosis with Eosinophilia
      2. Platelets may be elevated
        1. Low Platelets suggest Thrombocytopenic Purpura
    2. Sedimentation rate (ESR) variably elevated
    3. Urinalysis: Nephritis evaluation (nephrology evaluation if positive)
      1. Most important lab in suspected HSP
      2. Hematuria or Proteinuria in up to 50% of patients (risk of ESRD in 1% of patients over subsequent months)
    4. Stool Guaiac
      1. Occult or gross blood may be present
    5. Renal Function tests (BUN, Creatinine)
      1. Obtain if positive urine for Hematuria or Proteinuria
      2. Elevation may suggest Hypersensitivity Vasculitis
    6. Coagulation Studies (PTT and INR)
      1. Normal in HSP
      2. Consider in differential diagnosis for Purpura
  13. Labs: Other
    1. Consider ASO Titer
    2. Consider Blood Culture (in differential diagnosis for Purpura)
  14. Labs: Histology
    1. Skin Biopsy
      1. Leukocytoclastic Vasculitis
    2. Renal Biopsy
      1. Glomerular crescents
      2. Indistinguishable from IgA Nephropathy
  15. Imaging
    1. Not routinely indicated
    2. Abdominal Ultrasound or CT Abdomen
      1. Indicated for concurrent gastrointestinal symptoms suggestive of alternative diagnosis
    3. Barium Enema
      1. Indicated if Intussusception is suspected
  16. Management
    1. Supportive care (Primary strategy)
      1. Hydration
      2. Relative rest
      3. Elevate legs (may reduce Purpura)
    2. Joint Pain
      1. NSAIDs (with caution)
        1. Risk of renal disease
        2. Risk of Gastrointestinal Bleeding
    3. Nephritis (Hematuria or Proteinuria)
      1. Nephrology Consultation
      2. Renal biopsy
      3. Children with mild to moderate renal disease
        1. Systemic Corticosteroids (see below)
      4. Adults and children with moderate to severe disease
        1. High dose Corticosteroids with immunosuppressants (e.g. Azathioprine, Cyclophosphamide) or
        2. High dose IV Ig
        3. Plasmapheresis
  17. Management: Systemic Corticosteroids
    1. Indications
      1. Children with renal involvement
      2. Children with severe extrarenal symptoms (e.g. Abdominal Pain, Joint Pain)
      3. Scrotal Swelling
    2. Dosing
      1. Prednisone 1-2 mg/kg orally daily for two weeks
  18. Management: Hospitalization indicated
    1. Severe dehydration
    2. Intractable pain or Abdominal Pain requiring serial examination and observation
    3. Gastrointestinal Hemorrhage
  19. Course
    1. Onset over days to weeks
    2. Duration: 4-6 weeks
    3. Recurrence in 50% of patients
  20. Prognosis
    1. Excellent in general
      1. Resolves spontaneously in 94% of children
      2. Resolves spontaneously in 89% of adults
    2. Renal Disease develops in 5% (<1% develop ESRD)
      1. Although up to 50% will have Hematuria or Proteinuria
    3. Predictors of serious nephropathy or ESRD
      1. Bloody stools
      2. Rash persistence
      3. Nephritis-Nephrotic Signs
        1. Progresses to ESRD within 10 years in 50% of cases
      4. Renal Biopsy with glomerular crescents
        1. Progresses to ESRD in 100% of cases
  21. Monitoring: Renal involvement screening
    1. Blood Pressure initially and at each subsequent visit following the HSP diagnosis
    2. If first Urinalysis is normal (or isolated Hematuria)
      1. Monthly Urinalysis for 6 months after HSP diagnosis
    3. If any Urinalysis suggests nephritis (Hematuria and Proteinuria)
      1. Serum Creatinine
      2. Blood Urea Nitrogen
  22. References
    1. Kraft (1998) Am Fam Physician 58(2): 405-408 [PubMed]
    2. Gedalia (2004) Curr Rheumatol Rep 6(3):195-202 [PubMed]
    3. Reamy (2009) Am Fam Physician 80(7): 697-704 [PubMed]
    4. Saulsbury (2007) Lancet 369(9566): 976-8 [PubMed]
    5. Saulsbury (2001) Curr Opin Rheumatol 13(1):35-40 [PubMed]

Henoch-Schoenlein Purpura (C0034152)

Definition (NCI) A systemic, usually self-limited immune complex vasculitis, characterized by immunoglobulin A deposition in the small vessels and kidneys. It is manifested with small hemorrhages in the skin, gastrointestinal symptoms, arthritis, and nephropathy.
Definition (MSH) A systemic non-thrombocytopenic purpura caused by HYPERSENSITIVITY VASCULITIS and deposition of IGA-containing IMMUNE COMPLEXES within the blood vessels throughout the body, including those in the kidney (KIDNEY GLOMERULUS). Clinical symptoms include URTICARIA; ERYTHEMA; ARTHRITIS; GASTROINTESTINAL HEMORRHAGE; and renal involvement. Most cases are seen in children after acute upper respiratory infections.
Concepts Disease or Syndrome (T047)
MSH D011695
ICD9 287.0
ICD10 D69.0
SnomedCT 191306005, 367437009, 31912009, 246074004, 267565009, 191305009, 154823001, 21148002, 191308006
English Henoch-Schoenlein purpura, Allergic Purpura, Anaphylactoid Purpura, Purpura, Schoenlein Henoch, Purpura, Schoenlein-Henoch, Schoenlein Henoch Purpura, Schoenlein-Henoch Purpura, PURPURA ALLERGIC, ALLERGIC VASCULAR PURPURA, ANAPHYLACTIC VASCULAR PURPURA, Allergic purpura, HENOCH-SCHONLEIN PURPURA, PURPURA ANAPHYLACTOID, PURPURA VASCULAR ALLERGIC, Purpura, Allergic, Purpura, Anaphylactoid, Allergic purpura NOS, HENOCH SCHONLEIN SYNDROME, Henoch-Schonlein purpura (disorder), Henoch-Schönlein purpura, Henoch-Schönlein purpura (disorder), Henoch Purpura, Purpura, Henoch, Henoch-Scholein purpura, Henoch-Sch@nlein purpura, allergic purpura (diagnosis), allergic vascular purpura (diagnosis), allergic purpura, Henoch Schoenlein Purpura, Henoch-Schoenlein Purpura, Purpura, Henoch-Schoenlein, allergic vascular purpura, Henoch Schonlein purpura, Henoch Schonlein purpura (diagnosis), vascular allergic purpura, Purpura allergic, Allergic vascular purpura, Purpura vascular allergic, Anaphylactic vascular purpura, Purpura anaphylactoid, Anaphylactoid vascular purpura, Schoenlein-Henoch purpura, Henoch Shonlein purpura, Henoch-Schonlein, Henoch-Schonlein purpura, Purpuras, Schonlein-Henoch, Henoch Schonlein Purpura, Schonlein-Henoch Purpuras, Purpuras, Henoch-Schonlein, Henoch Schonlein Purpuras, Purpura, Schonlein Henoch, Henoch-Schonlein Purpuras, Purpura, Henoch Schonlein, Schonlein Purpura, Henoch, Purpura, Schonlein-Henoch, Schonlein Purpuras, Henoch, Schonlein-Henoch Purpura, Henoch-Schonlein Purpura, Purpuras, Henoch Schonlein, Purpura, Henoch-Schonlein, Purpura Henoch(-Schönlein), Purpura, Schoenlein-Henoch [Disease/Finding], henoch shonlein purpura, henoch purpura, henoch schonlein purpura, henoch-schoenlein purpura (HSP), spring fever, henoch schoenlein purpura, anaphylactoid purpura, henoch-schonlein, henoch's purpura, henoch-schoenlein purpura, hsp, henoch scholein purpura, henoch schonlein, henoch-schonlein purpura, henoch-scholein purpura, Henoch-Sch?nlein purpura, Allergic purpura (disorder), Autoimmune purpura (disorder), Henoch-Schonlein all. purpura, Allergic purpura NOS (disorder), Purpura: [allergic] or [Henoch-Schonlein allergy], Purpura: [allergic] or [Henoch-Schonlein allergy] (disorder), Henoch-Sch?nlein purpura (disorder), Anaphylactoid purpura, Autoimmune purpura, Henoch's purpura, Acute vascular purpura, Henoch-Schoenlein vasculitis, Spring fever, HSP - Henoch-Schonlein purpura, Schönlein; purpura, Schönlein, allergic; purpura, purpura; Schönlein, purpura; allergic, purpura; anaphylactoid, anaphylactoid; purpura, Autoimmune purpura (disorder) [Ambiguous], Purpura, anaphylactoid, Purpura, autoimmune, Purpura;Henoch-Schonlein
French PURPURA ALLERGIQUE, PURPURA ANAPHYLACTOIDE, Purpura vasculaire anaphylactique, Purpura vasculaire allergique, Purpura vasculaire anaphylactoïde, Purpura de Schoenlein-Henoch, Maladie de Shölen-Henoch, Henoch-Schonlein, PURPURA DE HENOCH- SCHONLEIN, PURPURA VASCULAIRE ALLERGIQUE, PURPURA VASCULAIRE ANAPHYLACTIQUE, Péliose rhumatismale, Purpura allergique, Purpura anaphylactoïde, Maladie de Schönlein-Henoch, Purpura de Schönlein-Henoch, Syndrome de Schönlein-Henoch, Purpura de Henoch-Schönlein
Portuguese PURPURA ALERGICA, PURPURA ANAFILACTOIDE, Púrpura vascular anafilactóide, Púrpura vascular alérgica, Púrpura alérgica, Púrpura anafilactóide, Púrpura vascular anafiláctica, Schönlein-Henoch, Púrpura de Henoch, PURPURA ANAFILACTICA VASCULAR, PURPURA DE HENOCH-SCHOENLEIN, PURPURA VASCULAR ALERGICA, Púrpura Anafilactoide, Púrpura de Schönlein-Henoch, Púrpura Alérgica, Púrpura de Henoch-Schoenlein, Púrpura de Schoenlein-Henoch
Spanish PURPURA ALERGICA, PURPURA ANAFILACTOIDE, Púrpura alérgica, Púrpura vascular alérgica, Púrpura anafilactoide, Púrpura vascular anafilactoide, Henoch-Schönlein, Púrpura vascular anafiláctica, Purpura alérgica, Purpura vascular alérgica, Púrpura anafiláctoide, púrpura de Henoch-Schönlein (trastorno), púrpura de Henoch-Schönlein, púrpura de Schönlein-Henoch (trastorno), púrpura de Henoch-Shonlein (trastorno), vasculitis de Henoch-Shonlein, púrpura de Henoch-Shonlein, Púrpura de Henoch, PURPURA DE HENOCH-SCHONLEIN, PURPURA VASCULAR ALERGICA, PURPURA VASCULAR ANAFILACTICA, púrpura de Schönlein-Henoch, Purpura: [allergic] or [Henoch-Schonlein allergy], púrpura alérgica, SAI (trastorno), púrpura autoinmunitaria (trastorno), Allergic purpura, Henoch-Schonlein all. purpura, púrpura autoinmunitaria, púrpura de Schonlein-Henoch (trastorno), púrpura anafilactoide, SAI, púrpura de Scholein-Henoch, púrpura autoinmune, púrpura alérgica (trastorno), púrpura de Schonlein-Henoch, púrpura de Henoch - Schonlein, Púrpura: [alérgica] o [alergia de Henoch-Schonlein] (trastorno), púrpura alérgica, púrpura alérgica, SAI, Púrpura: [alérgica] o [alergia de Henoch-Schonlein], púrpura de Henoch - Schonlein (trastorno), púrpura anafilactoide, púrpura de Henoch - Schonlein (concepto no activo), púrpura de Henoch, púrpura vascular aguda, Púrpura de Schönlein-Henoch, Púrpura Alérgica, Púrpura Anafilactoide, Púrpura de Henoch-Schoenlein, Púrpura de Schoenlein-Henoch
Italian Porpora di Henoch-Schonlein, Porpora vascolare allergica, Henoch-Schonlein, Porpora vascolare anafilattica, Porpora di Henoch Schonlein, Porpora vascolare anafilattoide, Porpora allergica, Porpora anafilattoide, Porpora di Schoenlein-Henoch
Dutch allergische purpura, anafylactische vasculaire purpura, anafylactoïde vasculaire purpura, purpura van Henoch-Schönlein, anafylactoïde purpura, allergische vasculaire purpura, purpura allergisch vasculair, Schönlein; purpura, allergisch; purpura, anafylactoïd; purpura, purpura; Schönlein, purpura; allergisch, purpura; anafylactoïd, Henoch-Schönlein purpura, Allergische purpura, Henoch-Schoenlein, Henoch-Schoenlein, ziekte van, Schoenlein-Henoch, ziekte van, Anafylactoïde purpura, Henoch-Schönlein, Henoch-Schönlein, syndroom van, Henoch-Schönlein, ziekte van, Schönlein-Henoch, ziekte van
German anaphylaktische vaskulaere Purpura, anaphylaktoide vaskulaere Purpura, Henoch-Shonlein Purpura, Henoch-Schonlein, anaphylaktoide Purpura, allergische Purpura, Purpura vaskulaer allergisch, Purpura allergisch, allergische vaskulaere Purpura, Schoenlein-Henoch Purpura, Purpura anaphylaktoid, ALLERGISCHE VASKULAERE PURPURA, ANAPHYLAKTISCHE VASKULAERE PURPUR, HENOCH-SCHOENLEIN PURPURA, PURPURA ALLERGISCH, PURPURA ANAPHYLAKTISCH, PURPURA VASKULAER ALLERGISCH, Purpura anaphylactoides, Henoch-Schoenlein Purpura, Allergische Purpura, Anaphylaktoide Purpura, Henoch-Schoenlein-Purpura, Purpura Schoenlein-Henoch, Schoenlein-Henoch-Purpura
Japanese アナフィラキシー性血管性紫斑病, アレルギー性血管性紫斑病, ヘノッホ・シェーンライン, アナフィラキシー様血管性紫斑病, アレルギーセイケッカンセイシハンビョウ, ヘノッホシェーンライン, アレルギーセイシハンビョウ, アナフィラキシーセイケッカンセイシハンビョウ, アナフィラキシーヨウケッカンセイシハンビョウ, アナフィラキシーヨウシハンビョウ, シェーンライン・ヘノッホ紫斑病, シェーンラインヘノッホシハンビョウ, ヘノッホシェーンラインシハンビョウ, ヘノッホ・シェーンライン紫斑病, アナフィラキシー様紫斑病, アナフィラクトイド紫斑病, 血管炎-出血性, ヘーノホ-シェーンライン紫斑病, ヘーノホ紫斑病, リウマチ性紫斑病, アナフィラクトイド紫斑, 血小板非減少性紫斑, 紫斑病-シェーンライン-ヘノッホ, ヘノッホ紫斑病, 出血性血管炎, 紫斑病-アレルギー性, Henoch-Schonlein紫斑病, アナフィラキシー紫斑病, 紫斑病-血小板非減少性, シェーンラインヘノッホ紫斑病, Schoenlein-Henoch紫斑病, シェーンライン-ヘノッホ紫斑病, 血小板非減少性紫斑病, 紫斑病-ヘノッホ, Schonlein-Henoch紫斑病, 紫斑病-リウマチ様, リウマチ様紫斑病, アレルギー性紫斑病, 紫斑病-アナフィラキシー様, シェーンライン・ヘノッホ紫斑病, 紫斑性出血, Henoch-Schoenlein症候群, 紫斑病-Schoenlein-Henoch
Swedish Purpura, Schoenlein-Henochs
Finnish Henoch-Schönleinin purppura
Czech Alergická purpura, Anafylaktická vaskulární purpura, Schoenlein-Henochova purpura, Henoch-Schoenlein, Anafylaktoidní purpura, Henoch-Schoenleinova purpura, Alergická vaskulární purpura, Anafylaktoidní vaskulární purpura, Henoch-Schönleinova purpura, anafylaktoidní purpura, Schönlein-Henochova purpura, alergická purpura, purpura Schoenleinova-Henochova, Henochova-Schönleinova purpura
Korean 알레르기성 자색반증
Polish Plamica reumatyczna, Plamica Schoenleina-Henocha, Plamica alergiczna, Choroba Schoenleina-Henocha, Zespół Schoenleina-Henocha
Hungarian Henoch-Schonlein purpura, Anaphylactikus vascularis purpura, Anaphylactoid vascularis purpura, Schoenlein-Henoch purpura, Henoch-Schonlein, Henoch-Shonlein purpura, Allergiás purpura, Allergiás vascularis purpura, Anaphylactoid purpura, Purpura allergiás, Purpura anaphylactoid, Purpura vascularis allergiás
Norwegian Allergisk purpura, Henoch-Schönleins purpura, Henoch-Schoenleins purpura
Derived from the NIH UMLS (Unified Medical Language System)

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