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Farhan Tahir MD, FACR
Rheumatic Disease Associates
     Willow Grove, PA
• Case review
• Purpura : causes and types
• Many faces of Purpuric rash
• Purpura from a Rheumatologist's eyes
• Discussion
• Conclusion
•Rash-Petechial purpura and ecchymosis (no prior history)
•Chronicity: over 3 months, progressive
•Preceding events: no infectious, traumatic or chemical
exposure, no herbal supplement exposure, no new drug
•Systemic features: dyspnea and fatigue, no
fever, diarrhea, hemoptysis
•Potential culprit medications: ASA, Prednisone
,Cellcept, Gabapentin
•Nutrition: Poor (peanut butter sandwich, no multivitamins)
•Co morbid conditions: SLE, chronic steroid use, osteoporosis
Med/Surg hx: Mitral valve prolapse, Hip replacement and
partial hysterectomy (no history of excessive bleeding)

Family: no bleeding diathesis

Social: lives alone, no drugs or alcohol abuse

Exam: Poor dental hygiene, tender bruising over
thighs, petechial lesions, 1+peripheral edema, normal
pulses in feet
•Purpura (from Latin: purpura, meaning
"purple")
  • Bleeding under skin or into mucosal
    membranes
  • Pinpoint area, < 2mm: petechiae
  • Larger confluent lesions: ecchymosis
    (bruises)
•Causes of Purpura
  • Disruption of vascular integrity
  • Primary or secondary hemostatic abnormality
Destruction of vascular wall
  •   Trauma, inflammation or infection
Impaired platelet plug and fibrin clot
  •   Injury-> vasoconstriction & retraction->platelet
      recognition vessel endothelial adhesion ->platelet granules-
      >platelet plug
  •   Tissue factor-VII complex->activation of coagulation
      cascade->Fibrin cross links->clot
Impaired collagen synthesis
  •   Disordered collagen and connective tissue synthesis and
      structure, congenital versus acquired
Small vessel vasculitis




 Arch Bronconeumol. 2008;44(8):428-36
• Palpable Purpura is usually inflammatory or
vasculitis
•   Hypersensitivity vasculitis
•   Henoch-Scolein purpura
•   SLE, RA and small vessel vasculitis
•   Infectious
•   Drug induced
•   Polyarteritis Nodosa
•   Pseudovasculitis
•   Non palpable purpura : Non vasculitic

Corticosteroid use
Idiopathic thrombocytopenic purpura
Thrombotic thrombocytopenic purpura
Disseminated intravascular coagulation
Vitamin K deficiency
Scurvy
•Platelet disorders (quantitative ) :
      Thrombocytopenia
•Platelet disorders (functional):
      Glycoprotein disorders
•Hemophilia
•Factor inhibitors
•Leukemia
•Disseminated intravascular coagulation
•Vitamin K Deficiency
•Scurvy
Hematology
WBC: 3.0, HGB: 10.5, platelets: 235 MCV: 82

Comprehensive metabolic panel
CR: 0.8 Ca:9.7, AST: 35, ALT:40, Albumin of 2.9

Cagulation profile
PT11.6/INR 1.1, PTT 21,
Factor Vll, lX X and Von WIllibrand Factor assays-normal,
Bleeding time- 6.3min(2.3-9.5min),

Rheumatologic profile
ESR 25, DSDNA:NEG, , C3: 167 (nl), C4:18(nl)
ANCA: NEG , PR3:NEG, Myeloperoxidase Ab: neg

Leukemic, malignancy
Normal wbc profile, SPEP: NEG, UPEP: NEG.
Questions to ask ourselves
Urticarial vasculitis and purpura?
Conditions that may present with a clinical syndrome mimicing
vasculitis:
(1) atrial myxoma
(2) septicemia
(3) chronic microthromboembolism
(4) infective endocarditis (both septic and microthrombemboli)
(5) rejection after organ transplantation
(6) drug or chemical related (ergotism, cocaine, etc)
Pseudovasculitis ?
Cocaine induced…
Drug induced
Thrombocytopenia ?
                     DIC ?
• Normal platelet and wbc suggesting against thrombocytopenia
  and bone marrow suppression : ITP, TTP

• Normal PT and PPT and bleeding time, goes against
  coagulation factor deficiencies states DIC, clotting factor
  inhibitors and vitamin K deficiency

• Normal inflammatory markers, absence of elevated dsDNA and
  hypocomplementemia suggests against a lupus vasculitis

• Absence of pulmonary renal failure, negative serologies for
  ANCA associated small vessels vasculitis
•Echocardiogram: normal ( excludes CHF, Pulm HTN)
•Doppler negative for DVT

•Colonoscopy- negative ( GI bleeding, HSP)
•V/Q scan: low probability for PE

•CT of the lower extremity showed a soft tissue
infiltration in the medial thigh (bleeding)
Risk of vitamin C deficiency
• Scurvy: dietary deficiency of vegetables and vitamins
• Deficiency can cause typical perifollicular hemorrhage, collagen
defect leads to superficial and deep tissue hemorrhage
Diagnosis : Scurvy
•Syptoms including fatigue, purpuric rash, synovitis with effision,
anemia and markedly elevated ESR and CRP
•One patient presenetd with severe pulmonary hypertension
•Exam consistnt with hemarthrosis and classic skin findings
•Treatment with Vitamin C 500mg-1000mg daily adequately replensihes
•Body stoes
•1-3 weeks for resolution of skin findings
•1-3 moths for hematologic and hemarthrosis
Pupura In A Patient With Lupus

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Pupura In A Patient With Lupus

  • 1. Farhan Tahir MD, FACR Rheumatic Disease Associates Willow Grove, PA
  • 2. • Case review • Purpura : causes and types • Many faces of Purpuric rash • Purpura from a Rheumatologist's eyes • Discussion • Conclusion
  • 3. •Rash-Petechial purpura and ecchymosis (no prior history) •Chronicity: over 3 months, progressive •Preceding events: no infectious, traumatic or chemical exposure, no herbal supplement exposure, no new drug •Systemic features: dyspnea and fatigue, no fever, diarrhea, hemoptysis •Potential culprit medications: ASA, Prednisone ,Cellcept, Gabapentin •Nutrition: Poor (peanut butter sandwich, no multivitamins) •Co morbid conditions: SLE, chronic steroid use, osteoporosis
  • 4. Med/Surg hx: Mitral valve prolapse, Hip replacement and partial hysterectomy (no history of excessive bleeding) Family: no bleeding diathesis Social: lives alone, no drugs or alcohol abuse Exam: Poor dental hygiene, tender bruising over thighs, petechial lesions, 1+peripheral edema, normal pulses in feet
  • 5. •Purpura (from Latin: purpura, meaning "purple") • Bleeding under skin or into mucosal membranes • Pinpoint area, < 2mm: petechiae • Larger confluent lesions: ecchymosis (bruises) •Causes of Purpura • Disruption of vascular integrity • Primary or secondary hemostatic abnormality
  • 6. Destruction of vascular wall • Trauma, inflammation or infection Impaired platelet plug and fibrin clot • Injury-> vasoconstriction & retraction->platelet recognition vessel endothelial adhesion ->platelet granules- >platelet plug • Tissue factor-VII complex->activation of coagulation cascade->Fibrin cross links->clot Impaired collagen synthesis • Disordered collagen and connective tissue synthesis and structure, congenital versus acquired
  • 7.
  • 8.
  • 9. Small vessel vasculitis Arch Bronconeumol. 2008;44(8):428-36
  • 10. • Palpable Purpura is usually inflammatory or vasculitis • Hypersensitivity vasculitis • Henoch-Scolein purpura • SLE, RA and small vessel vasculitis • Infectious • Drug induced • Polyarteritis Nodosa • Pseudovasculitis
  • 11. Non palpable purpura : Non vasculitic Corticosteroid use Idiopathic thrombocytopenic purpura Thrombotic thrombocytopenic purpura Disseminated intravascular coagulation Vitamin K deficiency Scurvy
  • 12. •Platelet disorders (quantitative ) : Thrombocytopenia •Platelet disorders (functional): Glycoprotein disorders •Hemophilia •Factor inhibitors •Leukemia •Disseminated intravascular coagulation •Vitamin K Deficiency •Scurvy
  • 13. Hematology WBC: 3.0, HGB: 10.5, platelets: 235 MCV: 82 Comprehensive metabolic panel CR: 0.8 Ca:9.7, AST: 35, ALT:40, Albumin of 2.9 Cagulation profile PT11.6/INR 1.1, PTT 21, Factor Vll, lX X and Von WIllibrand Factor assays-normal, Bleeding time- 6.3min(2.3-9.5min), Rheumatologic profile ESR 25, DSDNA:NEG, , C3: 167 (nl), C4:18(nl) ANCA: NEG , PR3:NEG, Myeloperoxidase Ab: neg Leukemic, malignancy Normal wbc profile, SPEP: NEG, UPEP: NEG.
  • 14. Questions to ask ourselves
  • 15.
  • 16.
  • 17.
  • 18.
  • 20.
  • 21.
  • 22.
  • 23.
  • 24. Conditions that may present with a clinical syndrome mimicing vasculitis: (1) atrial myxoma (2) septicemia (3) chronic microthromboembolism (4) infective endocarditis (both septic and microthrombemboli) (5) rejection after organ transplantation (6) drug or chemical related (ergotism, cocaine, etc)
  • 26.
  • 27.
  • 29.
  • 30.
  • 31.
  • 32. • Normal platelet and wbc suggesting against thrombocytopenia and bone marrow suppression : ITP, TTP • Normal PT and PPT and bleeding time, goes against coagulation factor deficiencies states DIC, clotting factor inhibitors and vitamin K deficiency • Normal inflammatory markers, absence of elevated dsDNA and hypocomplementemia suggests against a lupus vasculitis • Absence of pulmonary renal failure, negative serologies for ANCA associated small vessels vasculitis
  • 33. •Echocardiogram: normal ( excludes CHF, Pulm HTN) •Doppler negative for DVT •Colonoscopy- negative ( GI bleeding, HSP) •V/Q scan: low probability for PE •CT of the lower extremity showed a soft tissue infiltration in the medial thigh (bleeding)
  • 34. Risk of vitamin C deficiency • Scurvy: dietary deficiency of vegetables and vitamins • Deficiency can cause typical perifollicular hemorrhage, collagen defect leads to superficial and deep tissue hemorrhage
  • 35.
  • 36.
  • 38. •Syptoms including fatigue, purpuric rash, synovitis with effision, anemia and markedly elevated ESR and CRP •One patient presenetd with severe pulmonary hypertension •Exam consistnt with hemarthrosis and classic skin findings •Treatment with Vitamin C 500mg-1000mg daily adequately replensihes •Body stoes •1-3 weeks for resolution of skin findings •1-3 moths for hematologic and hemarthrosis