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Investigation of purpuric rash

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There are several causes for purpuras..... How to clinically approach a patient with purpuric rash???? List of investigations which are helpful in reaching upto the clinical diagnosis....

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Investigation of purpuric rash

  1. 1. Presented by : Dr. Simrat Jit Kaur Moderator : Dr. Mohanvir Kaur GMC PATIALA
  2. 2.  Disorders of hemostasis of vessels and platelets are grouped as PURPURIC DISORDERS as cutaneous and mucosal bleedings are prominent.
  3. 3. OVERVIEW OF TOPIC  What is PURPURA?  How we can investigate such cases?  How we classify these ?  Do we need the battery of tests?  Whether detailed history carries importance?  What protocol is followed?
  4. 4. DEFINITION  Purpura is the term used to describe the skin lesions that develop when red blood cells extravasate from capillaries.  Purpura refers to either pinpoint lesions called petechiae or more widespread lesions known as ecchymoses.  It can be differentiated from other erythematous lesions by the use of diascopy, which is the application of a glass slide to the border of the lesions. True purpura does not blanch with
  5. 5. WHERE IS THE CAUSE IN PURPURAS ????? ? VASCULAR INVOLVEMENT(Wrong with integrity of vessels) ? PLATELET INVOLVEMENT  IS IT QUANTITATIVE ?  IS IT QUALITATIVE?)
  6. 6. A.PURPURAS DUE TO VASCULAR CAUSE 1. ALLERGIC 2. HEREDITARY 3. ATROPHIC 4. MISCELLANEOUS B.PURPURAS DUE TO THROMBOCYTOPE NIA 1. PLATELET PRODUCTION IS DECREASED 2. PLATELET DESTRUCTION IS INCREASED DUE TO IMMUNE OR NONIMMUNE CAUSE 3. ABNORMAL POOLING C.PURPURAS DUE TO QUALITATIVE DEFECTS IN PLATELETS 1.Bernard-Soulier Syndrome 2.Glanzmann Thrombasthenia 3.Von Willebrand’s disease
  7. 7. 1.Vascular purpura ALLERGIC 1.HENOCH- SCHONLEIN PURPURA (HSP) 2.LEUKOCYT OCLASTIC ANGITIS HEREDITARY 1.H.H.T 2.EHLERS-DANLOS SYNDROME 3.MARFAN SYNDROME 4.PSEUDOXANTHOM A ELASTICUM 5.OSTEOGENESIS IMPERFECTA ATROPHIC 1.SENILE PURPURA 2.SCURVY MISC 1.AMYLOIDOSIS 2.SIMPLE EASY BRUISING 3.FAT EMBOLISM 4.PARAPROTEINE MIAS
  8. 8. 2.Thrombocytopenic Purpuras A.DECREASED PLATELET PRODUCTION 1.HYPOPLASIA OF MEGKAKARYOCYTES 2.INEFFECTIVE MEGKARYOPOIESIS 3.HEREDITARY THROMBOCYTOPENIA 4.DISORDER OF THROMBOPOIETIC CONTROL B. INCREASED PLATELET DESTRUCTION 1. IMMUNE DESTRUCTION (AUTOIMMUNE, ALLOIMMUNE) 2. NON-IMMUNE DESTRUCTION C. ABNORMAL PLATELET DISTRIBUTION OR POOLING 1.DISORDERS OF SPLEEN LIKE INFECTIONS, CONGESTIVE, INFILTERATIVE 2.HYPOTHERMIA 3.DILUTION OF PLATELET WITH MASSIVE TRANSFUSIONS.
  9. 9. Immune thrombocytopenia ( autoimmune) IDIOPATHIC-ACUTE AND CHRONIC ITP ONLY LABELLED AFTER EXCLUDING ALL THE SECONDARY CAUSES SECONDARY CAUSES ARE THERE 1. DRUGS(LONG LIST) 2. INFECTIONS 3. COLLAGEN VASCULAR DISORDERS 4. PREGNANCY 5. LPDS
  10. 10. Immune thrombocytopenia ( alloimmune) 1. NEONATAL THROMBOCYTOPENIA 2.POST TRANSFUSION PURPURAS
  11. 11. Non-immune thrombocytopenia THROMBOTIC MICROANGIOP ATHIES 1.T.T.P 2.HUS 3.DIC ABNORMAL VASCULAR SURFACES CAUSING PLATELET DAMAGE MASSIVE BLOOD TRANSFUSIONS MANY INFECTIONS
  12. 12. Clinical approach to the patient 1.Examination of rash  Appearance of lesions (size, shape, color)  Palpable or non palpable  Location and pattern of involvement.  Associated features ( fever, ulcer, scars).  Look for lesions in mucous membranes.  Presence of tenderness (suggest inflammatory process).
  13. 13. HESS CAPILLARY RESISTANCE TEST ( TORNIQUET TEST )  Pressure in it between diastolic and systolic for 5 minutes.  After deflation, the number of petechiae appearing in the next 5 minutes in 3 cm² area over the cubital fossa are counted.  Presence of more than 20 petechiae is considered a positive test.  The test is positive in 1. Increased capillary fragility 2. Thrombocytopenia.
  14. 14. 2. DETAILED HISTORY  Age : HSP in children, Senile purpura in Elderly.  Sex  Duration of lesion : ?Acute ?chronic.  Associated symptoms : like pain, arthralgia, features of collagen vascular disorders  Past history of similar episode  H/o Drug(with list of drugs in your hand), chemotherapy, radiotherapy  Family history  Is it a spontaneous bruise or after trivial trauma ?  Excessive bleeding after trauma
  15. 15.  Expensive and unnecessary additional laboratory studies can be avoided if importance is given to history  Like h/o Drugs taken provide a clue ?? Is it reducing PLT count ???  Is it causing PLT function defect e.g. Simply aspirin ingestion
  16. 16. 3.SYSTEMIC EXAMINATION  LOOK FOR PALLOR, ANY ORGANOMEGALY OR LYMPHADENOPATHY  ?? LEUKAEMIAS OR ???LPDS  ANY SIGNS OF CONNECTIVE TISSUE DISORDERS  NEUROLOGICAL SIGNS(?TTP )  SYSTEM RELATED FEATURES
  17. 17.  LIST OF INVESTIGATIONS 1. Do we need to go for all the tests at first level??? 2. What is the protocol followed?
  18. 18. List of INVESTIGATIONS  CBC (??IS ANY ASSOCIATED HAEMATOLOGICAL MALIGNANCY)  PLATELET COUNT, TO BE CONFIRMED BY PBF ALSO  MEAN PLATELET VOLUME, PLATELET DISTRIBUTION WIDTH  PBF(?ANY CLUE FOR UNDERLYING CAUSE ?LEUKAEMIAS?RED CELL FRAGMENTATION)  HHH
  19. 19. 4.INVESTIGATIONS cont….  BONE MARROW STUDY  IN SUSPECTED CASES OF DIC ALONG WITH PLATELET COUNT, OR WHEN ALONG WITH PURPURAS THERE IS H/O SPONTANEOUS BLEED (PT, APTT, TT)  AUTOANTIBODIES SCREEN  PLATELET SURVIVAL STUDY, TPO LEVELS  PLATELET FUNCTION TESTS
  20. 20. To start with we need…… Platelet count  Manual  Analyser  Confirmation from PBF  PC <10,000/cmm  10,000- 50,000/cmm  >50,000/cmm Bleeding time ????  Duke’s method  Ivy’s method  Template method
  21. 21.  SCREENING TESTS USUALLY SHOW NORMAL RESULTS IN PURPURAS RELATED TO VASCULAR ABNORMALITIES
  22. 22. Vascular purpuras…. ALLERGIC 1.HENOCH- SCHONLEIN PURPURA (HSP) 2.LEUKOCYT OCLASTIC ANGITIS HEREDITARY 1.H.H.T 2.EHLERS-DANLOS SYNDROME 3.MARFAN SYNDROME 4.PSEUDOXANTHOM A ELASTICUM 5.OSTEOGENESIS IMPERFECTA ATROPHIC 1.SENILE PURPURA 2.SCURVY MISC 1.AMYLOIDOSIS 2.SIMPLE EASY BRUISING 3.FAT EMBOLISM 4.PARAPROTEINE MIAS
  23. 23.  HOW TO INVESTIGATE SUCH CASE WHERE VASCULAR CAUSE IS PRESUMED?  CASE NO. 1
  24. 24. CASE -1  6 yr old child presented with acute colicky abdominal pain, arthritis and lower extremity palpable purpura. There was h/o sore throat, 3 weeks before.  Requisition for routine investigation was done  CBC- Leucocytosis with eosinophillia  PC –Normal  Urine examination revealed mild proteinuria, RBCs and
  25. 25.  Typical clinical history and investigations were suggestive of HENOCH- SCHONLEIN PURPURA CONFIRMATION BY  IMMUNOCOMPLEXES OF IgG AND IgA - MAY BE INCREASED  BIOPSY SHOWING GRANULOCYTES IN THE WALLS OF ARTERIOLES OR VENULES
  26. 26. What is HENOCH-SCHONLEIN PURPURA !  Incidence : 20 per lakh  Acute vasculitic syndrome  Children (4-11 yrs)  Preceded by β-hemolytic streptococcal sore throat, 1-3 weeks before  Deposition of circulating immune complexes within the vessels.
  27. 27. CASE- 2  A 22 year old male presented with complaint of epistaxis* . On examination telangiectasia on tongue and upper lip were appreciated.  h/o similar complaints in elder brother*  Investigations: CBC,
  28. 28.  Typical clinical presentation and family history was suggestive of Hereditary Hemorrhagic Telangiectasia  CONFIRMATION BY BIOPSY  FOLLOW THE CRITERIA FOR DIAGNOSIS  Biopsy : Post capillary venule dilatation, perivascular infiltrate  Mutations in endoglin gene or in the activin receptor–like kinase gene (ALK1) which result in vascular malformations.
  29. 29. SENILE PURPURA FEATURES  Old age  ??Thin skin, atrophy of collagen and loss of fat result in loss of support to small vessels. INVESTIGATIONS  CBC , PC , BT : Normal
  30. 30. SCURVY INVESTIGATIONS : normal  Vitamin C promote peptidyl hydroxylation of procollagen.  Deficiency causes weakened collagen strands predisposing to capillary fragility and delayed wound healing.  History helpsPERIFOLLICULAR HEMORRHAGES IN
  31. 31. CASE- 3  A 58 yr old female presented with bilateral periorbital purpura. The lesions appeared spontaneously without any trauma. No h/o any drug intake. 3 yr earlier she was diagnosed with multiple myeloma
  32. 32.  Clinical presentation and history was suggestive of AMYLOIDOSIS  In this case biopsy was planned for demonstration of amyloid  Biopsy showed amyloid deposits in the dermis and subcutaneous tissues, and scarce inflammatory cells.  light chain deposits in the cutaneous blood vessels.  Fragile vessels resulting in purpura on minor
  33. 33. Purpura Associated with Skin DiseasesPIGMENTED PURPURIC DERMATITIS INVESTIGATION  Biopsy : mononuclear upper dermal infiltrate without evidence of leukocytoclasis, extravasated RBC’s around capillaries & hemosiderin deposits are seen.
  34. 34. 2.Thrombocytopenic Purpuras DECREASED PLATELET PRODUCTION 1.HYPOPLASIA OF MEGKAKARYOCYTES 2.INEFFECTIVE MEGKARYOPOIESIS 3.HEREDITARY THROMBOCYTOPENIA 4.DISORDER OF THROMBOPOIETIC CONTROL B. INCREASED PLATELET DESTRUCTION 1.IMMUNE DESTRUCTION (AUTOIMMUNE, ALLOIMMUNE) 2. NONIMMUNE DESTRUCTION C. ABNORMAL PLATELET DISTRIBUTION OR POOLING 1.DISORDERS OF SPLEEN LIKE INFECTIONS, CONGESTIVE, INFILTERATIVE 2.HYPOTHERMIA 3.DILUTION OF PLATELET WITH MASSIVE TRANSFUSIONS.
  35. 35.  CASE STUDY WHEN PUPURAS ARE DUE TO INVOLVEMENT OF PLATELETS.  SCREENING TESTS WILL BE ABNORMAL IN QUANTITATIVE DEFECTS
  36. 36. CASE- 4  26 yr old female presented with repeated episodes of epistaxis, bleeding from gums, ecchymoses and petechiae around the ankles from last 6 months.  On detailed history & examination there was no h/o drug intake, no similar family history, no collagen disorder, no organomegaly Investigations CBC : Hb decreased  TLC , DLC : normal  PC decreased  MPV high  Platelet distribution width increased  Reticulocytosis PBF  Normocytic normochromic anaemia  Platelets were abnormally large and there was more variation in size and shape.
  37. 37. PBF Showing thrombocytopenia and large platelet
  38. 38.  Bone marrow was planned for labeling whether it was amegakaryocytic thrombocytopenia or megakaryocytic thrombocytopenia and to study the morphology.
  39. 39.  Diagnosis of ITP was made after excluding all other causes of thrombocytopenia  Demonstration of antiplatelet antibodies (specificity - 78 to 93% , sensitivity 49 to 66%)
  40. 40. What is ITP?  Incidence 1.6/10,000 ACUTE ITP  Thrombocytopenia occurring for <6 months  Usually resolving spontaneously  Children (2 and 6 years of age)  Eosinophillia and lymphocytosis common  F:M=1:1 CHRONIC ITP  Last for >6 months  Require therapy to improve the thrombocytopenia.  Adults.  F:M=2 to 3:1 PATHOPHYSIOLOGY
  41. 41. THROMBOCYTOPENIC PURPURA SECONDARY TO DRUGS  NOTING ON DRUG INTAKE WILL HELP  Drug induced purpuras Rifampicin(6 per 100 cases), Quinidine, quinine, Heparin (3 per 100), acetaminophen, trimethoprim- sulfamethoxazole, danazol, methyldopa, digoxin, gold(1 per 100), procainamide, carbamazepine, hydrochlorothiazide, ranitidine, chlorpropamide etc
  42. 42. SLE FEATURES  Specific platelet autoantibodies or immune complexes get deposited on platelets.  Malar rash INVESTIGATION  CBC: anaemia, lymphopenia, leukopenia  PC : decreased  BT : prolonged  Positive antinuclear antibody  Platelet autoantibodies
  43. 43. Cutaneous Leukocytoclastic Vasculitis  SKIN LESIONS  HHH  Confirmation by Skin biopsy: Fibrinoid necrosis, leukocytoclasis, inflammatory infiltrate  ANTIBODIES
  44. 44. CASE- 5  A 35 yr old male presented with fever, headache, joint pains, and rash over back in the month of Oct-Nov for 1 week.  Investigations :-positive tourniquet test, decreased platelet count, leukopenia, lymphocytosis  ??? Could be viral etiology  NS1 Ag & Dengue serology was planned which came out to be positive
  45. 45.  Suggestive history  Thrombocytopenia, Leucopenia  NS1 Ag/Ig M  No other tests needed  Diagnosis of DENGUE HEMORRHAGIC FEVER was made.
  46. 46. PURPURA IN INFECTIONS FEATURES  BACTERIAL AND VIRAL CAUSE  MECHANISM : DIC, vasculitis, septic emboli, vascular toxins (??direct vascular or endothelial invasion). INVESTIGATIONS  CBC : TLC- increased  PC : Normal or decreased  BT : prolonged  SEROLOGIC STUDIES : RISE IN ANTIBODY TITERS  Biopsy of the skin lesions with immunofluorescent identification of the organisms.
  47. 47. NEONATAL THROMBOCYTOPENIA FEATURES  Incidence : 1 in 1000  Result from the placental transfer of platelet antibodies formed as the result of active immunization of the mother by fetal platelet antigens. INVESTIGATION  PC : decreased  ????? Platelet antigen incompatibility between the parents  Presence of maternal antipaternal platelet antibodies
  48. 48. Non-immune thrombocytopenia THROMBOTIC MICROANGIOP ATHIES 1.T.T.P 2.HUS 3.DIC ABNORMAL VASCULAR SURFACES CAUSING PLATELET DAMAGE MASSIVE BLOOD TRANSFUSIONS MANY INFECTIONS
  49. 49. CASE- 6  A 38 yr old female presented with purpuras, fever and seizures  Investigations -CBC : anaemia, neutrophillia, thrombocytopenia, increased reticulocyte count  PBF : polychromasia, nucleated red cells, schistocytes .
  50. 50.  RFT – Blood urea and serum creatinine were elevated  LDH, unconjugated bilirubin were increased  Haptoglobin levels were reduced  Hemoglobinuria and Hemosiderinuria was present.
  51. 51. D/D  THROMBOTIC THROMBOCYTOPENIC PURPURA  HAEMOLYTIC UREMIC SYNDROME  Age group of the patient and no h/o haemorrhagic diarrhea ruled out the possibility of Haemolytic uraemic syndrome
  52. 52. THROMBOTIC THROMOCYTOPENIC PURPURA  Incidence : 2 to 7 per million  Hereditary : Mutations of ADAMTS13 gene  Acquired : abnormal inhibitors
  53. 53. TTP…….  BONE MARROW : infrequently performed, but may reveal erythroid hyperplasia, increased numbers of megakaryocytes, and occasionally microvascular hyaline thrombi.  ADAMTS13 activity levels are <10% of normal.
  54. 54. Summary  Detail history, clinical examination, indicated investigations as suggested in the history can guide for d/d of purpuras  Unnecessary burden and expenses can be avoided  Cause of purpuras can be made and treated accordingly.

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