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Approach to child with purpura

Approach to child with purpura. Ahmed S.Barefah KAAU,MBBS. Questions . What is the definition of purpuric rash? What are the causes of purpura ? How to approach such a case?. Definition . red, nonblanching maculopapular lesions caused by intradermal capillary bleeding.

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Approach to child with purpura

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  1. Approach to child with purpura Ahmed S.Barefah KAAU,MBBS

  2. Questions • What is the definition of purpuric rash? • What are the causes of purpura? • How to approach such a case?

  3. Definition • red, nonblanchingmaculopapular lesions caused by intradermal capillary bleeding. • classified as • petechiae (pinpoint hemorrhages less than 2 mm in greatest diameter), • purpura (2 mm to 1 cm) or • ecchymoses (more than 1 cm). • results from the extravasation of blood from the vasculature into the skin or mucous membranes.

  4. Causes Pupura Platelet Vascular Count Function Congenital Acquired

  5. Thrombocytopenia • may be caused by • increased platelet destruction • Immune • nonimmune • decreased platelet production • Congenital • Acquired • sequestration of platelets.

  6. Immune Thrombocytopenia • Idiopathic (immune) thrombocytopenic purpura • by far the most common etiology of thrombocytopenia in childhood. • caused by the development of IgGautoantibodies to platelet membrane antigens as a result of an unbalanced response to an infectious agent or autoimmunity • sudden onset of bruises, purpura, mucosal hemorrhage and petechiae in a child who is otherwise in excellent health.

  7. ITP • antecedent viral infection is common. • The peak incidence is between two and four years of age. • 80 to 90 percent of children recovering within six to 12 months. • Chronic idiopathic thrombocytopenic purpura is more likely to present in teenage girls and children with underlying immune disorders. It has a more insidious onset

  8. Immune Thrombocytopenia • Drugs • penicillin, valproic acid (Depakene), quinidine, sulfonamides, cimetidine (Tagamet) and heparin. • Post-transfusion purpura • acute onset of thrombocytopenia approximately five to 14 days after a transfusion. • Rarely • HIV, CMV, HSV • 10% of SLE cases

  9. Immune Thrombocytopenia • Neonatal isoimmune (alloimmune) thrombocytopenia • Neonatal autoimmune thrombocytopenia

  10. Non-Immune Thrombocytopenia • hemolytic-uremic syndrome • triad of microangiopathic hemolytic anemia, thrombocytopenia and acute renal injury. • infection by verocytotoxin-producing Escherichia coli • thrombotic thrombocytopenic purpura • occurs more often in adults, and neurologic (rather than renal) symptoms are more prominent

  11. Non-Immune Thrombocytopenia • disseminated intravascular coagulopathy • overwhelming sepsis, incompatible blood transfusion, snake bite, giant hemangioma and malignancy. • Purpurafulminans • acute, often lethal syndrome of DIC. • may develop because of a severe bacterial infection, notably meningococcal disease, or because of protein C or S deficiency

  12. Decreased Platelet Production • Congenital • Thrombocytopenia­absent radii (TAR) syndrome • inherited as an autosomal recessive trait • Fanconi anemia • pancytopenia, hyperpigmentation and café au lait spots, short stature, skeletal abnormalities • Wiskott-Aldrich syndrome

  13. Decreased Platelet Production • Acquired • Bone marrow suppression • Drugs such as alkylating agents, antimetabolites, anticonvulsants, chlorothiazide diuretics and estrogens • Infection as viral and bacterial infections, especially septicemia and Intrauterine infection with TORCH organisms • Bone marrow infiltration • patients with leukemia, histiocytosis, storage diseases, neuroblastoma, myelofibrosis and osteopetrosis

  14. Sequestration of Platelets • Splenomegaly or giant hemangioma can result in thrombocytopenia because of platelet sequestration. • The association of thrombocytopenia and giant hemangioma is referred to as Kasabach-Merritt syndrome.

  15. Platelet Dysfunction • Glanzmann'sthrombasthenia • autosomal recessive disorder caused by congenital deficiency in the platelet membrane glycoproteinsIIb and IIIa. • Bernard-Soulier disease • autosomal recessive disorder caused by a congenital deficiency in platelet membrane glycoprotein Ib

  16. Vascular Factors • Congenital Causes • Hereditary hemorrhagic telangiectasia • autosomal dominant disorder • development of fragile telangiectasia of the skin and mucous membranes • Ehlers-Danlos syndrome • characterized by skin hyperelasticity, joint hypermobility and fragility of the skin and blood vessels

  17. Vascular Factors Acquired Causes • Henoch-Schönleinpurpura • IgA-mediated systemic vasculitis of small blood vessels • nonthrombocytopenicpurpura, abdominal pain, arthritis and nephritis • the most common form of vasculitis in children • history of a preceding URTI • Characteristically palpable, gravity dependant purpura

  18. Vascular Factors Acquired Causes • Meningococcemia and rickettsial diseases may cause direct damage to blood vessels, with resultant purpura. • Child abuse

  19. History • Age of Onset • Birth   Intrauterine infection, maternal idiopathic thrombocytopenic purpura, maternal systemic lupus erythematosus, maternal medication, TAR syndrome, congenital amegakaryocytic thrombocytopenia • 2 to 4 years   Idiopathic thrombocytopenic purpura • 4 to 7 years   Henoch-Schönleinpurpura

  20. History • Onset/chronicity • Acute onset   ITP, HSP, medication, mechanical cause • Long duration   Abnormality of platelets, coagulopathy

  21. History • Pattern of bleeding • Mucosal bleeding   Thrombocytopenia, von Willebrand's disease • Intramuscular and intra-articular bleeding   Hemophilia

  22. History • Associated symptoms • Abdominal pain, blood in stools, joint pain   HSP • Lethargy, fever, bone pain   Leukemia • Intermittent fever, muscoskeletal symptoms   SLE • Lethargy, polyuria, polydipsia, failure to thrive   Uremia • Purpura, but otherwise healthy   ITP

  23. History • Past health • Antecedent viral infection, especially an upper respiratory tract infection   ITP, HSP • Drug use • Family history • Maternal history • Social history

  24. Examination • General findings • Poor growth   Chronic disorder Fever   Hypertension   Infection Chronic renal failure,renalvasculitis

  25. Examination • Characteristics of purpura • Location on lower extremetiesHenoch-Schönleinpurpura • Location on palms and soles   Rickettsial infection • Palpable purpuraVasculitis

  26. Laboratory Evaluation • A thorough history and a careful physical examination are critical first steps in the evaluation of children with purpura.

  27. RED FLAGS • Fever, • lethargy, • weight loss, • bone pain, • joint pain, • pallor, • Lymphadenopathy • hepatosplenomegaly

  28. Summary • Petechiae and purpura result from a wide variety of underlying disorders and may occur at any age • red, nonblanchingmaculopapular lesions caused by intradermal capillary bleeding • Classified into platelet or vascular causes • Idiopathic thrombocytopenic purpura is the most common cause of thrombocytopenia in children. • Henoch-Schönleinpurpura is the most common form of vasculitis in children, and the purpuric rash is almost always palpable.

  29. Thank you any question ;)

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