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, nonblanching maculopapular 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.

5. Causes
Pupura
Platelet
Vascular
Count
Function
Congenital
Acquired

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

7. Immune Thrombocytopenia
Idiopathic (immune) thrombocytopenic purpura
by far the most common etiology of thrombocytopenia in childhood.
caused by the development of IgG autoantibodies 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.

8. 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

9. 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

10. Immune Thrombocytopenia
Neonatal isoimmune (alloimmune) thrombocytopenia
Neonatal autoimmune thrombocytopenia

11. 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

12. Non-Immune Thrombocytopenia
disseminated intravascular coagulopathy
overwhelming sepsis, incompatible blood transfusion, snake bite, giant hemangioma and malignancy.
Purpura fulminans
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

13. 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

14. 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

15. 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.

16. Platelet Dysfunction Glanzmann's thrombasthenia
autosomal recessive disorder caused by congenital deficiency in the platelet membrane glycoproteins IIb and IIIa.
Bernard-Soulier disease
autosomal recessive disorder caused by a congenital deficiency in platelet membrane glycoprotein Ib

17. 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

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

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

20. 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-Schönlein purpura

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

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

23. 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

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

25. Examination General findings
Poor growth   Chronic disorder Fever   Hypertension   Infection Chronic renal
failure,renal vasculitis

26. Examination Characteristics of purpura
Location on lower extremeties   Henoch-Schönlein purpura
Location on palms and soles   Rickettsial infection
Palpable purpura   Vasculitis

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

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

30. Summary
Petechiae and purpura result from a wide variety of underlying disorders and may occur at any age
red, nonblanching maculopapular 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-Schönlein purpura is the most common form of vasculitis in children, and the purpuric rash is almost always palpable.

31. Thank you any question ;)