overview of all purpuric conditions in children

1. APPROACH TO CHILD
WITH PURPURA
By Muhammad Jawad.

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
o petechiae (pinpoint hemorrhages less than 2 mm
in greatest diameter),
o purpura (2 mm to 1 cm) or
o 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
Functio
n
Count
Congenita
l
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 verotoxin-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
should be considered in young males having
thrombocytopenia with small platelets.

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, storage diseases,
neuroblastoma, 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
 Characteristics of purpura
 Location on lower extremeties Henoch-
Schönlein purpura
 Location on palms and soles Rickettsial
infection
 Palpable purpura Vasculitis

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

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

29. 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.