Transcript 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.
• 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.
Causes
Pupura
Platelet
Count
Function
Vascular
Congenital
Acquired
Thrombocytopenia
• may be caused by
– increased platelet destruction
• Immune
• nonimmune
– decreased platelet production
• Congenital
• Acquired
– sequestration of platelets.
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.
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
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
Immune Thrombocytopenia
• Neonatal isoimmune (alloimmune)
thrombocytopenia
• Neonatal autoimmune thrombocytopenia
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
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
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
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
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 KasabachMerritt syndrome.
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
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
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
Vascular Factors
Acquired Causes
• Meningococcemia and rickettsial diseases may
cause direct damage to blood vessels, with
resultant purpura.
• Child abuse
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
History
• Onset/chronicity
• Acute onset ITP, HSP, medication, mechanical
cause
• Long duration Abnormality of platelets,
coagulopathy
History
• Pattern of bleeding
• Mucosal bleeding Thrombocytopenia, von
Willebrand's disease
• Intramuscular and intra-articular
bleeding Hemophilia
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
History
• Past health
• Antecedent viral infection, especially an upper
respiratory tract infection
ITP, HSP
• Drug use
• Family history
• Maternal history
• Social history
Examination
• General findings
• Poor growth
Chronic disorder
Fever Hypertension Infection Chronic renal
failure,renal vasculitis
Examination
• Characteristics of purpura
• Location on lower extremeties HenochSchönlein purpura
• Location on palms and soles Rickettsial
infection
• Palpable purpura Vasculitis
Laboratory Evaluation
• A thorough history and a careful physical
examination are critical first steps in the
evaluation of children with purpura.
RED FLAGS
Fever,
lethargy,
weight loss,
bone pain,
joint pain,
pallor,
Lymphadenopathy
hepatosplenomegaly
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.
Thank you
any question ;)