Approach to child with purpura

Ahmed S.BarefahKAAU,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 Vascular

Function Count 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 – Thrombocytopenia absent 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 Kasabach-Merritt 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 Henoch-

Schönlein 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 ;)