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 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.

Causes Pupura Platelet Vascular Count Function 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 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.

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