Allergic purpura

Hematology

Clinicals - History

Fact Explanation
Anorexia Anorexia is a presenting complaint which occurs during the prodromal period. Anorexia
Anorexia is a presenting complaint which occurs during the prodromal period.
Fever Some affected patients can be febrile. Low grade fever is common. Fever
Some affected patients can be febrile. Low grade fever is common.
Rash After the prodrome, patients develop skin rash, which is predominantly distributed over the legs. Rash
After the prodrome, patients develop skin rash, which is predominantly distributed over the legs.
Abdominal pain Involvement of the small vessels of the intestines can produce intestinal ischemia and abdominal pain. Around 85% of the patients develop gastrointestinal symptoms. Patients develop colicky abdominal pain with intussusception. Pain over the right lower quadrant is indicative of acute appendicitis, which is a recognized complication of the disease. Abdominal pain
Involvement of the small vessels of the intestines can produce intestinal ischemia and abdominal pain. Around 85% of the patients develop gastrointestinal symptoms. Patients develop colicky abdominal pain with intussusception. Pain over the right lower quadrant is indicative of acute appendicitis, which is a recognized complication of the disease.
Vomiting 30% of the patients present with vomiting. Vomiting
30% of the patients present with vomiting.
Joint pain Polyarthralgia is a common presentation of AP. Knees, elbows and ankles are commonly involved. The triad of symptoms suggestive of AP includes abdominal pain, palpable purpura and arthritis. Joint pain
Polyarthralgia is a common presentation of AP. Knees, elbows and ankles are commonly involved. The triad of symptoms suggestive of AP includes abdominal pain, palpable purpura and arthritis.
Scrotal edema Scrotal swelling and orchitis develop in about 35% of boys with AP. Scrotal edema
Scrotal swelling and orchitis develop in about 35% of boys with AP.
Bleeding per-rectum Bleeding per-rectum is a complication of gastrointestinal tract involvement. Some children may rarely develop hematochezia (passage of fresh blood per-rectum). Bleeding per-rectum
Bleeding per-rectum is a complication of gastrointestinal tract involvement. Some children may rarely develop hematochezia (passage of fresh blood per-rectum).
Hematemesis Hematemesis is another gastrointestinal complication of AP. Rarely some may develop massive upper gastrointestinal haemorrhage. Hematemesis
Hematemesis is another gastrointestinal complication of AP. Rarely some may develop massive upper gastrointestinal haemorrhage.
Hematuria Hematuria is another presenting complain of AP. Hematuria
Hematuria is another presenting complain of AP.
Recent exposure to an allergen Although the exact allergen is not known, AP can develop after being exposed to some foods, infections, drugs, vaccinations (meningitis C), and insect bites. Infections that may lead to the development of AP include group A streptococci, mycoplasma, Epstein-Barr, and Varicella virus. Recent exposure to an allergen
Although the exact allergen is not known, AP can develop after being exposed to some foods, infections, drugs, vaccinations (meningitis C), and insect bites. Infections that may lead to the development of AP include group A streptococci, mycoplasma, Epstein-Barr, and Varicella virus.
Chest pain Patients with AP may rarely develop myocardial infarction secondary to involvement of coronary arteries. Children may complain of acute left sided chest pain, which radiates to the left arm and jaw. Chest pain
Patients with AP may rarely develop myocardial infarction secondary to involvement of coronary arteries. Children may complain of acute left sided chest pain, which radiates to the left arm and jaw.
Neurological presentations Headache and behavioral changes are the common presenting complains that are due to vasculitis of the central nervous system. Rarely children can develop seizures, hemiplegia due to intracranial hemorrhage and encephalopathy. Neurological presentations
Headache and behavioral changes are the common presenting complains that are due to vasculitis of the central nervous system. Rarely children can develop seizures, hemiplegia due to intracranial hemorrhage and encephalopathy.

Clinicals - Examination

Fact Explanation
Fever Low grade fever can be detected in some patients. Fever
Low grade fever can be detected in some patients.
Rash Palpable purpura is commonly seen over the legs, buttocks and the extensor surfaces of the body. Characteristically the rash is distributed symmetrically. Rash
Palpable purpura is commonly seen over the legs, buttocks and the extensor surfaces of the body. Characteristically the rash is distributed symmetrically.
Scrotal edema Some affected boys can develop orchitis and scrotal swelling. Scrotal edema
Some affected boys can develop orchitis and scrotal swelling.
Examination of the joints The affected joints are swollen and tender. Knees, elbows and ankles are the commonest joints that are involved. Examination of the joints
The affected joints are swollen and tender. Knees, elbows and ankles are the commonest joints that are involved.
Blood pressure Measurement of blood pressure is mandatory in children with AP as elevated blood pressure indicates renal involvement, which is the most common and serious complication of AP. Blood pressure can be reduced in children with massive gastrointestinal tract hemorrhage and nephrotic syndrome. Blood pressure
Measurement of blood pressure is mandatory in children with AP as elevated blood pressure indicates renal involvement, which is the most common and serious complication of AP. Blood pressure can be reduced in children with massive gastrointestinal tract hemorrhage and nephrotic syndrome.
Hepatosplenomegaly Hepatosplenomegaly can be detected in very few children with AP. Hepatosplenomegaly
Hepatosplenomegaly can be detected in very few children with AP.

Investigations - Diagnosis

Fact Explanation
Full blood count Presence of purpura in the absence of thrombocytopenia is the hallmark of diagnosing AP. Some children develop leukocytosis. Full blood count
Presence of purpura in the absence of thrombocytopenia is the hallmark of diagnosing AP. Some children develop leukocytosis.
Urine full report UFR can be normal during the early disease. Hematuria is common and some patients can develop proteinuria. Both indicate the presence of renal involvement. Urine full report
UFR can be normal during the early disease. Hematuria is common and some patients can develop proteinuria. Both indicate the presence of renal involvement.
Erythrocyte sedimentation rate (ESR) ESR can be mildly elevated. Erythrocyte sedimentation rate (ESR)
ESR can be mildly elevated.
Stool occult blood test Stool occult blood can be detected in the presence of occult gastrointestinal bleeding. Stool occult blood test
Stool occult blood can be detected in the presence of occult gastrointestinal bleeding.
Plasma D-dimer Hyperfibrinolysis can be seen in patients with AP especially in the presence of nephritis. Fibrinolysis will further damage the kidneys and worsen nephritis. Plasma D-dimer
Hyperfibrinolysis can be seen in patients with AP especially in the presence of nephritis. Fibrinolysis will further damage the kidneys and worsen nephritis.
Antistreptolysin O (ASO) If post streptococcal AP is suspected ASO can be assessed. Antistreptolysin O (ASO)
If post streptococcal AP is suspected ASO can be assessed.
Complement levels C3 and C4 are decreased in some affected children. This leads to abnormal activation of B lymphocytes and excessive secretion of IgA. Complement levels
C3 and C4 are decreased in some affected children. This leads to abnormal activation of B lymphocytes and excessive secretion of IgA.
Immunoglobulin Both IgG and IgA are elevated. Immunoglobulin
Both IgG and IgA are elevated.
Biopsy According to the diagnostic guidelines of American College of Rheumatology, presence of granulocytes in the walls of small arterioles or venules, palpable purpura and gastrointestinal complains is diagnostic of AP. Biopsy
According to the diagnostic guidelines of American College of Rheumatology, presence of granulocytes in the walls of small arterioles or venules, palpable purpura and gastrointestinal complains is diagnostic of AP.
Renal biopsy Although not routinely indicated renal biopsy is indicated in children with AP and nephritis. Renal biopsy
Although not routinely indicated renal biopsy is indicated in children with AP and nephritis.
Video capsule endoscopy Video capsule endoscopy is helpful in evaluating gastrointestinal tract lesions. Video capsule endoscopy
Video capsule endoscopy is helpful in evaluating gastrointestinal tract lesions.
MRI MRI of the head is highly sensitive in detecting cerebral vasculitis. MRI
MRI of the head is highly sensitive in detecting cerebral vasculitis.

Investigations - Management

Fact Explanation
Renal function test 20- 60% of affected children may develop nephritis due to deposition of IgA. These children have higher mortality and morbidity. Altered serum sodium and potassium levels and elevated serum creatinine enable early detection of nephritis.
Nephritis may progress to nephropathy in very few children. 50% of patients with mixed nephritic- nephrotic syndrome may develop chronic renal failure.
Renal function test
20- 60% of affected children may develop nephritis due to deposition of IgA. These children have higher mortality and morbidity. Altered serum sodium and potassium levels and elevated serum creatinine enable early detection of nephritis.
Nephritis may progress to nephropathy in very few children. 50% of patients with mixed nephritic- nephrotic syndrome may develop chronic renal failure.
Urine full report (UFR) UFR is indicated in patient followup up to about six months for the detection of nephritis. Urine full report (UFR)
UFR is indicated in patient followup up to about six months for the detection of nephritis.
Biomarkers Urinary levels of cystatin C (Cys C) and neutrophil gelatinase-associated lipocalin (NGAL) are elevated in children with HSP. These are useful in early detection of chronic renal involvement as serum creatinine is elevated relatively late in the disease process. Biomarkers
Urinary levels of cystatin C (Cys C) and neutrophil gelatinase-associated lipocalin (NGAL) are elevated in children with HSP. These are useful in early detection of chronic renal involvement as serum creatinine is elevated relatively late in the disease process.
Full blood count If the child is awaiting surgery for the treatment of gastrointestinal complications of AP assessment and correction of hemoglobin is indicated prior to surgery. Full blood count
If the child is awaiting surgery for the treatment of gastrointestinal complications of AP assessment and correction of hemoglobin is indicated prior to surgery.
Renal function test Similarly fitness for general anesthesia is assessed with renal function test, which includes serum electrolytes and serum creatinine. Renal function test
Similarly fitness for general anesthesia is assessed with renal function test, which includes serum electrolytes and serum creatinine.
Renal biopsy Renal biopsy is indicated to stage the renal involvement in children with AP. Renal biopsy
Renal biopsy is indicated to stage the renal involvement in children with AP.

Management - Supportive

Fact Explanation
Hydration Patients with gastrointestinal tract bleeding may require initial assessment of airway, breathing and circulation. Fluid replacement should be started with intravenous crystalloids followed by colloids and cross-matched blood if needed. Hydration
Patients with gastrointestinal tract bleeding may require initial assessment of airway, breathing and circulation. Fluid replacement should be started with intravenous crystalloids followed by colloids and cross-matched blood if needed.
Analgesics Simple analgesics or non-steroidal anti-inflammatory drugs are indicated for the treatment of arthritis. Analgesics
Simple analgesics or non-steroidal anti-inflammatory drugs are indicated for the treatment of arthritis.

Management - Specific

Fact Explanation
Conservative management AP is often self-limiting and do not usually require specific treatment. Conservative management
AP is often self-limiting and do not usually require specific treatment.
Corticosteroids Corticosteroids are indicated for children with severe renal and central nervous system involvement, such as rapidly progressive glomerulonephritis (RPGN). Corticosteroids
Corticosteroids are indicated for children with severe renal and central nervous system involvement, such as rapidly progressive glomerulonephritis (RPGN).
Immunosuppressors Immunesuppressors such as cyclophosphamide, cyclosporine, mycophenolate mofetil and azathioprine are used in the treatment of severe nephritis. Immunosuppressors
Immunesuppressors such as cyclophosphamide, cyclosporine, mycophenolate mofetil and azathioprine are used in the treatment of severe nephritis.
IV immunoglobulin G (IVIg) High-dose IVIg is indicated for the treatment of severe renal involvement. IV immunoglobulin G (IVIg)
High-dose IVIg is indicated for the treatment of severe renal involvement.
Plasmapheresis Plasmapheresis is indicated for children with severe nephritis and central nervous system involvement. Plasmapheresis
Plasmapheresis is indicated for children with severe nephritis and central nervous system involvement.
Surgery Children who develop intussusception, intestinal perforation, bowel necrosis, and massive gastrointestinal bleeding require surgical intervention. Surgery
Children who develop intussusception, intestinal perforation, bowel necrosis, and massive gastrointestinal bleeding require surgical intervention.
Kidney transplantation In end-stage renal failure kidney transplantation is indicated. Kidney transplantation
In end-stage renal failure kidney transplantation is indicated.

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