What is purpura?

Purpura is a medical condition characterised by the appearance of purple or red spots on the skin, which are caused by bleeding underneath the skin.¹ While purpura can occur in people of all ages, it is particularly concerning in children due to its potential association with underlying medical conditions.

What are the causes of purpura in children?

Purpura in children can have various causes, including:

Immune Thrombocytopenic Purpura (ITP): ITP is a condition in which the immune system mistakenly attacks and destroys platelets, leading to a decrease in platelet count and an increased risk of bleeding. According to a study published in the Journal of Paediatrics, the estimated annual incidence of ITP in children is approximately 5.3 per 100,000 children under the age of 15.²

Henoch-Schönlein Purpura (HSP): HSP is a type of vasculitis that affects small blood vessels, leading to inflammation and bleeding in the skin, joints, and internal organs. It has a higher male predominance and affects children usually around the age of 6-8 years.³ It is one of the most common types of vasculitis seen in children.⁴

Thrombotic Thrombocytopenic Purpura (TTP): TTP is a rare blood disorder characterized by the formation of small blood clots throughout the body, leading to a decrease in platelet count and organ damage
Infections: certain viral and bacterial infections, such as meningococcaemia or Rocky Mountain Spotted Fever, can cause purpura as a result of blood vessel inflammation and damage.

Medication reactions: some medications, particularly antibiotics and nonsteroidal anti-inflammatory drugs (NSAIDs), can cause an allergic reaction or drug-induced thrombocytopenia, leading to purpura.
Underlying medical conditions: purpura may also occur as a symptom of underlying medical conditions such as leukaemia, von Willebrand disease, or systemic lupus erythematosus (SLE).

What are the symptoms of purpura?

Skin lesions: the hallmark symptom of purpura is the appearance of purple or red spots on the skin, known as petechiae or ecchymoses.⁵ These spots may vary in size and distribution and are caused by bleeding underneath the skin.

Easy bruising: children with purpura may experience easy bruising, where even minor trauma can lead to the development of bruises or ecchymoses on the skin.

Bleeding from mucous membranes: in addition to skin lesions, purpura may also cause bleeding from other parts of the body, such as the nose, mouth, or gums. This bleeding may manifest as nosebleeds (epistaxis), bleeding gums, or blood in the saliva.

Blood in urine or stool: in severe cases of purpura, children may develop haematuria (blood in the urine) or melaena (black, tarry stools) due to bleeding in the urinary tract or gastrointestinal tract respectively.

Joint pain or swelling: some children with purpura, particularly those with conditions like HSP, may experience joint pain or swelling. Joint symptoms may include arthritis-like pain, stiffness, or swelling, typically affecting the knees, ankles, or elbows.

Abdominal pain: children with purpura, especially those with systemic vasculitis like HSP or Kawasaki disease, may experience abdominal pain or discomfort. Abdominal symptoms may include cramping, tenderness or bloating.

Fatigue or weakness: chronic or severe purpura can lead to fatigue or weakness in affected children, possibly due to anemia resulting from chronic blood loss or underlying systemic inflammation.

How is purpura in children diagnosed?

Medical history: the healthcare provider will begin by obtaining a detailed medical history, including information about the child's symptoms, recent illnesses, medications, vaccinations, and any relevant family history of bleeding disorders or autoimmune conditions.

Physical examination: a comprehensive physical examination is crucial in evaluating purpura. The healthcare provider will carefully examine the child's skin for the presence of purpuric lesions, including petechiae (small, pinpoint-sized spots) or ecchymoses (larger, bruise-like patches). The distribution, size, and colour of the lesions may provide important clues about the underlying cause of the purpura.

Laboratory Tests:

• Full Blood Count (FBC): FBC is a routine blood test that measures the number of red blood cells, white blood cells, and platelets in the blood. In children with purpura, the FBC may reveal thrombocytopenia (low platelet count), which can indicate conditions such as ITP or TTP
• Coagulation studies: coagulation tests, such as prothrombin time (PT), activated partial thromboplastin time (aPTT), and fibrinogen levels, evaluate the child's blood clotting function. Abnormalities in these tests may suggest underlying coagulopathies or clotting disorders
• Platelet function tests: Platelet function tests assess the child's platelet function and ability to form blood clots. These tests may include platelet aggregation studies or bleeding time tests, which measure the time it takes for a small puncture wound to stop bleeding. Abnormal results may indicate platelet dysfunction or qualitative platelet disorders
• Serological tests: Serological tests may be performed to screen for underlying infections or autoimmune conditions associated with purpura. These tests may include tests for viral or bacterial pathogens (e.g., hepatitis B and C, HIV) or autoimmune markers (e.g., antinuclear antibodies, rheumatoid factor)
• Bone marrow aspiration: in some cases, a bone marrow aspiration may be indicated to evaluate for underlying bone marrow disorders or leukaemia, especially if the CBC or other tests suggest abnormalities in blood cell production

Imaging: in certain cases, imaging such as ultrasound, computed tomography (CT), or magnetic resonance imaging (MRI) may be ordered to assess for underlying causes of purpura, such as vascular abnormalities, organ enlargement, or intra-abdominal bleeding.
Skin biopsy: in cases where the diagnosis remains uncertain or when skin lesions are atypical, a skin biopsy may be performed to obtain a tissue sample for microscopic examination. A skin biopsy can help differentiate between various types of purpura and rule out other skin conditions.
Specialised testing: depending on the suspected underlying cause of purpura, additional specialised tests may be ordered. For example, testing for specific autoantibodies (e.g., antiplatelet antibodies in ITP) or genetic testing (e.g., for inherited coagulation disorders) may be warranted in certain cases.

How is purpura treated?

Observation and supportive care: in mild cases of purpura without significant bleeding or other complications, observation and supportive care may be sufficient. This may include rest, adequate hydration, and close monitoring of the child's symptoms.

Addressing underlying causes: if purpura is secondary to an underlying medical condition or trigger, such as an infection or medication, addressing the underlying cause is essential. This may involve treating the infection with appropriate antibiotics, discontinuing offending medications, or managing underlying autoimmune or systemic disorders.

Medications:

• Corticosteroids: corticosteroids, such as prednisolone, may be prescribed to suppress inflammation and immune-mediated processes in conditions like ITP or HSP. Corticosteroids can help increase platelet counts and reduce the severity of symptoms
• Intravenous Immunoglobulin (IVIG): IVIG therapy may be used in cases of severe or refractory purpura, particularly in ITP or HSP. IVIG contains antibodies that can help modulate the immune response and increase platelet counts
• Platelet transfusions: platelet transfusions may be necessary in cases of severe thrombocytopenia or active bleeding to increase platelet counts and prevent further bleeding. However, platelet transfusions are typically reserved for life-threatening situations due to the risk of complications and the transient nature of their effectiveness

Plasmapheresis: in rare cases of severe TTP or other thrombotic microangiopathies, plasmapheresis may be performed to remove circulating antibodies and other factors contributing to abnormal blood clotting. Plasmapheresis is often used in conjunction with other treatments, such as corticosteroids or immunosuppressive agents.

Pain management: children with purpura-associated joint pain or abdominal discomfort may benefit from pain management strategies, such as nonsteroidal anti-inflammatory drugs (NSAIDs) or analgesics. It's important to use pain medications cautiously and under medical supervision, especially in children with underlying bleeding disorders or renal impairment.

Close monitoring and follow-up: children with purpura may require close monitoring of their platelet counts, symptoms, and overall clinical status. Regular follow-up appointments with healthcare providers are essential to assess treatment response, monitor for potential complications, and adjust treatment plans as needed.

Preventive measures: in cases of recurrent or chronic purpura, preventive measures may be recommended to minimize the risk of future flare-ups or complications. This may include lifestyle modifications, such as avoiding certain medications or environmental triggers, as well as long-term management strategies tailored to the child's specific condition and needs.

Summary

Purpura in children can be a worrisome symptom that requires careful evaluation and management. Understanding the underlying causes, recognising associated symptoms, and implementing appropriate treatment strategies are essential for optimising outcomes and ensuring the well-being of affected children. Parents and caregivers should seek medical attention promptly if their child develops unexplained purpura or other concerning symptoms.

By providing a detailed yet simple overview of purpura in children, including its causes, symptoms, diagnosis, and treatment options, healthcare professionals can better educate parents and caregivers about this condition and guide them in making informed decisions about their child's care and management. Early diagnosis and intervention are key to preventing complications and promoting the best possible outcomes for children with purpura.