Purpura: Symptoms, Causes and Treatment

Purpura is a medical term that describes the appearance of purple or red spots on the skin, mucous membranes, or organs due to bleeding underneath the surface. These spots are not only visually striking but can also be a sign of underlying health issues ranging from minor to life-threatening. Understanding the symptoms, causes, and treatment options for purpura is essential for early recognition and effective management. In this article, we provide a comprehensive overview, drawing on current research to clarify what patients and caregivers need to know about this condition.

Symptoms of Purpura

Purpura presents with a diverse range of symptoms that can vary depending on the underlying cause and the specific type of purpura. Recognizing these symptoms early is crucial for prompt diagnosis and treatment. While the hallmark is the appearance of purple or red spots on the skin, there are often associated systemic symptoms that reflect involvement of other organs.

Symptom	Description	Associated Conditions	Source(s)
Skin Rash	Non-blanching purple/red spots or patches	HSP, TTP, Purpura Fulminans, PPP	1 3 4 7 13
Joint Pain	Arthralgia or arthritis, sometimes severe	HSP, TTP	1 3 4 14
Abdominal Pain	May include GI bleeding, melena, or colic	HSP, Purpura Fulminans	1 3 7 14
Renal Involvement	Hematuria, proteinuria, nephritis	HSP, TTP	1 3 4 11 14
Edema	Swelling, often subcutaneous	HSP, TTP	1 3 4
Systemic Symptoms	Fever, fatigue, lethargy, myalgia	HSP, TTP, Purpura Fulminans	3 4 7 12
Neurological Signs	Headache, confusion, difficulty communicating	TTP, Purpura Fulminans	4 7 12
Other	Orchitis, burning/itchy lesions, mood swings	HSP, EIP, TTP	1 3 4 8

Table 1: Key Symptoms

Understanding the Skin Manifestations

The most characteristic feature of purpura is the skin rash—non-blanching, purple or red spots that can range in size from small petechiae to larger ecchymoses. These lesions are often distributed on the lower limbs but may be widespread. In Henoch-Schönlein purpura (HSP), for example, the rash is typically palpable and may be accompanied by swelling or urticarial plaques, especially after physical activity (as seen in exercise-induced purpura) 1 3 8.

Joint and Abdominal Symptoms

Joint involvement is common, presenting as pain (arthralgia) or swelling (arthritis), especially in children with HSP. Sometimes, joint pain even precedes the appearance of the rash 1 3 14. Abdominal pain is another frequent symptom, which can be severe and sometimes signals gastrointestinal bleeding or more serious complications 1 3 14.

Renal and Systemic Involvement

Renal manifestations such as hematuria (blood in urine), proteinuria, and nephritis are significant, particularly in HSP and thrombotic thrombocytopenic purpura (TTP). These symptoms may indicate a more aggressive disease course and require close monitoring 1 3 4 11 14. Systemic features like fever, fatigue, and myalgia can also occur, especially in more severe forms or in the context of systemic involvement 3 4 7 12.

Neurological and Other Symptoms

In conditions like TTP and purpura fulminans, neurological symptoms such as headache, confusion, and difficulty communicating may develop. These indicate more widespread vascular involvement and are clinical red flags 4 7 12. Additional symptoms may include orchitis (testicular inflammation), burning or itchy lesions (especially in exercise-induced purpura), and even mood disturbances in chronic or congenital cases 1 3 4 8.

Causes of Purpura

Purpura can result from a wide variety of underlying conditions that disrupt normal blood vessel integrity or hemostasis. The root cause determines the clinical course, severity, and approach to treatment.

Cause Type	Mechanism/Trigger	Examples/Notes	Source(s)
Vascular Damage	Inflammation, immune complex	HSP, vasculitis, allergic purpura	1 5 14
Platelet Disorders	Decreased number/function	TTP, ITP, drug-induced purpura	4 5 6 9
Coagulation Issues	Clotting factor deficiency/DIC	Purpura fulminans, liver failure	7 9 12
Infections	Bacterial or viral	Meningococcemia, post-strep purpura	1 5 7 12
Drugs & Toxins	Immune-mediated or direct toxicity	Sedormid, quinine, other medications	5
Physical Triggers	Trauma, exercise, autosensitization	Exercise-induced, autoerythrocyte purpura	6 8
Hereditary Factors	Genetic protein deficiencies	Congenital TTP, protein C/S deficiency	4 7 12

Table 2: Major Causes of Purpura

Vascular and Immune-Mediated Causes

Many forms of purpura, such as HSP and vasculitis-associated purpura, are driven by immune-mediated inflammation of small blood vessels. In HSP, deposition of IgA immune complexes in vessel walls leads to inflammation and leakage of blood into surrounding tissues 1 14. Allergic and idiopathic purpura may also result from hypersensitivity reactions, although the exact mechanism is sometimes unclear 5.

Platelet and Coagulation Disorders

Disorders affecting platelets, such as TTP and idiopathic thrombocytopenic purpura (ITP), lead to increased bleeding risk due to insufficient platelet numbers or function 4 5 9. Some drugs can induce immune-mediated destruction of platelets, as seen with sedormid or quinine 5. Coagulation disorders, including disseminated intravascular coagulation (DIC) in purpura fulminans, cause widespread clotting and subsequent bleeding as clotting factors are consumed 7 12.

Infections

Purpura can be triggered by infections, particularly with bacteria like Streptococcus or Neisseria meningitidis. In some cases, purpura appears during the height of infection or as an immune response during recovery 1 5 7 12. Severe infections can precipitate DIC, leading to purpura fulminans, a life-threatening emergency 7 12.

Drug-Induced and Physical Triggers

Certain medications can cause purpura by either directly damaging blood vessels or triggering immune responses that result in vascular or platelet injury 5. Physical triggers include trauma or unusual exertion, as in exercise-induced purpura, and rare syndromes like autoerythrocyte sensitization, where the body reacts against its own red blood cells after injury 6 8.

Hereditary and Genetic Causes

Rarely, purpura may be due to hereditary deficiencies of proteins involved in coagulation, such as congenital TTP or inherited protein C/S deficiency, predisposing to microvascular thrombosis and purpuric lesions 4 7 12.

Treatment of Purpura

Managing purpura involves both addressing the underlying cause and providing supportive care for symptoms. Treatment strategies can range from simple monitoring to advanced immunosuppressive therapies and emergency interventions.

Treatment	Indication/Approach	Notes/Outcomes	Source(s)
Supportive Care	Mild cases, symptom relief	Rest, hydration, pain management	1 3 9 14
Corticosteroids	Immune-mediated, severe joint/abdominal sx	Rapid symptom relief, not always curative	1 3 14
Immunosuppressants	Renal or organ-threatening involvement	Mycophenolate, cyclosporine, rituximab	11 14
Plasma Exchange	TTP, severe purpura fulminans	Mainstay for TTP, improves survival	10 12
Bioflavonoids/Vit C	Pigmented purpura	May improve capillary strength	13
Treat Underlying Cause	Infections, drug reactions, deficiencies	Antibiotics, drug cessation, protein C replacement	7 12
Compression/Topical	Exercise-induced purpura	Compression, topical steroids	8

Table 3: Main Treatment Approaches

Supportive and Symptom-Directed Care

For mild, self-limited forms of purpura, supportive care is often sufficient. This may include rest, hydration, and pain or itch management. Many cases of HSP in children resolve without specific treatment 1 3 9 14.

Corticosteroids and Immunosuppressive Therapy

Corticosteroids can rapidly relieve joint and abdominal pain in immune-mediated purpura like HSP, but evidence for altering long-term organ outcomes is mixed 1 3 14. In severe renal involvement (HSP nephritis), additional immunosuppressive agents such as mycophenolate mofetil, cyclosporine A, or rituximab may be needed 11 14. Treatment is individualized based on disease severity and organ involvement.

Plasma Exchange and Intensive Therapies

In life-threatening conditions such as TTP and purpura fulminans, plasma exchange is the mainstay of therapy and can be lifesaving 10 12. This procedure helps remove antibodies and replenish deficient enzymes or factors.

Targeted and Adjunctive Therapies

In chronic pigmented purpura, bioflavonoids (such as rutoside) and vitamin C have shown promise in increasing capillary resistance and clearing lesions 13. For exercise-induced purpura, preventive measures like compression stockings, venoactive drugs, and topical steroids can help reduce recurrences 8.

Treating Underlying and Contributing Conditions

Identifying and treating the underlying cause is crucial. This might involve antibiotics for infections, discontinuation of causative drugs, or replacement of deficient proteins in hereditary conditions 7 12. In drug-induced purpura, stopping the offending agent is essential 5.

Conclusion

Purpura is a complex clinical sign with a wide spectrum of underlying causes and manifestations. Early recognition, careful assessment of accompanying symptoms, and identification of the root cause are essential for effective management. Here are the main takeaways:

• Purpura manifests as non-blanching purple or red skin lesions and may be accompanied by joint, abdominal, renal, or systemic symptoms 1 3 4 7 14.
• Causes include vascular inflammation (vasculitis), platelet or coagulation disorders, infections, drugs, physical triggers, and hereditary deficiencies 1 4 5 7 12 14.
• Treatment is tailored to the underlying cause and ranges from supportive care to corticosteroids, immunosuppressants, plasma exchange, and targeted therapies 1 3 10 11 13 14.
• Prompt diagnosis and intervention are essential, especially in severe forms like TTP and purpura fulminans, to reduce the risk of complications and improve outcomes 4 7 10 12.
• Patient-centered care and multidisciplinary approaches help address both the physical and emotional impacts of purpura and its underlying causes.

By understanding the diverse presentations and causes of purpura, clinicians and patients alike can work together toward timely diagnosis and effective treatment.