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Henoch-Schonlein Purpura(HSP) – Clinical features || Pathophysiology || Diagnosis and Treatment

HSP-henoch-schonlein purpura

This occurs when vasculitis occurs in childhood. This disease is characterised by deposition of IgA in the small blood vessels of skin, joint, kidney, and gastrointestinal tract.

1. Clinical Faetures :

  1. Most common feature of HSP is vasculitis.
Vasculitis in ear blood vessels
Vasculitis in ear blood vessels
Vasculitis in leg
Vasculits in Leg

2. Skin lesions – Periorbital oedema, oedema in dorsum of hands and feet, scrotum and sometimes scalp also.

3. Musculoskeletal manifestations – These are arthritis and arthralgia.

4. Gastrointestinal manifestations – Like Abdominal pain, vomiting, diarrhoea, paralytic ileum, Selena, intussusception and mesenteric ischaemia or perforation.

5. Renal system involvement – These are hypertension, hematuria, proteinuria, nephrotic syndrome,frank nephritis,frank nephritis, and ultimately acute/chronic renal failure.

6. CNS System involvement – seizures, headaches, Intracerebral haemorrhage, orchitis, carditis, inflammatory eye disease, testicular torsion and pulmonary haemorrhage.

2. Pathophysiology :

HSP pathogenesis/Pathophysiology

Most commonly caused by Upper respiratory tract infections which includes group-A streptococcus infection. Now IgA acts to this infections and starts to deposit throughout the blood vessels of the body like in Skin, Joints, Kidney and GI tract.

3. Diagnosis :

A. Clinical diagnosis :

The clinical diagnosis mainly based on oedema, and palpable purpura. The patient may present with :

  1. Fever
  2. Palpable purpura
  3. Edema of face, hands, feet, scrotum.

AHE that is Acute hemorrhagic oedema is an isolated leukocytoclastic vasculitis which affects infants <2 years of age. AHE mainly express oedema of face, hands and feet, and scrotum.

B. Lab diagnosis :

  1. Increased ESR
  2. Increased CRP
  3. Mild anaemia
  4. Leukocytosis
  5. Thrombocytosis
  6. Occult blood is frequently found in the stool specimen

4. Treatment :

As the disease is due to activity of the immune system, so the drug of choice is corticosteroids which will suppress the Immune response.

  1. Prednisolone – 1mg/kg/day for 1 to 2 weeks followed by tapper.
  2. Incase of chronic HSP with renal disease – We can use variety of immunosuppressants like Azathioprine, Cyclophosphamide, and Mycophenolate mofetil.

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