From the Research
Introduction to Henoch-Schönlein Purpura (HSP)
Henoch-Schönlein Purpura (HSP) is a systemic IgA vasculitis that affects small vessels, commonly presenting in children and rarely in adults 1. The condition is characterized by its impact on various body systems, including the skin, joints, gastrointestinal tract, and kidneys.
Major Manifestations of HSP
The major manifestations of HSP include:
- Cutaneous purpura, which is non-thrombocytopenic and palpable, typically found on the lower extremities and buttocks 2, 1
- Arthritis, presenting as joint pain and swelling
- Gastrointestinal symptoms, such as abdominal pain and gastrointestinal bleeding
- Renal involvement, which may range from mild hematuria to severe nephritis 1, 3, 4
Less Common Manifestations
Less common manifestations of HSP include:
- Urological involvement, affecting the kidney, ureter, bladder, prostate, scrotum, testicle, and penis 3
- Pulmonary, cardiac, genital, and neurological symptoms, although these are rare 1
Diagnosis and Treatment
Diagnosis of HSP is based on clinical presentation and histological examination of skin biopsies, which show signs of small vessel leukocytoclastic vasculitis and IgA deposits in vessel walls 2. Treatment options vary depending on the severity of the disease:
- Supportive care, including nonsteroidal anti-inflammatory drugs for pain relief
- Systemic corticosteroids, which may be beneficial in reducing the extent of lesions and minimizing sequelae of disease 2, 1
- Immunosuppressive drugs, such as methylprednisolone, for severe cases of HSP nephritis 3, 4
- Plasmapheresis and peritoneal and hemodialysis may be required in severe cases 3
Caveats and Considerations
It is essential to note that the prognosis of HSP depends on the presence and severity of renal involvement 1, 4. Early diagnosis and treatment can prevent severe complications, and patients with HSP nephritis should be followed for longer periods with regular measurement of renal function and proteinuria 4.
Treatment Dosages and Durations
Treatment dosages and durations may vary depending on the severity of the disease and the patient's response to treatment. For example, systemic corticosteroids may be tapered over several weeks, as seen in the case of a patient treated with systemic corticosteroids tapered over 5 weeks 2.
Differential Diagnoses
Differential diagnoses for HSP include other vasculitides and conditions that present with similar symptoms, such as palpable purpura, abdominal pain, and renal involvement. A thorough clinical evaluation and histological examination are necessary to confirm the diagnosis of HSP.