Henoch-Schonlein Purpura

Aashifa

Physician Assistant, Kauvery Hospital Cantonment, Trichy

Abstract

Henoch-Schonlein purpura (IgA vasculitis) is the most common form of systemic vasculitis in children. It involves the small vessels of the joints, kidneys, gastrointestinal tract, and skin. It includes the acute onset of fever palpable purpura on the lower extremities and buttocks, abdominal pain, arthritis and haematuria. Henoch-Schonlein purpura is named aftertwo German physicians, Dr. Johann Schonlein and his student Eduard Henoch.

We Report a 12-year-old female child presented with purpuric rashes over both forearm, buttocks and legs along with abdominal pain. Patient was diagnosed with HSP and treated with oral prednisolone and other supportive measures.

Case Presentation

A 12-year-old female child brought by her mother with complaints of purpuric rash for past 11 days (dark red in colour). The rashes arise on both upper extremities and later it spreads on buttocks, lower extremities. She had a complaint of right knee joint pain for 2 days and abdominal pain for 1 day before 11 days and it relieved by medication. No History of fever/loose stools/ vomiting/haematuria. She had a past history of same purpuric rash 3 months back and it disappeared spontaneously. A week before, She was previously treated outside with calamine lotion and clobetasol cream. Previous hospital investigation suggestive of Antistreptolysin O(ASO) titre was negative.

Antenatal and Birth History:

Term baby, normal vaginal birth, cried immediately after birth, One day Nicu admission required.

Development history:

Attained developmental mile stones at appropriate age.

Immunization History:

Immunized till date.

On examination, general condition of the patient was fair and vitals were stable. There was presence of non- tender, non- blanching purpuric rash over the medial aspect of both upper and lower extremities along with buttocks. Abdomen was soft and non-tender.

Relevant investigations were done. Hb – 14.3g/dl, WBC- 1 1,600, PLT – 390000, CRP-0.610, Urea-19.3, Creatinine-0.5. Urine routine was normal.USG Abdomen showed Mild Hepatomegaly.

Diagnosis of HSP was made as per the European League against Rheumatism (EuLaR) or Paediatric Rheumatology European Society ( PRES) Creteria

Criterion Description
Mandatory criterion Palpable purpura especially in lower extremities
Additional criteria Diffuse abdominal pain
Any biopsy with predominant IgA deposits
Arthritis or arthralgia
Renal involvement in the frm of haematuria or proteinuria

Child was initially treated with oral prednisolone for 8 days. On 2nd post-admission day, she developed abdominal pain and the rashes were increased. Her vitals were stable and continued the same medications. On 4th post-admission day, purpuric rashes spread on the right forearm. The child responded to above mentioned treatment child also received other supportive care in the form of PPI, anti-histamines and fluids.

Our case did not have any renal involvement manifesting in the form of haematuria and neurological manifestations. Patient was advised follow up in the OPD with serial urine analysis.

Discussion

HSP was first described by William Heberden in 1801. Later, HSP was named after two German physicians, Dr. Johann Schonlein and his student Eduard Henoch. HSP is the most common vasculitis of the children.

Our case had symptoms of rashes over both upper and lower extremities, abdomen pain and right side knee joint pain. It indicates GIT and joint involvement. There were no signs of hematuria and proteinuria. The diagnosis of HSP made by EuLaR or PRES criteria.

The patient is said have HSP if mandatory criteria are present along with atleast one of the additional criteria. Our case fulfilled the mandatory criteria along with diffuse abdominal pain and arthralgia as additional criteria.

Steroids are more often used for the relief of abdominal pain, joint pain and skin disease. Alternatively, dapsone has been quite effective for the treatment of chronic abdominal pain and skin involvement. Prednisolone is the commonly used steroid for the treatment of HSP. The disease has an excellent prognosis except for renal involvement.

Conclusion

HSP is the most common form of systemic vasculitis in children. It includes the acute onset of fever, palpable purpura on the lower extremities and buttocks, abdominal pain, arthritis and hematuria. Treating with steroids will give positive outcome in earlier stage. The syndrome has good prognosis but the renal involvement of the disease has high mortality and morbidity.

Asahifa

Asahifa

Physician Assistant

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