G137(P) Atypical manifestations of henoch-schönlein purpura; a 3 case series

Abstract

Background Henoch-Schonlein Purpura (HSP) is the most common childhood vasculitis. It is often self-limiting and skin, gastro-intestinal (GI) and joint manifestations predominate. We report 3 cases of HSP with atypical manifestations: (1) para-spinal and sub-cutaneous haematomas with GI bleed (2) recurrent HSP with severe epididymo-orchitis and GI bleed (3) recurrent HSP with glomerulonephritis complicated by E. Coli infections. Case 1 A 9-year-old boy presented with typical HSP rash and ankle swelling and subsequently developed a 25 cm long painful right para-spinal swelling with normal overlying skin. Imaging confirmed subcutaneous haematoma. He developed further subcutaneous haematomas in the shoulder and scalp regions and his clinical course was further complicated by GI bleeding. The active vasculitis was treated with oral steroids to good effect. Case 2 A 2-year-old boy presented with uncomplicated HSP with self-limiting skin and ankle manifestations. 2 years later he had a recurrence which progressed to include painful penile and scrotal swelling and purpura. Ultrasound confirmed bilateral epididymitis. He subsequently developed significant GI bleeding that was treated with oral steroids. Due to persistent and worsening disease he required a skin biopsy, extended vasculitis screen and pulse Methylprednisolone resulting in disease remission. Case 3 An 11-year-old girl presented with macroscopic haematuria and abdominal pain 4 weeks after developing a typical HSP rash. Urine dip and microscopy, and renal ultrasound were suggestive of HSP nephritis. Initial renal function was normal, but an inter-current UTI caused worsening nephritis, renal dysfunction and hypertension. Renal biopsy confirmed HSP glomerulonephritis and she was treated with pulse Methylprednisolone. A few weeks later she had a further recurrence of HSP rash and renal dysfunction associated with E Coli sepsis and required pulse cyclophosphamide, long term anti-hypertensives and diuretics. Conclusion Significant sub-cutaneous haematoma, profound scrotal and penile involvement, recurrent HSP and HSP nephritis complicated by recurrent infections are described in these cases, with corresponding management options. These manifestations of HSP are relatively uncommon, but appropriate monitoring, parental counselling and involvement of sub-specialty services are essential for timely management. Long term outcomes are good with appropriate supportive, immuno-modulant and chemotherapeutic interventions.