Henoch-Schönlein purpura with intracerebral haemorrhage in an adult patient: a case report

Henoch-Schönlein purpura with intracerebral haemorrhage in an adult patient: a case report

Published: Journal of Medical Case Reports 2008, 2:200
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Abstract
Introduction:
Henoch-Schönlein purpura is a small vessel vasculitis that affects mainly the skin, joints, gastrointestinal tract and kidneys. The central nervous system is also occasionally affected, although the majority of patients experience only mild symptoms such as headaches and behavioural changes. Intracerebral haemorrhage is a rare complication of Henoch-Schönlein purpura that so far has mainly been described in children and young adolescence.

Case presentation:
We describe a 42-year-old man with Henoch-Schönlein purpura who developed an acute intracerebral haemorrhage that coincided with a reactivation of his vasculitis and the development of renal failure following discontinuation of steroids. In this patient, both the Henoch-Schönlein purpura and his neurological symptoms were successfully treated with intravenous cyclophosphamide and methylprednisolone, followed by a short course of oral cyclophosphamide and long-term oral prednisolone. His renal function also recovered sufficiently not to require renal replacement therapy.

Conclusion:
The management of Henoch-Schönlein nephritis remains unclear, especially in the presence of severe complications such as intracerebral haemorrhage. We describe a successful outcome in such a patient.

Introduction
Henoch-Schönlein purpura (HSP) is a small vessel vasculitis characterized by IgA1 deposition in the renal mesangium and in the blood vessels. It is seen most frequently in early childhood, although it can occur at any age [1,2]. It is usually preceded by upper respiratory tract infections, having a peak incidence in the autumn and winter [1,2]. In most cases it is a self-limiting disorder and tends to resolve within 1 month of presentation, although it can re-occur in a third of cases [1,2].

HSP affects mainly the skin, joints, gastrointestinal tract and kidneys [1-3]. The severity of symptoms is usually worse in older patients, who tend to have more frequent skin, joint and renal involvement [2]. The central nervous system is also occasionally affected, although the majority of patients experience only mild symptoms such as headaches and behavioural changes [3]. More serious neurological complications are rare and include seizures, cranial or peripheral neuropathies, intracerebral haemorrhage and encephalopathy [1,3]. We describe a man with HSP who developed an acute intracerebral haemorrhage that coincided with a reactivation of his vasculitis.

Keywords:
intracerebral subarachnoid haemorrhage, intracerebral hemoorhage stroke, acute intracerebral hemorrhage, intracerebral brain haemorrhage, intracerebral hemorrhage surgery, Henoch-Schönlein purpura diagnosis, Henoch-Schönlein purpura syndrome, Henoch-Schönlein purpura vasculitis, Henoch-Schönlein purpura rash,

References:
  1. Saulsbury FT: Clinical update: Henoch-Schönlein purpura. Lancet 2007, 369:976-978.
  2. Kellerman PS: Henoch-Schönlein purpura in adults. Am J Kidney Dis 2006, 48:1009-1016.
  3. Sevcan AB, Mesiha E, Necmiye T, Gûlhis D, Erden İlhan, Tansel E: Cerebral vasculitis in Henoch-Schönlein purpura. Nephrol Dial Transplant 2000, 15:246-248.
  4. Imai T, Okada H, Nanba M, Kawada K, Kusaka T, Itoh S: Henoch- Schönlein purpura with intracerebral hemorrhage. Brain Dev 2002, 24:115-117.
  5. Clark JH, Fitzgerald JF: Hemorrhagic complications of Henoch- Schönlein syndrome. J Pediatr Gastroenterol Nutr 1985, 4:311-315.
  6. Chiaretti A, Caresta E, Piastra M, Pulitano S, Di Rocco C: Cerebral hemorrhage in Henoch-Schönlein syndrome. Childs Nerv Syst 2002, 18:365-367.
  7. Altinors N, Cepoglu C: Surgically treated intracerebral hematoma in a child with Henoch-Schönlein purpura. J Neurosurg Sci 1991, 35:47-49.
  8. Ng CC, Huang SC, Huang LT: Henoch-Schönlein purpura with intracerebral hemorrhage: case report. Pediatr Radiol 1996, 26:276-277.
  9. Wen YK, Yang Y, Chang CC: Cerebral vasculitis and intracerebral hemorrhage in Henoch-Schönlein purpura treated with plasmapheresis. Pediatr Nephrol 2005, 20:223-225.
  10. Lévaif F, Szücs LH, Jászai Z: Cerebral hemorrhage and acute glomerulonephritis in Schonlein-Henoch syndrome in old age. Z Gesamte Inn Med 1971, 26:309-311.
  11. Sang-Wuk J, Keun-Hwa J, Kon C, Hee-Joon B, Seung-Hoon L, Jae-Kyu R: Clinical and radiologic differences between primary intracerebral hemorrhage with and without microbleeds on gradient-echo magnetic resonance images. Arch Neurol 2004, 61:905-909.
  12. Niaudet P, Habib R: Methylprednisolone pulse therapy in the treatment of severe forms of Schonlein-Henoch purpura nephritis. Pediatr Nephrol 1998, 12:238-243.

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