August 2019 | Case of the Month

History

A 37 year old man presented with a rash that started on his legs and buttocks and then spread to his arms, there was limited involvement of the trunk. The rash felt a little uncomfortable and there was additional arthralgia and mild abdominal pain. The patient had two previous episodes when younger that responded to systemic steroids, completely resolving over several months.

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Diagnosis

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The diagnosis is Henoch–Schönlein purpura (HSP) with 50% of cases having renal involvement. This patient had no renal problems.

HPS is defined as a small vessel vasculitis involving deposition of IgA immune complexes, that characteristically involves the skin, gastrointestinal system and glomeruli with or without arthritis or arthralgia
90% of cases arise under the age of 10. Occasionally older children and adults are affected
In the majority no trigger is found, although Group A beta-haemolytic streptococci is found in approximately 30% of cases
Clinical features
- Patients often present with a rash, abdominal pain and arthralgia
- Cutaneous lesions are classically symmetrical affecting extensor surfaces, particularly the elbow, knees and buttocks. The trunk and face can also be affected
- The lesions are erythematous patches within which develop areas of haemorrhage and palpable purpura. Necrotic ulcers are sometimes seen
- Crops of new lesions arise periodically until the cutaneous features subside, which is usually around 2-3 months
Systemic features
- 75% of cases have joint involvement
- 65% of cases have gastrointestinal symptoms with abdominal pain and/or GI bleeding
- 50% of cases have renal involvement, although less than 3 % progress to end-stage renal failure
Histopathology shows a leucocytoclastic vasculitis
HSP is usually self-limiting, the main exception being those patients with moderate-severe renal involvement

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