February 2019 | Case of the Month

History

A 42 year old man was referred with a 6 week history of an asymptomatic rash affecting his lower abdomen, buttocks, lower legs and forearms. This was associated with some swelling and aching of his ankles and to a lesser extent his knees. There had been no preceding infection or other trigger. The patient also complained of occasional bouts of abdominal pain.

The patient had two previous episodes, the first in his late teens and the second one 10 years earlier, the latter went on for almost 15 months. In between episodes his skin was normal.

The patient was otherwise well and took no medication. He had an office based job and drinks a moderate amount of alcohol. There are no relevant hobbies.


Examination

OE there was a non-blanching red-purple macular rash.


Investigations

What is the differential diagnosis?


Images

Diagnosis

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The diagnosis was consistent with Henoch-Schonlein purpura (HSP). During all three occasions there had been no renal or other systemic involvement, although there was occasional abdominal pain.

HSP 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. 

Refer to the chapter on vasculitis for more information.

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