Day #330 - HSP

Today we heard about a patient with a diagnosis of Henock Schonlein Purpura (Henock left, Schonlein, right). A similar case is presented in the NEJM here.
They presented with a prodromal URTI (more common in patients younger than 30) and then:

• Lower extremity purpuric rash (picture here)
• Abdominal pain with or without gastrointestinal bleeding (GI manefestations reviewed here)
  
• Symetrical arthritis of the large joints in the lower limbs
• Macroscopic hematuria with the development of renal failure and nephrotic syndrome

While mainly a disease of children, approximately 10% of patients with HSP will be diagnosed as adults. In children severe renal disease is rare; however, in up to 30% of adults renal involvement can be severe and up to 10-15% of adults will develop severe renal disease. Treatment in these cases involve steroids with consideration of other immunosuppressants.

Poor prognostic signs include:

• Creatinine more than 120


• Proteinurea more than 1g/day


• Fibrosis or necrosis on kidney biopsy greater then 10%.

The major differential of purpuric skin rash and macroscopic hematuria includes drug-induced hypersensitivity vasculitis, which usually has a good prognosis and cryoglobulenemic vasculitis, which has a much worse prognosis.

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If you speak german, I think this is schonlein's book from 1832 where he first describes this condition.

I've been on a medical history theme this week -- here is an article from 1914 by Osler on HSP.