Henoch-Schonlein purpura (HSP)/Anaphylactoid purpura:
• disease of childhood (could also occur in adults) peakage of onset around 5 years– consists of vasculitis limited to skin, gastrointestinal tract and kidneys that usually follows an upper respiratory tract infection
• IgA immune complexes deposit in the tissues (may be a hypersensitivity reaction to an infection). Infection and and exposure to drugs are considered common triggers.
• abrupt onset – flu like symptoms. Within days palpable purpura over tibia, ankles and feet; abdominal pain and GI bleeding.
• 75% develop joint symptoms – mostly knees and ankles – occasionally small joints of hands and feet – usually bilateral and symmetrical – usually only lasts up to a week (no permanent sequelae)
• American College of Rheumatology criteria for diagnosis is when 2 of the 4 criteria are present (palpable purpura, <20 years of age; bowel angina; wall granulocytes on biopsy)
• PAPAH – Purpura, Abdominal Pain, Arthralgias, Haematouria
• usually self limiting – 80% make full recovery in 6 to 16 weeks; management is mostly supportive
• NSAID’s may help joint pain
Skin lesions of bullous variant with ankle oedema (courtesy Robert Issacs):
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