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Background: IgA vasculitis and hypersensitivity reactions following exposure to non-steroidal anti-inflammatory drugs (NSAIDs) are very rarely associated with purpura fulminans (PF). The latter is a coagulation event characterised by decreased levels of protein C and a rapidly progressive purpuric rash, often leading to ischaemia, amputations and death.

Case Summary: A previously healthy 66-year-old man presented with a vasculitic rash and abdominal pain following exposure to naproxen (NSAID), which quickly deteriorated to purpura fulminans-like eruption and skin necrosis, mainly involving the face and hands.

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Catastrophic Antiphospholid Syndrome - An Unusual Case Report.

Indian J Crit Care Med

June 2019

Critical Care Medicine, Lilavati Hospital and Research Centre, Mumbai, Maharashtra, India.

Antiphospholipid syndrome (APLS) is characterised by venous or arterial thrombosis and/or adverse pregnancy outcome in the presence of persistent laboratory evidence of antiphospholipid antibodies. Catastrophic Antiphospholipid Syndrome (CAPS) is a severe and rare form of antiphospholipid syndrome characterised by multiple site thrombosis involving small, medium and large blood vessels occurring over a short period of time (usually 1 week) causing multiorgan failure. We present an unusual case of left upper limb acute arterial thrombosis with purpura fulminans like skin lesions precipitated by swine flu (H1N1) infection with adult respiratory distress syndrome subsequently developing acute renal failure, retinal infarcts, multiple acute cerebral infarcts, cardiac valvular vegetations and hemolytic anemia with recurrent bleeding episodes.

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Homozygous protein C deficiency affects approximately 1/400,000 to 1/1,000,000 live births. Homozygous protein C deficiency is associated with catastrophic and fatal purpura fulminans-like or thrombotic complications and disseminated intravascular coagulation. In the present patient, genetic study revealed Arg178Trp, a mutation found widely in European population; but this is the first case of homozygous Arg178Trp mutation who suffered from catastrophic purpura fulminans phenotype.

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Homozygous protein C deficiency or homozygous protein S deficiency are rare genetic diseases with catastrophic and fatal purpura fulminans-like or thrombotic complications occurring during the neonatal period. These diseases can now be successfully treated. Purpura fulminans is at least in part a cutaneous manifestation of the syndrome of systemic DIC.

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