Anti-phospholipid Antibody Syndrome

Erythematous macules on the face.

Clinical Background:

This 24 year old woman developed a catastrophic antiphospholipid syndrome.
The onset was abrupt, with bilateral adrenal infarction and resulting Addison's disease, right kidney infarction, and deep vein thrombosis of the left leg with pulmonary embolism.
Laboratory examinations revealed immunohemolytic anemia and thrombocytopenia (Evans' syndrome).
Moreover, she had high titres of anticardiolipin antibodies of IgG and IgM class and lupus anticoagulant.
At the time of her admission to our Department she had areas of cutaneous necrosis on the hears and erythematous macules on the face.
The pathological examination of a biopsy from a plaque on the face showed microthrombosis in the dermal vessels without vasculitis.