Anti-phospholipid Antibody Syndrome

Generalized Livedo Racemosa

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Clinical Background:

A 35 year old female patient who at the age of 24 reported the appearance of persistent cyanotic erythematous manifestations, initially confined to the legs, that progressively extended to involve the lower extremities and the trunk.
These manifestations were accompanied by small skin ulcerations on the ankle and back of the feet, which appeared more frequently during the summer months.
From the age of 29 she complained of Raynaud's phenomenon and at the age of 34 had an ischaemic episode on the fourth finger of the left hand.
The patient had no previous neurologic manifestations other than some episodes of vertigo.
Clinical examination revealed widespread purple erythematous areas involving the entire cutaneous surface which were consistent with the diagnosis of generalized livedo racemosa.
Irregular stellate scars were present on the ankles as a result of previous ulcerations (livedoid vasculitis) and marked acrocyanosis on the extremities.
Laboratory investigations revealed only the presence of high titers of IgG anti-cardiolipin autoantibodies.


This case does not fulfill the criteria for the diagnosis of antiphospholipid antibody syndrome. However, livedo could be the first clinical manifestation and therefore a longer period of observation is necessary.
Furthermore it has been hypotesized that GLR with summer ulceration and GLR with Sneddon's syndrome could represent two separate subsets with different physiopathology and clinical evolution

All contents copyright of the "On-line Archives of Rheumatology", 1996