“Written informed consent was obtained from the patient to
publish this report in accordance with the journal’s patient consent
policy”
Dear Editor;
Livedoid Vasculopathy (LV) is a chronic condition that presents as
recurrent, painful, ulcers of the lower leg, ankle, or dorsal foot. It
is an uncommon disease. We present a case of LV in a young woman.
A 30-year-old woman with no past medical history, presented in our
dermatology department for a 4-years history of painful ulcerated
lesions with serosanguineous crusts in the dorsal surface of her feet,
that appeared in summer season then healed spontaneously on winter. She
did not present Raynaud’s phenomenon or other systemic symptoms. She had
no history of spontaneous recurrent abortions or deep vein thrombosis.
Physical examination revealed several, painful, well-defined, purpuric
ulcerations, erythematous red-violaceous plaques and stellate, porcelain
white scars surrounded by punctate telangiectasia and brownish
pigmentation around ankle and dorsum of her feet (Figure 1a, 1b,
1c) . All peripheral pulses were palpable. Examination with dermoscopy
revealed central crusted violaceus ulcers surrounded by peripheral ivory
white areas (Figure 2a) . Skin biopsy showed a superficial
dermal vessel with thrombi, and hyalinized vessel walls (Figure
2b) . Antinuclear antibodies and cytoplasmic antineutrophil cytoplasmic
antibodies were negative. A coagulation investigation including
prothrombin activity, cephalin and thrombin times, functional
fibrinogen, protein C and protein S, antithrombin III, Factor V Leiden
mutation, anticardiolipin and anti-2-glycoprotein I antibodies, lupus
anticoagulant, cryoglobulins and cold agglutinins was negative. Serum
homocysteine levels were normal. The diagnosis of LV was made based on
clinics and histologic findings. The patient was treated with
anticoagulation with good improvement within two weeks (Figure
3a, 3b) .
Livedoid Vasculopathy (LV) is a noninflammatory condition, characterized
by recurrent painful purpuric ulcerations around the ankle and dorsum of
the feet that heal with stellate porcelain white scars called “atrophie
blanche”. It is an uncommon disease entity affecting 1% to 5% of the
population (1) classically presents in a middle-aged woman. it
does occur in males, and there are a few case reports of disease in
children (2) . It was originally described as a clinical
manifestation of vasculitis. However, at present, the main
physiopathogenic mechanism considered is a vasoocclusive phenomenon due
to intraluminal thrombosis of dermal venules. In general, there are
three main factors that predispose to thrombosis: endothelial damage,
changes in the blood flow and blood disorders leading to
hypercoagulability (3) . The presence of lupus anticoagulant
and/or anticardiolipin antibodies may also be demonstrated. Although
there is no consensus, and many cases such our patient, remain
idiopathic. Histopathology reveals segmental vessel wall hyalinization
of dermal blood vessels with fibrinoid deposition in lumen and minimal
perivascular lymphocytic infiltration (1) . Polyarteritis nodosa
(PAN) is a close differential diagnosis. Skin biopsies that include the
deep subcutaneous tissue are required for diagnosis. Its shows a
necrotizing vasculitis affecting medium-sized vessels (4) .
However, the coexistence of clinical and histological features of
cutaneous PAN and LV has been described (5). Several
therapeutic approaches have been employed with varying degrees of
success: hyperbaric oxygen therapy, Anticoagulant and Vasodilator drugs
are the most efficient. Livedoid vasculopathy remains an enigmatic
condition in terms of its etiopathogenesis. More investigations are
needed to explain it.