Introduction
Livedoid vasculopathy is a chronic, painful, thrombo-occlusive cutaneous vasculopathy that involves the lower extremities. It is a rare disease that has the clinically characteristic clinical features of livedoid skin changes (linear or angular, erythematous nodules), atrophie blanche (smooth, ivory-white plaques), and recurrent painful ulceration. It is most common in young to middle-aged women, and the course seems to be chronic with seasonal exacerbations.
The pathogenesis of livedoid vasculopathy is controversial and the disorder can be a primary or a secondary manifestation of inflammatory and hypercoagulation disorders. The diagnosis of this disease relies on clinical features and skin biopsy.
Systemic involvement is not common and clinical symptoms of this disease are mainly limited to skin. Peripheral nervous system involvement may occur due to multifocal thrombosis and ischemia of the vasa nervorum, but such cases are rare. So, we report a patient presenting with mononeuropathy multiplex combined with livedoid vasculopathy, which was confirmed by electrophysiological and pathological studies.
Case report
A 46-year-old woman visited our clinic complaining of left leg pain and foot drop in in February 2019. She had suffered from radiating pain in left lower extremity three months ago. She was diagnosed with livedoid vasculopathy in 1996 and was taking medication at another hospital.
On admission, neurological examination showed severe weakness in left ankle dorsiflexion and great toe extension (grade 0) and mild weakness in left ankle eversion, inversion, and plantarflexion. There was no sensory loss or pathologic reflex, and tendon reflex was normal. The patient had ulcerative skin lesions with livedo reticularis and multiple purpuric patches at the both ankles and distal lower legs. There were also painful chronic ulcerations in left foot with fibrosclerotic white scars.
Nerve conduction study showed axonal injury of both sensory and motor fibers nerve and sural nerves (table 1). In needle electromyography, there was no motor unit action potential in left tibialis anterior muscle, and no abnormal findings were found in other sampled muscles. This supported a diagnosis of mononeuropathy multiplex. At this stage, sural nerve biopsy was performed on the left sural nerve.
Microscopic pathological examination represented asymmetric loss of myelinated fibers both within and between fascicles. There were hyalinized dermal blood vessels with intravascular thrombosis in the perineurium and extensive infarct of the peripheral nerve and Schwann cells. Perivascular mononuclear cell infiltrates were rarely observed.
This patient was diagnosed with ischemic neuropathy associated with livedoid vasculopathy and treated with enoxaparin and oral methylprednisolone. Five days after the start of treatment, enoxaparin was converted to rivaroxaban. After a follow-up period of 2 weeks, neurological symptoms had considerably improved.
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Fig 1. Sural nerve biopsy. (A) hyalinized dermal blood vessels with intravascular thrombosis (B) Severe loss of myelinated fibers and some axonal degeneration due to ischemic necrosis (paraffin section, hematoxylin–eosin staining, ×100).
Table 1. Nerve conduction study. (A) Sensory nerve conduction study. (B) Motor nerve conduction study.
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