clinical findings are petechiae with painful ulcers in both lower extremities , which heal to become hyperpigmented and porcelain - white satellite lesions . there are only a few reported cases of livedoid vasculitis presenting in combination with peripheral neuropathy . we report the first case of a korean patient presenting with mononeuritis multiplex combined with livedoid vasculitis , which was confirmed by electrophysiological and pathological studies . our report supports the possible vaso - occlusive etiology of livedoid vasculitis in multifocal ischemic neuropathy . livedoid vasculitis is a rare disease that has the clinically characteristic symptoms of purpuric macules and papules of the lower legs and feet . these purplish lesions develop into irregularly shaped ulcers that ultimately heal into stellate white scars or atrophie blanche . it is most common in young to middle - aged women , and the course seems to be chronic with seasonal exacerbations.1 because of these characteristic findings it has many other synonyms , such as segmental hyalinizing vasculitis , atrophie blanche , livedo reticulitis with summer / winter ulceration , hypersensitivity - type vasculitis , and painful purpuric ulcers with reticular pattern of the lower extremities.2 although the typical dermatological findings and pathogenesis of this condition have been emphasized previously , the involvement of peripheral neuropathy with this dermatological vasculopathy is very rare.3 - 5 a 48-year - old woman presented with a 2-year history of tingling sensation and numbness of her right fingers . two years prior to her hospital visit , she began to notice that her right ring finger and little finger were numb and tingling ; 7 months later , bruises suddenly appeared on her whole body , and these persisted only on both legs . these multiple purpuric patches developed into painful ulcerated wounds located mostly on the left lateral malleolar areas . the ulcers healed within 2 months , leaving atrophic scars . more and more similar skin lesions developed on both lower extremities , exhibiting a waxing and waning course . during this time , the patient also complained of num - bness and a tingling sensation on the dorsum of both feet , which was more severe on the right . her previous medical history included a diagnosis of multiple cysts in the kidney and pancreas . she had also undergone a distal pancreatectomy ; the removed tissue had pathologically confirmed serous oligocystic adenoma . when initially evaluated in our hospital , her cranial nerve function , motor function including muscle bulk , tone , power and deep - tendon reflex , cerebellar function test , and gait were all normal . however , a sensory examination revealed about a 50% hypesthesia for touch and pinprick sensation on the dorsum of both feet , the right fourth and fifth fingers , and the medial palm , which is innervated by the ulnar nerve . multiple irregularly shaped ulcerative skin lesions and eschar with some healed ivory - white colored atrophic scars were seen in the left lateral malleolar area ( fig . the results of the following laboratory studies regarding autoimmune disease and coagulation defects were normal , negative , or nonspecific : complete blood count , routine blood chemistry with blood glucose , urinalysis , serological test for syphilis , human immunodeficiency virus , and hepatitis b , sedimentation rate , antinuclear antibody , anti - double - stranded dna antibodies , antineutrophilic cytoplasmic antibodies , antiphospholipid antibody , anticardiolipin antibody , anti - sjgren 's syndrome ( ss)a / ssb antibody , protein c , protein s , cryoglobulin , rheumatoid factor , thyroid function test , vitamin b12 , folate , creatine kinase , platelet aggregation panel , antithrombin iii activity , gene study of factor v leiden mutation , and prothrombin 20210 g > a . a cerebrospinal - fluid examination and doppler ultrasound of both lower extremities produced normal results . the initial nerve conduction study ( table 1 ) revealed a diminished right ulnar sensory nerve action potential , and both sural sensory nerve action potentials were absent , even with repetitive stimulation . the amplitudes of the compound motor nerve action potentials of both the peroneal and posterior tibial nerves were decreased . the findings were consistent with livedoid vasculopathy with fibrin deposition within the vessel walls , and thrombi and scattered perivascular lymphocytic inflammation . a nerve biopsy sample was removed from the right sural nerve , which revealed endoneurial capillary ectasia and congestion with hemorrhage , with extensive infarct of the peripheral nerve and schwann cells . a marked degeneration of axons and the myelin sheath were observed , and the schwann cell cytoplasm contained many autophagic vacuoles and myelin figures ( fig . 2 ) . there was no definite evidence of vasculitis in the form of vessel necrosis and leukocytoclasia . this patient was diagnosed with ischemic neuropathy associated with livedoid vasculitis and treated with the antithrombotic and antifibrinolytic agents pentoxifylline and clopidogrel , respectively . while the name livedoid vasculitis is generally used , histopathological studies have shown that this condition can be more accurately described as livedoid vasculopathy . typical histopathological findings of livedoid vasculitis are fibrin deposition within the affected walls of blood vessels and thrombus formation within the lumen . the extent of any inflammation is very limited.6,7 these findings were consistent with our patient 's skin and nerve biopsy findings , showing ischemic neuropathy more than vasculitic neuropathy . livedoid vasculitis has been described as a sole entity or in association with other diseases , such as primary systemic lupus erythematosus , antiphospholipid antibody syndrome,8,9 and other conditions associated with abnormalities of the coagulation system ( e.g. , protein c deficiency,10 abnormalities of the tissue plasminogen activator system,11 antithrombin iii deficiency,12 elevated homocysteine levels,13 and factor v leiden mutations).14 although the pathogenesis of livedoid vasculitis between vaso - occlusive and vasculitis continues to be debated , these pathologic and etiological studies lean toward the vaso - occlusive mechanism as a primary pathogenesis.15 the sequential nature of the numbness and tingling sensation in the patient 's right hand and both feet is a classic feature of mononeuropathy multiplex . many systemic diseases can be related to mononeuropathy multiplex , such as vasculitis , connective tissue disease , cryobulinemia , sarcoidosis , diabetes , amyloidosis , neoplasm , and infections . the patient exhibited mononeuropathy multiplex with characteristic skin lesions , and so we could limit the disease entity to connective tissue diseases such as systemic lupus erythematosus , ss , and antiphospholipid antibody syndrome , and vasculitis conditions such as polyarteritis nodosa , infectious diseases such as leprosy or syphilis , and livedo vasculopathy . however , our laboratory results did not support the presence of any connective tissue diseases or vasculitis . furthermore , the skin lesions that formed petechiae - which comprised recurrent , irregularly shaped , hyperpigmented ulcers and atrophie blanche - also differed from the derma - tological findings of both leprosy and syphilis . leprosy is usually associated with more hypopigmented and well - defined lesions , with occasional plaque and tuberculoid - like changes.16 syphilis characteristically presents hard , slightly elevated , round ulcers in the mouth and genital area known as chancres , and the subsequent formation of gummata.17 with the aid of an exclusive diagnostic process and examination of histopathological findings , we concluded that the patient was suffering from livedoid vasculopathy , and more specifically , livedoid vasculitis . her mononeuropathy multiplex can be explained by multifocal axonal damage from thrombotic obstruction of the vasa nervorum and the consequent ischemia . however , given its potential pathogenesis of coagulation abnormality and ischemia phenomenon , many antithrombotic and antifibrinolytic agents have been routinely used , including heparin,18 tissue plasminogen activator,19 pentoxifylline , aspirin , and dipyridamole,20 with long - term anticoagulation,21 danazol,22 ketanserin,23 beraprost,12 and prostacyclin.24 other immunomodulating therapies such as steroids and immunoglobulins have also been applied , but it has been shown that they do not provide long - term benefits.25 we report herein a patient who experienced livedoid vasculitis combined with a rare neurological disease , mononeuropathy multiplex , which was proven to be ischemic neuropathy by nerve biopsy . these phenomena are thought to be attributable to a coagulation abnormality , which is also the main pathological cause in livedoid vasculitis . other causes of coagulation defects were not revealed in this case . to the best of our knowledge , this is one of only very few reports of ischemic neuropathy associated with livedoid vasculitis , and has provided valuable insights into the disease pathomechanism . our pathologic findings and other laboratory findings were all negative for systemic vasculitis , thus supporting the vaso - occlusive mechanism of livedoid vasculitis .
backgroundlivedoid vasculitis is a chronic dermatological problem with an unclear etiology . 
 clinical findings are petechiae with painful ulcers in both lower extremities , which heal to become hyperpigmented and porcelain - white satellite lesions . 
 there are only a few reported cases of livedoid vasculitis presenting in combination with peripheral neuropathy.case reportwe report the first case of a korean patient presenting with mononeuritis multiplex combined with livedoid vasculitis , which was confirmed by electrophysiological and pathological studies.conclusionsour report supports the possible vaso - occlusive etiology of livedoid vasculitis in multifocal ischemic neuropathy .