Livedoid vasculitis: an update / 中华皮肤科杂志

Livedoid vasculitis is a kind of skin disease caused by local vascular occlusion.It mainly manifests as erythematous and purpuric maculas or papules on the legs and ankles,which may progress into intensely painful ulcers and heal leaving porcelain-white atrophic scars called atrophie blanche.Histopathologically,inflammatory response is weak or absent in local lesions of livedoid vasculitis,hence,it is commonly considered to be related to local thrombosis and other autoimmune diseases.Now,it is mainly managed with anticoagulation-based comprehensive treatment in clinic.

Ischemic Neuropathy Associated with Livedoid Vasculitis

BACKGROUND: Livedoid 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 REPORT: 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. CONCLUSIONS: Our report supports the possible vaso-occlusive etiology of livedoid vasculitis in multifocal ischemic neuropathy.

Successful treatment of refractory livedoid vasculitis with rituximab and cyclophosphamide / 대한내과학회지

Livedoid vasculitis (LV) is a chronic recurrent cutaneous disease that is characterized by a netlike vascular pattern of purpuric lesions that progress to ulcers and atrophic white scars. The etiology of LV is unknown. Various therapies have been used for LV with inconsistent results, including antiplatelet agents, fibrinolytics, anticoagulants, prostaglandin E1, and immunosuppressive agents, such as systemic corticosteroids, azathioprine, methotrexate, cyclosporine, and mycophenolate mofetil. We present a patient with idiopathic LV refractory to immunosuppressive therapy who had a good response to treatment with rituximab and cyclophosphamide.

Livedoid Vasculitis: a Clinico-Pathological Study of 19 Patients / 대한피부과학회지

BACKGROUND: Livedoid vasculitis is a distinctive dermatosis characterized by recurrent chronic ulceration and infiltrated purpuric papules on the lower limbs. OBJECTIVE: The purpose of this study was aimed at evaluating the clinical and histopathological features of livedoid vasculitis. METHODS: The hospital charts and histopathologic slides of patients with livedoid vasculitis diagnosed at Asan Medical Center from 1989 to 1999 were reviewed. RESULTS: Twelve male and seven female patients were enrolled in this study(M: F=1.7: 1). The mean age at onset was 30.3 years in men and 25.3 years in women. The mean duration of illness was 3.3 years in male patients and 5.7 years in female patients. All the patients were presented with recurrent chronic ulceration and atrophic scarring. Twelve patients(63.2%) complained of severe pain and tenderness of the lesions and 5 patients(26.3%) complained of itching sensation. In three patients(15.8%), the lesions developed or aggravated in summer. Alcohol intake was aggravating factor in two patients(10.5%) and smoking was in one patient(5.3%). In five patients(26.3%), livedoid vasculitis develops in association with several diseases. Associated diseases were diabetes mellitus in 2 patients(10.5%), antiphospholipids antibody syndrome in 2 patients(10.5%), and protein S deficiency in 1 patient(5.3%). Histopathological examination revealed hyalinized blood vessels, partial to complete obstruction of dermal blood vessels with fibrinoid thrombi, endothelial swelling, and extravasation of RBCs in upper and mid-dermis. Panniculitis-like feature was common finding(73%). In six patients(31%), moderate to severe inflammatory reaction was observed in dermis. Five patients were treated with aspirin, dipyridamole, and pentoxifylline, and among them, only 1 patient(20%) were improved. All the treatment response including pentoxifylline alone, or pentoxifylline plus aspirin, dipyridamole, or corticosteroid was unsatisfactory. In cases of three patients whom were treated with low-dose danazol, all the patients(100%) showed marked improvement. One patient was treated with dapsone with improvement. CONCLUSION: Livedoid vasculitis is a distinct dermatosis with characteristic clinico-pathological features. Low-dose danazol or dapsone may be useful therapeutic options in this intractable disease.

Three Cases of Livedoid Vasculitis Improved by Low-dose Danazol

Livedoid vasculitis is characterized clinically by smooth or depressed ivory-white scars surrounded by hyperpigmentation and telangiectasia with or without preceding purpuric in-filtrated papules and plaques and histologically by intravascular deposition of fibrin. Its pathophysiology is still obscure. Many treatment modalities have been used, but results are not satisfactory. Recently, there have been experiences of successful treatments with danazol, a synthetic androgen. We used danazol in three patients who presented with livedoid vasculitis and were relatively resistant to treatments with aspirin and/or pentoxifylline. After 4 to 8 weeks, there was remarkable clinical improvement in all three patients.

A Case of Livedoid Vasculitis / 대한피부과학회지

A 47-year-old male patient presented us with erythematous, telangiectatic, purpuric or hyperpigmented patqhes, recurrent painful ulceration and ivory white atrophic scars on the lower portions of his legs, ankles and dorsal surfaces of both feet exacerbating on every summer for 5 years. The histopathalogical findings of an atrophic patch showed endothelial proliforetiion, partial occlusion of the dermal capillaries and fibrinoid material) on the superficial blood vessels, This patient was treated with aspirin and dipyriclamole for 4 months, and the skin lesions were improved significantly.