Clinical Analyses Of Livedoid Vasculopathy In 42 Cases

Background and ObjectiveLivedoid vasculopathy is a rare,chronic,recurrent thrombo-occlusive skin disease,which is mainly manifested in the presence of macules or purpuric papules with a tendency to form irregular ulcers which develop into star-shaped atrophic scars and peripheral hyperpigmentation.The pathogenesis of LV is unclear,and it is currently believed to be mainly related to blood hypercoagulability and autoimmunity.The histopathological features of LV are intraluminal thrombus,endothelial cell proliferation,and segmental hyaline degeneration or fibrinoid degeneration of the dermal blood vessels.Although there are many methods for treating LV,there isn't a specific treatment method currently,and the intractable cases often require combined treatment of multiple therapeutic modalities.Dermatoscopy can observe the microstructure and pigmented structures that are not visible to the naked eye in the lower epidermis,the epidermal junction,the dermal papilla layer,and the deep dermis.The dermoscopic features are sensitivite and specific to diagnose some skin diseases,and has corresponding relationship with the certain histopathological changes.To summarize the clinical manifestations,pathologic,and dermatoscopic characteristics of LV,and to explore the possible connections between dermoscopic and pathological features,so as to improve the diagnosis of LV in clinical practice,so that patients can obtain comprehensive treatment without delay and improve the prognosis.MethodsFrom January 2018 to January 2020,the total of 42 patients with livedoid vasculopathy in First Affiliated Hospital of Zhengzhou University were enrolled.These patients met the diagnostic criteria of clinical and histopathological features of livedoid vasculopathy.The general data,skin manifestations,concomitant diseases,laboratory inspection results,histopathological results,dermatoscopy,misdiagnosis,treatment plan and other data of patients included were collected,summarized and analyzed.Results1.GenderAmong the 42 patients with livedoid vasculopathy,14 were male and 28 were female.The ratio of male to female was 1:2.There was no significant difference in the gender and site of skin lesions(P>0.05).2.AgeAmong the 42 patients with livedoid vasculopathy,the youngest was 9 years old,the oldest was 56 years old,and the mean age was 25.45±13.1 years old.The course of disease was 1 to 240 months(29.36±40.9 months).3.Season and location3.1 The developed or worsened season of lesions of 42 LV patients:3 cases in spring,33 in summer,4 in autumn and 2 in winter.3.2 Among the 42 patients,there were only 3 cases with lesions around the ankle,5 cases in the calf,6 cases in the calf and the ankle,12 cases in the ankle and the back of the foot,14 cases in the calf,the ankle and the back of the foot,and 2 cases in the thigh,the calf,the ankle and the back of the foot.4.Skin lesions4.1 Subjective symptoms:All 42 cases had different degrees of pain,there were obviously pruritus in 8 cases.4.2 Features of skin lesion:Purpura-like rash was seen in 42 patients,and scab,sulcers,atrophic white scars with periphery telangiectasia and pigmentation were seen in 36 cases.5.Concomitant diseases3 patients with hypertension,2 patients with diabetic,1 patient with rheumatoid arthritis,1 patient with varicose veins of lower limb and 1 patient with parotitis.6.Inspection resultsIn this group of patients,1 case was rheumatoid factor positive,1 case was strongly positive for anti-Ro52 antibody,4 cases were ANA positive with low titer,and 3 cases were C3 decreased;Protein S activity decreased in 7 cases,protein S increased in 3 cases,protein C activity decreased in 3 cases,homocysteine levels increased in 6 cases,and folate levels decreased in 2 cases;Four patients had high antistreptolysin O,and 27 patients had elevated C-reactive protein and erythrocyte sedimentation levels.7.Histopathology and immunopathology examinationForty-two patients took skin lesions biopy for pathological examination.42 cases showed different degrees of hyaline degeneration or fibrinoid degeneration of vessel wall in the superficial dermal,28 cases with local red blood cell overflow,20 cases had lobular hyperplasia in the upper dermis and thrombosis was seen in some blood vessels,a small amount of inflammatory cells infiltrated around the blood vessels were seen in 20 cases,and 16 cases showed epidermal atrophy.Immunofluorescence examination of twenty-two patients showed C3 and IgM deposition in the dermal blood vessels,of which 16 were C3 and 6 were IgM.8.DermatoscopyDermatoscopy was performed on 42 patients with rash.There were 42 cases with pink and white background and linear and small globular vessels;36 cases with central ulcers,scabs,or white unstructured areas with peripheral pigmentation.9.MisdiagnosisOf the 42 patients,30 were misdiagnosed in previous visits,and there were multiple misdiagnosis.The situation is as follows:15 misdiagnosed as allergic vasculitis,8 misdiagnosed as Henoch-Schonlein purpura,5 misdiagnosed as nodular vasculitis,and 3 cases were misdiagnosed as pigmented purpuric dermatosis and 1 case was misdiagnosed as Wegener's granulomatosis.10.TreatmentAll 42 patients received systemic treatment.Six patients were in progressing stage at the time of admission and were given methylprednisolone sodium succinate intravenous guttae 30-40 mg/day.According to the stage and severity of LV,rivaroxaban tablets,aspirin enteric-coated tablets,and beraprost sodium tablets were selected orally.Saline wetdressing was applied to the patients with exudation if needed.Conclusion1.The most common pathological changes of LV are hyaline degeneration or fibrinoid degeneration of vessel wall and/or thrombosis in the blood vessel.Histopathological changes are the basis of dermoscopic features.2.Dermoscopic features of livedoid vasculopathy are pink and white background and linear and small globular vessels and white unstructured areas with peripheral reticular pigmentation.