| Background This disease was first described by Von Baerensprung in 1863.It is called unilateral nevi,also known as linear epidermal nevus,verrucous nevus,verrucous nevus,etc.There are dozens of different names for the same disease.Epidermal nevi(EN)is a group of heterogeneous benign skin hamartomas,which usually occur at birth or early childhood,especially in the first year after birth.The incidence of EN in adults is very rare.It is mainly distributed,but there are also family reports.EN originates from the ectoderm and is usually distributed along the Blaschko line.Molecular studies of skin lesions in patients with unilateral pseudohair follicle keratosis(Darier 's disease)and hyperkeratotic keratosis have revealed that EN is now considered to be a manifestation of skin gene mosaic mutations.Verrucous Epidermal Nevus(VEN)is the most common epidermal nevus,with an incidence rate of about one-thousandth.The specific classification of VEN has not yet reached a unified understanding.Based on its clinical manifestations and the extent of skin lesions,it is currently clinically divided into three types,namely localized VEN,generalized or systemic VEN,and inflammatory linear verrucous epidermal nevus.Localized VEN lesions are often arranged as unilateral continuous or intermittent bundles,bands,or patches.Head skin lesions are often patchy.Skin lesions of the limbs are often distributed along the limbs,reaching the extremities,and are arranged in a line like a line on the trunk.If it is only on one side of the body,it is also called unilateral mole.Generalized or systemic VEN can be bilateral,multiple or widespread,or even widely distributed throughout the body,with swirling or arc-shaped stripes,and the severe case is called porcupine ichthyosis.Inflammatory linear verrucous epidermal nevus(ILVEN)has a special performance.It is a clinical and pathological variant of verrucous epidermal nevus.The incidence is higher in women than in men,and the ratio of male to female is 1: 4.It mainly affects the left side of the body,especially the left lower extremity.Skin lesions are often accompanied by itching.If EN is accompanied by neurological,ocular,skeletal dysplasia or other abnormalities,such as heart and kidney dysplasia,it is called epidermal nevus syndrome(ENS).The treatment of EN is difficult,and topical drugs,such as topical Vitamin A drugs;disruptive therapies,such as electrocoagulation or cryotherapy;these treatments can only temporarily relieve symptoms,and relapse is inevitable.The best treatment is a full-thickness excision of the skin,but it is more difficult for patients with generalized hairstyles.CO2 laser treatment is a good choice.However,scars and pigmentation are potential permanent complications,especially for patients with darker skin.At present,there are few researches on verrucous epidermal nevus at home and abroad.On the one hand,in order to understand the clinical characteristics of verrucous epidermal nevus patients in China,their associated diseases and the level of cognition of their own diseases,and to understand the patients and their families On the other hand,considering that the incidence of verrucous epidermal nevus is not particularly high,and the number of outpatients in public hospitals in China is large,and due to problems such as time or geographical difficulties in seeking medical treatment,we decided to use the form of an online questionnaire to apply " The epidemiological survey of verrucous epidermal nevus is a questionnaire survey of verrucous epidermal nevus patients and their families across the country.We hope to use this study to summarize and analyze the clinical characteristics and associated diseases of verrucous epidermal nevus patients in China.It provides theoretical basis for individualized diagnosis and treatment of patients with verrucous epidermal nevus,which is conducive to disease education,enhance patients 'self-management awareness,and improve patients' quality of life.Object Investigate the clinical characteristics,concomitant diseases and cognitive level of patients with verrucous epidermal nevus in China,and understand the needs of patients with verrucous epidermal nevus.Methods The questionnaire survey of patients with epidermal nevus verrucous nevus and their families throughout the country was carried out using the questionnaire in the form of a network questionnaire.Results Of the 530 patients with verrucous epidermal nevus,the youngest was 1 month old and the oldest was 58 years old.The male to female ratio was 1: 1.04,with no significant difference.The proportion of each type of epidermal nevus is 54.34% of the localized type,25.66% of the generalized or systemic type,9.06% of the inflammatory type,and 10.94% of the unclear type.Only 3.39% of patients had a family history.It is worth noting that 74.91% of the patients and their families said that the disease affected mental health.Patients or family members have a high awareness of the disease,88.49% of patie.Conclusions In this survey,patients with verrucous epidermal nevus in China were mainly concentrated in eastern and southern China.The sex ratio of men and women with epidermal nevus was 1: 1.04,and the male-to-female ratio of inflammatory linear verrucous epidermal nevus was 1.53: 1.Local epidermal nevus is more common in China,followed by pan-type verrucous epidermal nevus,and inflammatory linear epidermal nevus only accounts for 10.17%(excluding patients who do not know the type in the survey).Epidermal moles are most commonly diagnosed at birth from the first year after birth.The main way to confirm the diagnosis of the patient is doctor's notification and pathological examination.Skin lesions in non-ILVEN patients usually do not feel or feel dry,while skin lesions in ILVEN patients feel the majority itching.In non-ILVEN patients,the initial sites of skin lesions are more common in the lower limbs,trunk,upper limbs,and neck.The initial sites of ILVEN skin lesions are more common in the hips,upper limbs,lower limbs,and trunk.The incidence of disease associated with ILVEN patients is higher than that of non-ILVEN patients,and the skin lesions of ILVEN patients are more susceptible to seasonal changes.Patients and their families need to solve the problems of continuous enlargement of skin lesions and appearance of skin lesions.And the disease seriously affects the mental health of patients.Patients and their families have a certain degree of awareness of the disease,but the disease has not received enough attention from clinicians... |
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