Linear IgA Dermatosis in a Child: Clinical Case

Nikolay N. Murashkin, Alexander I. Materikin, Roman V. Epishev, Maria A. Leonova, Leonid A. Opryatin, Roman A. Ivanov, Alena A. Savelova, Oksana R. Katunina, Kirill A. Kulikov, Daniel R. Danielyan
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Abstract

Background. Linear IgA dermatosis is a rare autoimmune bullous disease characterized by vesiculo-bullous subepidermal lesions (affecting the skin and mucous membranes) and by linear homogeneous IgA deposition in the epidermis basal membrane. Clinical case description. Clinical case of linear IgA dermatosis in children is presented. Clinical data assessment, histological examination of the skin biopsy (to determine the depth of bullous), immunofluorescent examination (to reveal IgA deposition in the epidermis basal membrane) are crucial for reliable disease diagnosis according to the studies. Altogether it helps to establish the final diagnosis and determine the patient's management. Dapsone is the first-line treatment for this disease, it has proven to be an effective and safe medication. Conclusion. This case of linear IgA dermatosis is of concern due to disease severity and its rarity in clinical practice. Differential diagnosis is rather complicated and clinically requires high-tech research methods. Only immunofluorescent examination allows to diagnose linear IgA dermatosis accurately. Lesions' regression was achieved due to systemic therapy with dapsone (1.8 mg/kg/day).
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1例儿童线状IgA皮肤病临床病例
背景。线性IgA皮肤病是一种罕见的自身免疫性大疱性疾病,其特征是表皮下的囊泡大疱性病变(影响皮肤和粘膜)和表皮基膜的线性均匀IgA沉积。临床病例描述。报告1例儿童线状IgA皮肤病。临床资料评估、皮肤活检组织学检查(确定大疱深度)、免疫荧光检查(显示表皮基膜IgA沉积)对可靠的疾病诊断至关重要。总之,它有助于建立最终诊断和确定病人的管理。氨苯砜是这种疾病的一线治疗药物,它已被证明是一种有效和安全的药物。结论。本例线状IgA皮肤病因其严重程度和临床罕见而引起关注。鉴别诊断相当复杂,临床上需要高科技的研究方法。只有免疫荧光检查才能准确诊断线性IgA皮肤病。病变的消退是由于全身治疗氨苯砜(1.8 mg/kg/天)。
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来源期刊
Voprosy Sovremennoi Pediatrii - Current Pediatrics
Voprosy Sovremennoi Pediatrii - Current Pediatrics Medicine-Pediatrics, Perinatology and Child Health
CiteScore
0.70
自引率
0.00%
发文量
48
审稿时长
8 weeks
期刊介绍: The main purpose of the academic journal "Current Pediatrics" is to cover the issues related to health, emotional state, and social adaptation of children. In the journal we publish the most pressing issues of research and applied problems (such as social paediatrics; clinical findings, anamnesis, and diagnosis of paediatric diseases; prevention and rehabilitation; supply of healthy and sick child), reviews on the current state of medical science and management of health care in Russia. Along with the concern for providing a high level of basic research publications, the journal, being a mass media tool, tends to meet the interests and requirements of practitioners from different regions and bring up vital and urgent questions. To accomplish this purpose the journal includes the materials of practical interest presented in the following sections: "Exchange of experience", "Doctor''s aid", "Continuous professional education", "Expert Opinion". The journal presents actual official information from the Union of Paediatricians of Russia and publishes materials on the history of medical science, congresses, seminars, conferences, both in our country and abroad.
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