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巴西利亚大学医院皮肤科门诊所诊治的大疱性表皮松解症患者的营养、血液学和感染特征与疾病类型分类之间的相关性

Correlation between nutritional, hematological and infectious characteristics and classification of the type of epidermolysis bullosa of patients assisted at the Dermatology Clinic of the Hospital Universitário de Brasília.

作者信息

Sousa Márcia Carolline dos Santos, Paula Carmen Dea Ribeiro de, Tauil Pedro Luiz, Costa Izelda Maria Carvalho

机构信息

Universidade de Brasília, Brasília, DF, Brazil.

出版信息

An Bras Dermatol. 2015 Nov-Dec;90(6):922-4. doi: 10.1590/abd1806-4841.20154575.

Abstract

Epidermolysis bullosa comprises a group of phenotypically different genodermatosis, hereditary or acquired, characterized by skin fragility and subsequent formation of blisters in response to mechanical trauma, and which may also affect mucous membranes. This study aimed to analyze the relation between the nutritional, hematologic, infectious characteristics and the type of epidermolysis bullosa, through a descriptive case study based on data from medical records of 10 patients with epidermolysis bullosa assisted regularly at the Dermatology Clinic of the Hospital Universitário de Brasília. The old classification of the type of epidermolysis bullosa, weight and height, blood count, white blood cell count, platelet count and description of the type and frequency of secondary infections during the service were considered. We verified a predominance of iron deficiency anemia, chronic leukocytosis, thrombocytosis, chronic malnutrition, low height for age and thinness, and people with epidermolysis bullosa simplex exhibited appropriate relation between height/age and BMI/age. The non-specific skin infection was the most prevalent in both sexes. The severity of the type of epidermolysis bullosa and frequency of secondary infections did not form a directly proportional relation. The absence of direct proportion in all cases between the type of epidermolysis bullosa and the analysis parameters suggest a possible significant interference from other aspects such as the extent of the affected skin area, extracutaneous type of engagement and specific genetic mutation. The inclusion of these factors in the new classification proposed by Fine et al can contribute significantly to a better correlation of clinical parameters and appropriate preventive and therapeutic approaches.

摘要

大疱性表皮松解症是一组表型各异的遗传性或获得性遗传性皮肤病,其特征为皮肤脆弱,在受到机械创伤后会形成水疱,并且也可能累及黏膜。本研究旨在通过一项描述性病例研究,基于巴西利亚大学医院皮肤科门诊定期诊治的10例大疱性表皮松解症患者的病历数据,分析营养、血液学、感染特征与大疱性表皮松解症类型之间的关系。研究考虑了大疱性表皮松解症类型的旧分类、体重和身高、血常规、白细胞计数、血小板计数以及就诊期间继发感染的类型和频率描述。我们发现缺铁性贫血、慢性白细胞增多、血小板增多、慢性营养不良、年龄别身高偏低和消瘦较为常见,单纯型大疱性表皮松解症患者的身高/年龄与BMI/年龄之间呈现出适当的关系。非特异性皮肤感染在两性中最为普遍。大疱性表皮松解症的严重程度与继发感染的频率并未形成直接的比例关系。在所有病例中,大疱性表皮松解症类型与分析参数之间缺乏直接比例关系,这表明可能受到其他方面的显著干扰,如受影响皮肤区域的范围、皮肤外受累类型和特定基因突变。将这些因素纳入Fine等人提出的新分类中,可显著有助于更好地关联临床参数以及采取适当的预防和治疗方法。

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