面部和颈部毛囊性红斑黑变病——一项横断面描述性研究
Erythromelanosis Follicularis Faciei et Colli - A Cross-sectional, Descriptive study.
作者信息
Rather Shagufta, Yaseen Atiya, Mukhija Mani
机构信息
Department of Dermatology, Venereology and Leprology, Government Medical College and Hospital, Srinagar, Jammu and Kashmir, India.
Pathologist, Lal Paths Lab, New Delhi, India.
出版信息
Indian J Dermatol. 2016 May-Jun;61(3):308-13. doi: 10.4103/0019-5154.182419.
BACKGROUND
Erythromelanosis follicularis faciei et colli (EFFC) has always been reported as a rare disorder, and more data are needed to define its etiology and epidemiology.
OBJECTIVES
To present a descriptive study of this disorder from Kashmir and present a review of literature on the same.
MATERIALS AND METHODS
A cross-sectional, descriptive study was conducted on 14 patients with clinical lesions suggestive of EFFC, presenting to our dermatology outpatient clinic between May 2013 and April 2015. After obtaining informed consent from all patients, the demographic and clinical data were collected and punch biopsies were taken which after being fixed in formalin were stained for hematoxylin and eosin. The study was approved by Institutional Review Board.
RESULTS
Age of the patients ranged from 12 to 35 years with a mean age of 24.8 years. Females outnumbered males in a ratio of 1.3:1. Family history was positive in one case, and one patient had unilateral presentation. The classical triad of erythema, pigmentation, and follicular papules were present in 100% patients. Telangiectasias were observed in 28.57% patients only. Classical sites described were involved in all the cases (100%). In addition, lesions were found to involve eyebrows (28.57%), forehead (28.57%), upper lips (14.28%), and pinna (21.42%). Keratosis pilaris was present in all patients (100%); most common sites affected were upper arms and thighs. Two (14.28%) had generalized distribution. Histopathology in all correlated well with clinical findings.
CONCLUSION
EFFC has always been reported as a rare disorder, but we believe that it is not so. The reason could be either lack of awareness of the disease by the patient due to its generally asymptomatic nature or under-recognition by the physicians. Awareness about the disease on part of dermatologist and recognition of clinical presentation is important for correct diagnosis and to help find more effective therapeutic modalities.
背景
面部及颈部毛囊性红斑黑变病（EFFC）一直被报道为一种罕见疾病，需要更多数据来明确其病因和流行病学特征。
目的
对克什米尔地区的这种疾病进行描述性研究，并对相关文献进行综述。
材料与方法
对2013年5月至2015年4月期间到我们皮肤科门诊就诊的14例临床皮损提示为EFFC的患者进行了横断面描述性研究。在获得所有患者的知情同意后，收集了人口统计学和临床数据，并进行了打孔活检，活检组织用福尔马林固定后进行苏木精和伊红染色。该研究获得了机构审查委员会的批准。
结果
患者年龄在12至35岁之间，平均年龄为24.8岁。女性与男性的比例为1.3:1。有1例患者有家族史，1例患者为单侧发病。100%的患者出现了红斑、色素沉着和毛囊丘疹这一经典三联征。仅28.57%的患者观察到毛细血管扩张。所有病例（100%）均累及经典描述部位。此外，发现皮损还累及眉毛（28.57%）、前额（28.57%）、上唇（14.28%）和耳廓（21.42%）。所有患者（100%）均有毛发角化病；最常受累的部位是上臂和大腿。2例（14.28%）呈泛发性分布。所有患者的组织病理学表现均与临床 findings相符。
结论
EFFC一直被报道为一种罕见疾病，但我们认为并非如此。原因可能是由于该病通常无症状，患者对其缺乏认识，或者医生认识不足。皮肤科医生对该病的认识以及对临床表现的识别对于正确诊断和寻找更有效的治疗方法很重要。
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