Bandhlish Anshu, Aggarwal Asok, Koranne Ravinder V
Behl Skin Institute and School of Dermatology, Zamrudpur, Greater Kailash-I, New Delhi, India.
Indian J Dermatol. 2012 Jul;57(4):269-74. doi: 10.4103/0019-5154.97662.
Macular amyloidosis (MA) is the most subtle form of cutaneous amyloidosis, characterized by brownish macules in a rippled pattern, distributed predominantly over the trunk and extremities. MA has a high incidence in Asia, Middle East, and South America. Its etiology has yet to be fully elucidated though various risk factors such as sex, race, genetic predisposition, exposure to sunlight, atopy and friction and even auto-immunity have been implicated.
This study attempts to evaluate the epidemiology and risk factors in the etiology of MA.
Clinical history and risk factors of 50 patients with a clinical diagnosis of MA were evaluated. Skin biopsies of 26 randomly selected patients were studied for the deposition of amyloid.
We observed a characteristic female preponderance (88%) with a female to male ratio of 7.3:1, with a mean age of onset of MA being earlier in females. Upper back was involved in 80% of patients and sun-exposed sites were involved in 64% cases. Incidence of MA was high in patients with skin phototype III. Role of friction was inconclusive
Lack of clear-cut etiological factors makes it difficult to suggest a reasonable therapeutic modality. Histopathology is not specific and amyloid deposits can be demonstrated only in a small number of patients. For want of the requisite information on the natural course and definitive etiology, the disease MA remains an enigma and a source of concern for the suffering patients.
黄斑部淀粉样变(MA)是皮肤淀粉样变最隐匿的一种形式,其特征为呈波纹状的褐色斑疹,主要分布于躯干和四肢。MA在亚洲、中东和南美洲发病率较高。尽管性别、种族、遗传易感性、阳光暴露、特应性、摩擦甚至自身免疫等多种危险因素与之相关,但其病因尚未完全阐明。
本研究旨在评估MA病因学中的流行病学及危险因素。
对50例临床诊断为MA的患者的临床病史和危险因素进行评估。对随机选取的26例患者的皮肤活检标本进行淀粉样蛋白沉积研究。
我们观察到MA有明显的女性优势(88%),男女比例为7.3:1且女性MA发病平均年龄更早。80%的患者上背部受累,64%的病例阳光暴露部位受累。皮肤光型为III型的患者MA发病率较高。摩擦的作用尚无定论。
缺乏明确的病因因素使得难以提出合理的治疗方式。组织病理学不具有特异性,仅在少数患者中能证实有淀粉样蛋白沉积。由于缺乏关于自然病程和确切病因的必要信息,MA疾病仍然是一个谜,也是患者痛苦的根源。