淀粉样蛋白沉积可能是许多慢性疾病的原因。原发性局部皮肤淀粉样变性(PLCA)是一种慢性皮肤病,在乳头状真皮中存在淀粉样蛋白沉积。最常见的角质形成细胞衍生形式的PLCA包括黄斑(MA),地衣(LA),和双相(BA)淀粉样变性。亚洲人口中PLCA的估计患病率为0.98/10,000,高于欧洲人口;因此,白种人人群中PLCA的流行病学数据有限.我们进行了一项回顾性单中心研究,分析了中欧PLCA人群的流行病学特征。关于年龄的流行病学数据,性别,皮肤光型(Fitzpatrick量表I-VI),疾病持续时间,合并症,过敏症的历史,收集PLCA家族史。临床特征,PLCA病变的定位,还分析了应用的疗法和治疗结果.还评估了皮肤特征。共纳入41例诊断为PLCA的患者,22岁出现黄斑,18与地衣,1为双相淀粉样变性。男女比例为16/25,诊断时的平均年龄为54.6±15.2岁(范围27-87岁)。在MA中,PLCA发作的平均年龄为53±16.1岁(范围19-79岁),洛杉矶46.7±18.2年(范围14-73年),在BA工作了26年。MA的肩胛骨间区域和LA的下肢伸肌表面被证明是与定位相关的区域。在我们的中心,应用了广泛的治疗选择,在所有类型的PLCA中,处方最多的是局部用皮质类固醇。我们提出了一个回顾,中欧地区PLCA流行病学的单中心研究。通过检查大量PLCA患者的医疗数据,我们将我们的流行病学数据与亚洲PLCA人群的流行病学数据进行了比较.由于这种情况的罕见,需要进一步的随机对照试验和指南来改善治疗结果.
Amyloid deposits can be the cause of many chronic diseases. Primary localized cutaneous amyloidosis (PLCA) is a chronic dermatologic condition with amyloid deposits in the papillary dermis. The most common types of the keratinocyte-derived form of PLCA include macular (MA), lichen (LA), and biphasic (BA) amyloidosis. The estimated prevalence of PLCA in the Asian population is 0.98/10,000, which is higher than in the European population; thus, epidemiologic data on PLCA in the Caucasian population are limited. We performed a retrospective single-center study analyzing epidemiologic characteristics of a Central European PLCA population. Epidemiologic data regarding age, sex, skin phototype (Fitzpatrick scale I-VI), disease duration, comorbidities, history of atopy, and family history of PLCA were collected. Clinical characteristics, localization of PLCA lesions, applied therapies and treatment outcomes were also analyzed. Dermoscopic characteristics were also evaluated. A total of 41 patients diagnosed with PLCA were included, with 22 presenting with macular, 18 with lichen, and 1 with biphasic amyloidosis. The male/female ratio was 16/25, and mean age at diagnosis was 54.6 ± 15.2 years (range 27-87 years). The mean age at the onset of PLCA was 53 ± 16.1 years (range 19-79 years) in MA, 46.7 ± 18.2 years (range 14-73 years) in LA, and 26 years in BA. The interscapular region in MA and the extensor surface of the lower extremities in LA proved to be localization-related areas. In our center, a wide range of therapeutic options was applied, with the most prescribed being topical corticosteroids in all types of PLCA. We presented a retrospective, monocentric study on the epidemiology of PLCA in the Central European region. By examining the medical data of a significant number of PLCA patients, we compared our epidemiologic data with that of the Asian PLCA population. Due to the rarity of the condition, further randomized controlled trials and guidelines are needed to improve therapeutic outcomes.