Pudasaini Prajwal, Paudel Sushil, Gc Sagar, Adhikari Sadiksha, Pudasaini Prashanta, Das Kinnor, Pietkiewicz Paweł
Department of Dermatology Civil Service Hospital Kathmandu Nepal.
Department of Dermatology Gandaki Medical College and Teaching Hospital Pokhara Nepal.
Skin Health Dis. 2023 Nov 27;4(1):e316. doi: 10.1002/ski2.316. eCollection 2024 Feb.
Amyloidosis, deposition of misfolded protein in body, is a fairly common condition. The deposition of misfolded proteins in skin which occurs in absence of systemic comorbidities, namely Primary Cutaneous Amyloidosis (PCA) is also a well-known entity in skin of colour patients of Asian subcontinent. Primary Cutaneous Amyloidosis is usually diagnosed with good clinical acumen and typical clinical phenotype and involved site. Dermoscope has been used as an adjunct non-invasive tool to confirm cases with diagnostic uncertainty and in those in whom biopsy is deferred. Typical dermoscopic features of PCA helps differentiate it from other pigmentary dermatoses and avoids unwanted invasive biopsies and investigations especially in resource poor settings with financial constraints.
This study aims to identify and corroborate clinically, typical dermoscopic features in PCA in 42 patients which includes Macular Amyloidosis (MA) and Papular Amyloidosis (PA) predominantly in skin of colour patients from government based hospital of a south east Asian country.
Patients with classic clinical features of PCA were selected. Primary Cutaneous Amyloidosis was subclassified into MA or PA and their corresponding clinically corroborative dermoscopic features were enlisted respectively. All patients (treatment naïve and previously treated), who consented to participate in the study were included. Patients were diagnosed based on the prototypical clinical features. Dermoscopy was done using DermLite III DL3N Polarised and Fluid Dermoscope w/PigmentBoost Brand (3Gen, DermLite LLC, San Juan Capistrano, CA, USA) and images were obtained to create digital dermoscopy system by attaching camera-equipped mobile device via an optional connection kit (Redmi Note 11, MIUI version 13.0.5, CHINA) and the findings were enlisted concurrently.
In this study of dermoscopic findings of PCA, 42 patients were evaluated for their clinical lesions along with its corroboration with the dermoscopic features. Macular Amyloidosis was seen in 30 patients and 12 patients had typical cutaneous phenotypic and dermoscopic feature of PA. The most common dermoscopic finding seen in patients with MA was shiny to dull white, circular or oval central hub surrounded with halo of light brown dots. Most common configuration of brownish pigmentation around central hub was fine streak type. Also eccrine clues were seen in some cases of MA, which was a unique finding. Similarly in the PA subtype, the central hub was replaced by scar like structureless translucent white area surrounded by brownish black dot like structures, especially in those with large and thick plaques.
Dermoscopic findings of PCA and their clinical corroboration is a much-needed aspect in treating patients with pigmentary disorders and in those with skin of colour, especially in developing countries. Utilization of dermoscope in clinical settings of low income countries and in government based hospitals will decrease the add on economic burden of invasive diagnostic modalities like biopsy and other inadvertent tests done to rule out pigmentary conditions.
淀粉样变性是一种较为常见的病症,即错误折叠的蛋白质在体内沉积。在没有全身性合并症的情况下,错误折叠的蛋白质在皮肤中沉积,即原发性皮肤淀粉样变性(PCA),在亚洲次大陆有色人种患者的皮肤中也是一种广为人知的病症。原发性皮肤淀粉样变性通常凭借良好的临床敏锐度、典型的临床表型及受累部位得以诊断。皮肤镜已被用作辅助性非侵入性工具,以确诊诊断存疑的病例以及那些推迟活检的病例。PCA典型的皮肤镜特征有助于将其与其他色素性皮肤病区分开来,避免不必要的侵入性活检及检查,尤其是在资源匮乏且经济受限的环境中。
本研究旨在临床识别并确证42例PCA患者典型的皮肤镜特征,这些患者主要来自东南亚某国一家公立医院的有色人种患者,包括斑状淀粉样变性(MA)和丘疹性淀粉样变性(PA)。
选取具有PCA典型临床特征的患者。原发性皮肤淀粉样变性被细分为MA或PA,并分别列出其相应的临床确证皮肤镜特征。纳入所有同意参与研究的患者(未经治疗及先前接受过治疗的)。患者根据典型的临床特征进行诊断。使用DermLite III DL3N偏振光和配备PigmentBoost品牌的液体皮肤镜(3Gen,DermLite LLC,美国加利福尼亚州圣胡安卡皮斯特拉诺)进行皮肤镜检查,并通过可选连接套件(红米Note 11,MIUI版本13.0.5,中国)连接配备摄像头的移动设备获取图像,以创建数字皮肤镜系统,同时记录检查结果。
在这项PCA皮肤镜检查结果的研究中,对42例患者的临床皮损及其与皮肤镜特征的相关性进行了评估。30例患者出现斑状淀粉样变性,12例患者具有PA典型的皮肤表型和皮肤镜特征。MA患者最常见的皮肤镜表现为中央呈亮白色至暗白色、圆形或椭圆形的中心核,周围环绕着浅棕色点状晕圈。中心核周围褐色色素沉着最常见的形态为细条纹型。在一些MA病例中还可见到小汗腺线索,这是一个独特的发现。同样,在PA亚型中,中心核被类似瘢痕的无结构半透明白色区域所取代,周围环绕着棕黑色点状结构,尤其是在那些斑块大且厚的患者中。
PCA的皮肤镜检查结果及其临床确证对于治疗色素性疾病患者以及有色人种患者来说是一个急需的方面,尤其是在发展中国家。在低收入国家的临床环境以及公立医院中使用皮肤镜将减轻诸如活检等侵入性诊断方式以及为排除色素性疾病而进行的其他不必要检查所带来的额外经济负担。