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A Persistent Dark Macule on the Hand of a Hispanic Patient. 一位西班牙裔患者手部的顽固黑斑。
Fanny Cecilia Cordero-Martinez, Adrian Cuellar-Barboza, Jorge Ocampo-Candiani

A 38-year-old Hispanic man without comorbidities presented to our dermatology clinic for the evaluation of an asymptomatic dark macule on his left hand, which had gradually grown since he was a child. The hyperpigmentation involved the dorsum and palm (Figure 1). The patient was right-handed and denied previous trauma, inflammation, occupational exposure to chemicals, or using any medications. During physical examination, no other similar pigmentation was found on the rest of his body. An incisional biopsy of the left palm was performed (Figure 2). The histopathology revealed the presence of spindle-shaped cells with melanin granules in the superficial and middle dermis, surrounding the blood vessels, and between collagen bundles, which are findings compatible with acquired dermal melanocytosis (1,2). On dermoscopy, we found a pattern of regular pigment with a gray-brown tone and whitish spots within. We discussed the benignity of this rare entity with the patient, and he decided not to pursue treatment. Acquired dermal melanocytosis (ADM) is a rare condition, with isolated presentation on the hand and with less than 10 cases reported (1). Dermal melanocytosis includes several benign pigmented lesions histologically characterized by the presence of melanocytes in the dermis, which are spindle-shaped dendritic cells containing brown melanin pigment. Melanocytes can also be identified with immunoperoxidase staining for S100 and Fontana-Masson melanin stain (2). The physiopathology of ADM remains unclear, but it has been proposed that it involves reactivation of latent dermal melanocytes due to external factors such as trauma, inflammation, chemical exposure, sunlight, drugs, and hormonal treatment with estrogen and/or progesterone (3). ADM with hand involvement usually appears in the Asian population without sex predilection. The lesions develop in adolescence or young adulthood and tend to affect both hands and other body areas such as the face or the legs; there have also been two reported cases in the Hispanic population (both by Fitzpatrick III) (3,4). ADM must be differentiated from ectopic Mongolian spots, plaque-type blue nevi, tinea nigra, or other pigmented neoplasms. A biopsy is mandatory to establish a proper diagnosis. Ectopic Mongolian spots and plaque-type blue nevi are both congenital dermal melanocytoses that may present as bluish macules on the hand. However, these lesions show deep and more widely scattered distribution of melanocytes (1). There have also been some reports of malignant melanoma and acquired dermal melanocytosis that appeared on congenital nevus spilus (5). ADM is a benign condition, and reassurance should be offered to these patients.

一名无合并症的 38 岁西班牙裔男子到我院皮肤科就诊,他的左手上有一个无症状的深色斑丘疹,自孩提时代起就逐渐增大。色素沉着涉及手背和手掌(图 1)。患者是右撇子,否认曾有外伤、炎症、职业性接触化学物质或使用任何药物。体格检查时,在他身体的其他部位没有发现类似的色素沉着。医生对患者的左手掌进行了切口活检(图 2)。组织病理学检查发现,在真皮浅层和中层、血管周围以及胶原束之间存在带有黑色素颗粒的纺锤形细胞,这些结果与获得性真皮黑色素细胞增多症相符(1,2)。在皮肤镜检查中,我们发现了一种灰褐色调的规则色素斑,内部有白色斑点。我们与患者讨论了这种罕见病的良恶性,他决定不再继续治疗。获得性真皮黑素细胞增多症(ADM)是一种罕见病,仅在手部出现,目前报道的病例不到 10 例(1)。真皮黑素细胞增多症包括几种良性色素病变,组织学特征是真皮中存在黑素细胞,它们是纺锤形树突状细胞,含有棕色黑素。黑色素细胞也可通过 S100 免疫过氧化物酶染色法和 Fontana-Masson 黑色素染色法鉴别出来(2)。ADM 的生理病理尚不清楚,但有人认为它涉及潜伏的真皮黑色素细胞因创伤、炎症、化学暴露、日光、药物和使用雌激素和/或孕酮的激素治疗等外部因素而重新激活(3)。手部受累的 ADM 通常出现在亚洲人群中,无性别偏好。病变发生在青春期或青年期,往往累及双手和其他身体部位,如面部或腿部;在西班牙裔人群中也有两例报道(均为菲茨帕特里克三世病例)(3,4)。ADM 必须与异位蒙古斑、斑块型蓝痣、黑色素痣或其他色素性肿瘤相鉴别。要确定正确的诊断,必须进行活检。异位蒙古斑和斑块型蓝痣都是先天性真皮黑素细胞增多症,可能表现为手部淡蓝色斑丘疹。不过,这些病变的黑素细胞分布较深且较分散(1)。也有一些关于恶性黑色素瘤和后天性真皮黑素细胞增多症的报道,它们出现在先天性黑痣上(5)。ADM 是一种良性病变,应向这些患者提供安慰。
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引用次数: 0
Skin Changes in Suspected Lyme Disease. 疑似莱姆病的皮肤变化。
Pero Vržogić, Ante Perica

Dear Editor, Ticks carry many diseases, bacteria, and viruses and represent a very important healthcare issue both in Croatia and globally. Although most ticks are not infected with pathogens dangerous to humans, some ticks can transmit infectious diseases with significant morbidity and mortality. This is caused by the increasing incidence of many tick-borne diseases over a growing geographical area. Many factors influence which species of ticks are present in a given geographical area, as well as the density of their population and the risk of human exposure to infected ticks. The average morbidity from Lyme borreliosis in the Republic of Croatia is 6.51 infected per 100,000 inhabitants. There can be no Lyme borreliosis without ticks infected by Borrelia burgdorferi (1,2). In Europe, Lyme borreliosis (LB) is caused by the Borrelia burgdorferi sensu lato complex genotype. There are three skin manifestations of LB: erythema migrans (EM), borrelial lymphocytoma (BL), and acrodermatitis chronica atrophicans (ACA) (3,4). Herein we describe a female patient with a diagnosis of Lyme disease based on the non-specific clinical picture and laboratory diagnostics, in whom successful treatment led to complete regression of all skin manifestations. The patient was a 58-year-old woman with no previous history of severe illness. Notably, the patient history showed that, eight months prior to presenting for the dermatological exam, the patient had observed the appearance of edema and demarcated macular exanthema around both ankles and subsequently on the dorsum of the right hand, which spread to the left hand and with gradual spread to both lower legs and the lower extremities, with more pronounced changes on the left leg. The initial dermatological examination found pronounced skin changes on both legs, especially the left leg, with erythematous changes in the form of figurate erythema forming confluences up to the size of a smaller palm; the skin of the left leg was partially mottled with normal turgor and elasticity (Figure 1a and Figure 1b). Inguinal lymph nodes were enlarged and painless on palpation. Changes were minimal and discrete on the right leg and were absent on the torso, upper extremities, and skin. Subjectively, there was no itching, burning, or tingling sensation in the affected areas of the skin. The patient subjectively reported feeling well. Family history showed that the patient's father had died from prostate cancer and that the mother had died from melanoma. Laboratory findings were as follows: hematological, biochemical, and immunological parameters were normal. Venous and arterial ultrasound of both legs was normal, with the presence of reactively enlarged left inguinal lymph nodes. Lyme disease was suspected based on the clinical picture, with a differential diagnosis of possible livedo reticularis. A biopsy of the skin changes was also performed, with the results showing that the histological picture in the examined material cou

亲爱的编辑,蜱虫携带多种疾病、细菌和病毒,在克罗地亚和全球都是一个非常重要的卫生保健问题。虽然大多数蜱虫不会感染对人类有害的病原体,但有些蜱虫会传播传染性疾病,造成严重的发病率和死亡率。这是因为许多蜱传疾病的发病率在不断增长。影响特定地区蜱虫种类、种群密度和人类接触受感染蜱虫风险的因素很多。在克罗地亚共和国,莱姆包虫病的平均发病率为每 10 万居民中有 6.51 人感染。没有受鲍氏包虫病感染的蜱虫,就不会有莱姆病(1,2)。在欧洲,莱姆包虫病(Lyme borreliosis,LB)是由勃氏包柔氏菌(Borrelia burgdorferi sensu lato complex genotype)引起的。莱姆病有三种皮肤表现:迁徙性红斑(EM)、包虫性淋巴细胞瘤(BL)和慢性萎缩性皮炎(ACA)(3,4)。在此,我们描述了一名根据非特异性临床表现和实验室诊断被诊断为莱姆病的女性患者,成功的治疗使她的所有皮肤表现完全消退。患者是一名 58 岁的女性,既往无严重疾病史。值得注意的是,患者病史显示,在接受皮肤科检查的 8 个月前,患者发现双脚踝周围出现水肿和分界不清的斑丘疹,随后右手背出现水肿和分界不清的斑丘疹,水肿和分界不清的斑丘疹扩散到左手,并逐渐扩散到双小腿和下肢,其中左腿的变化更为明显。皮肤科初步检查发现,患者双腿皮肤变化明显,尤其是左腿,红斑变化为无花果状红斑,形成的红斑汇合处可达小手掌大小;左腿皮肤部分斑驳,但韧度和弹性正常(图 1a 和图 1b)。腹股沟淋巴结肿大,触诊无痛。右腿的变化很小且不连续,躯干、上肢和皮肤均无变化。主观上,患处皮肤没有瘙痒、烧灼或刺痛感。患者主观感觉良好。家族病史显示,患者的父亲死于前列腺癌,母亲死于黑色素瘤。实验室检查结果如下:血液学、生化和免疫学指标正常。双腿静脉和动脉超声检查正常,左腹股沟淋巴结反应性肿大。根据临床表现,怀疑是莱姆病,鉴别诊断可能是网状青斑。此外,还对皮肤变化进行了活组织检查,结果显示所检查材料的组织学特征与活组织网状结构病的临时临床诊断相符。此外,还进行了鲍曼不动杆菌特异性 IgM 和 IgG 检测:IgG 呈边缘性,而 IgM 呈阳性,为 218 U/mL。在接下来的三周里,治疗方案中加入了阿莫西林 500 毫克,每天三次。治疗结束后,所有皮肤变化逐渐消退,没有出现新的皮损(图 2a 和图 2b)(图 3a 和图 3b)。在接下来一年对患者的随访中,没有发现类似的皮肤病变复发。在此,我们描述了一例非典型皮肤病变患者,该患者体内存在博氏包柔氏菌抗体,在确诊并接受适当的抗生素治疗后,所有皮肤病变均已消退。莱姆病的临床表现范围很广,一般可分为三个阶段:早期局部阶段、早期播散阶段和疾病晚期阶段。不过,不同阶段也有可能重叠,甚至晚期没有任何早期阶段的症状和体征。局部早期。以皮肤变化为特征--迁徙性红斑(EM)--通常在蜱虫叮咬后一个月内(通常是叮咬后 7-14 天)出现(图 4 和图 5)。约有 80% 的患者会出现 EM,但只有 25% 的患者能回忆起被蜱虫叮咬的情景。皮肤病变通常发生在腋窝、腹股沟、肘窝或腰部周围。病变一般不痛,但会发痒或触摸时发热。它们会在数天或数周内逐渐扩散,生长半径可达 20 厘米。起初,皮肤的颜色会在几天内保持一致,之后围绕中心区域的红晕会消失(4-6)。多种皮肤变化是螺旋体血症的表现,而不是多次蜱虫叮咬的结果。
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引用次数: 0
Revive the Dennie-Morgan Fold: A Forgotten Sign of Atopic Dermatitis in Children. 重现丹尼-摩根皱褶:被遗忘的儿童特应性皮炎体征
Rohan Shah, William C Lambert, Robert A Schwartz

In 1980, Hanifin and Rajka (1) proposed major and minor diagnostic criteria for atopic dermatitis (AD). Major associations included pruritus, dry skin, and history of atopy. One minor feature included Dennie-Morgan Folds (DMFs), which manifest as secondary creases in the skin underneath the inferior eyelid, usually found in infants (2). In an attempt to refine these criteria, a study evaluating 210 patients with an existing AD diagnosis observed DMF in 84% of AD cases. A pediatric study in Bijapur, India on 174 children under 16 years of age with AD identified DMF to be the most prevalent minor criterion. DMFs were found in 71.8% of the study's population and was followed by palmar hyperlinearity and xerosis in prevalence (2). Although DMF pathophysiology remains unclear, we suggest a theory stemming from nocturnal pruritus (NP). The two leading causes of NP are AD and psoriasis, both of which interfere with the patient's sleep quality. Children with increased NP have greater sleep fragmentation and difficulty waking up in the morning (3). A lack of melatonin rhythmic secretions resulting in circadian misalignment may serve as an intermediary for DMF onset. As more blood perfuses the skin under the eyes, edematous fluid enhances dysregulation of the collagen fibers under the eyelids. NP also manifests as facial touching and rubbing or scratching the eyelids during sleep, which can aggravate tissue surrounding the eye (3). AD affects 15-20% of the pediatric population, whereas food allergies, some life-threatening, are found in up to 30% of children with AD, compared with 0.1-0.6% of children in the general population (2). Identifying DMF in children can facilitate the diagnosis of AD and thus lead to the necessary tests to determine whether conditions associated with AD exist, such as food allergy. DMFs are indicative of an AD diagnosis and can be especially critical for children who have life-threatening food allergies associated with their AD (3). One challenge in using DMF as a marker for AD are its different manifestations across ethnic and racial groups. In a study of 160 children aged 3-11 years in London, England, DMFs were present in 34/69 children classified as "black", regardless of whether the child presented with AD. In children classified as "white", only 11/44 had DMFs regardless of AD diagnosis. When cases of AD were excluded, 25% of the white children and 49% of black children had DMFs (4). In a separate study evaluating the differences in prevalence of AD minor characteristics between African Americans and European Americans, African Americans were more likely to have extensor involvement along with diffuse xerosis, palmar hyperlinearity, and DMFs (5). Furthermore, in individuals with darker skin tones, DMFs may be of greater importance, as other characteristics such as erythema may be harder to recognize (5). Life-threatening food allergies are rare, but represent serious sequelae associated with AD. DMFs can serve as a cr

1980 年,Hanifin 和 Rajka(1)提出了特应性皮炎(AD)的主要和次要诊断标准。主要特征包括瘙痒、皮肤干燥和过敏史。一个次要特征包括 Dennie-Morgan 皱褶(DMFs),表现为下眼睑下方皮肤的继发性皱褶,通常出现在婴儿身上(2)。为了完善这些标准,一项对 210 名已确诊为注意力缺失症的患者进行评估的研究发现,84% 的注意力缺失症病例存在 DMF。印度比贾布尔的一项儿科研究对174名16岁以下的AD患儿进行了调查,结果发现DMF是最常见的次要标准。研究发现,71.8%的研究对象存在DMF,其次是掌纹过长和干燥症(2)。尽管 DMF 的病理生理学尚不清楚,但我们提出了一种源自夜间瘙痒(NP)的理论。引起夜间瘙痒的两个主要原因是注意力缺失症和银屑病,这两种疾病都会影响患者的睡眠质量。夜间瘙痒症加重的儿童睡眠碎片更多,早晨起床困难(3)。缺乏褪黑激素节律性分泌导致昼夜节律失调可能是 DMF 发病的中间因素。由于更多的血液灌注到眼下的皮肤,水肿的液体加剧了眼睑下胶原纤维的失调。NP还表现为面部触摸、睡眠时揉搓或抓挠眼睑,这会使眼睛周围的组织恶化(3)。15%-20%的儿童患有注意力缺失症,而高达30%的注意力缺失症患儿对食物过敏,其中有些会危及生命,而普通人群中对食物过敏的儿童仅占0.1%-0.6% (2)。识别儿童 DMF 有助于 AD 的诊断,从而进行必要的检查以确定是否存在与 AD 相关的疾病,如食物过敏。DMF 是 AD 诊断的指示性指标,对于那些因食物过敏而导致 AD 并危及生命的儿童尤为重要 (3)。将DMF作为AD的标志物所面临的一个挑战是,不同民族和种族群体的DMF表现各不相同。在一项针对英国伦敦 160 名 3-11 岁儿童的研究中,34/69 名被归类为 "黑人 "的儿童出现了 DMF,无论他们是否出现 AD。在被归类为 "白人 "的儿童中,无论是否诊断出注意力缺失症,只有11/44的儿童有DMFs。如果排除注意力缺失症病例,25%的白人儿童和49%的黑人儿童有DMFs(4)。另一项研究评估了非裔美国人和欧裔美国人在注意力缺失症次要特征患病率上的差异,结果显示,非裔美国人更有可能出现伸肌受累、弥漫性角化病、掌纹过度线性和 DMFs(5)。此外,对于肤色较深的人来说,DMFs 可能更为重要,因为红斑等其他特征可能更难识别(5)。危及生命的食物过敏并不多见,但却代表着与 AD 相关的严重后遗症。DMF 可以作为 AD 的一个非常简单、易于识别的标记,或许可以在出现严重后遗症之前识别出病情。DMFs可以指导临床医生进一步询问其他与AD相关的症状,从而改善对重要儿科疾病的临床评估。
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引用次数: 0
Mycosis fungoides with large cell transformation (CD30+) and B-cell chronic lymphocytic leukemia. 真菌病伴大细胞转化(CD30+)和 B 细胞慢性淋巴细胞白血病。
Mikela Petković, Ivana Ilić, Ružica Jurakić Tončić, Ivo Radman-Livaja, Romana Čeović

Mycosis fugnoides (MF) is an indolent cutaneous T-cell lymphoma (CTLC) and is the most common of all cutaneous lymphomas. An increased risk for developing a second primary malignancy in patients with CTCL has been described in several studies, with a range from 1.04 to 2.4 (1-4). Caucasian males are at higher risk for MF development. MF is often diagnosed at ages between 55 and 67 years, and second malignancy usually occurs 5 or 6 years after the diagnosis of MF was established (5). The most common second primary malignancies include non-Hodgkin lymphoma (NHL), Hodgkin lymphoma (HL), lung carcinoma, bladder carcinoma, and melanoma. Even though a higher incidence rate of all NHL was described in patients with MF (15/1000) in comparison with the general population (0.32/1000), there are still only a few cases of B-cell NHL following MF described in the literature (6,7). We describe a rare case of a patient with MF and simultaneous large cell transformation (LCT) and a small B-cell lymphocytic lymphoma/chronic lymphocytic leukemia (B-CLL). In 2017, an 82-year-old man previously treated for MF presented with two fast growing tumorous lesions with ulceration on the right tight (Figure 1). A biopsy was performed, and a diagnosis of MF with LCT was established (Figure 2). During hospitalization, mild leukocytosis (12.2 x109 L-1), lymphocytosis (64%, total count of 7.81 x109 L-1), and anemia were found. Bone marrow biopsy was not performed due to low pain threshold. Bone marrow aspirate showed 70% of atypical lymphocytes and few "smudged" cells. Immunophenotyping by flow cytometry detected 49% monoclonal kappa+ B-cells with phenotypic features typical for B-CLL (CD5+, CD23+, kappa +). Of overall bone marrow cells, the ratio of monoclonal kappa + B-cells with the B-CLL phenotype was 21%. Immunophenotyping of peripheral blood showed up to 50% monoclonal kappa+ B-cells with phenotypic features typical for B-CLL (CD5+, CD23+, kappa +). Of overall peripheral blood cells, the ratio of monoclonal kappa+ B-cells with the B-CLL phenotype was 28%. Multi-sliced computed tomography was within normal ranges. A flow cytometry showed lymphocytes with phenotypic findings for CD20+ B-CLL. A diagnosis of MF with LCT (CD30+) clinical grade IIB (T3, N0, M0) and B-CLL was established. The patient was treated with fractionated superficial irradiation that resulted in applanation and regression of the tumorous lesions. No hematologic treatment was indicated other than regular follow-up. On dermatologic follow up for 2 years, the patient was stable, with no active skin lesions and no progression of MF. The patient was subsequently lost to follow-up. This is a rare case of MF with LCT and B-CLL occurring simultaneously. Large cell transformation in patients with MF can occur in 20-55% of advanced MF, as in our case, and this something physicians must be aware of, so repeated biopsies are advised (8). We also should keep in mind that patients with MF are at higher risk of d

布氏杆菌病(MF)是一种不活跃的皮肤 T 细胞淋巴瘤(CTLC),是所有皮肤淋巴瘤中最常见的一种。多项研究表明,CTCL 患者罹患第二种原发性恶性肿瘤的风险增加,范围从 1.04 到 2.4 (1-4)。白种男性罹患 MF 的风险更高。骨髓纤维瘤的确诊年龄通常在 55 至 67 岁之间,第二次恶性肿瘤通常发生在骨髓纤维瘤确诊后的 5 或 6 年(5)。最常见的第二原发恶性肿瘤包括非霍奇金淋巴瘤(NHL)、霍奇金淋巴瘤(HL)、肺癌、膀胱癌和黑色素瘤。尽管与普通人群(0.32/1000)相比,MF 患者所有 NHL 的发病率更高(15/1000),但文献中关于 MF 后 B 细胞 NHL 病例的描述仍然寥寥无几(6,7)。我们描述了一例罕见的MF患者,他同时患有大细胞转化(LCT)和小B细胞淋巴细胞淋巴瘤/慢性淋巴细胞白血病(B-CLL)。2017年,一名曾接受过MF治疗的82岁男性患者出现了两个快速生长的肿瘤病灶,右侧紧贴处伴有溃疡(图1)。经活检,确诊为 MF 伴 LCT(图 2)。住院期间发现轻度白细胞增多(12.2 x109 L-1)、淋巴细胞增多(64%,总计数为 7.81 x109 L-1)和贫血。由于疼痛阈值较低,没有进行骨髓活检。骨髓穿刺显示 70% 的非典型淋巴细胞和少量 "污点 "细胞。通过流式细胞术进行免疫分型,发现49%的单克隆kappa+ B细胞具有B-CLL的典型表型特征(CD5+、CD23+、kappa+)。在所有骨髓细胞中,具有 B-CLL 表型的单克隆 kappa + B 细胞比例为 21%。外周血免疫分型显示,具有 B-CLL 典型表型特征(CD5+、CD23+、kappa +)的单克隆 kappa + B 细胞高达 50%。在所有外周血细胞中,具有 B-CLL 表型的单克隆 kappa+ B 细胞比例为 28%。多切片计算机断层扫描结果在正常范围内。流式细胞术显示淋巴细胞表型为 CD20+ B-CLL。最终确诊为中频伴LCT(CD30+)临床分级IIB(T3,N0,M0)和B-CLL。患者接受了分次表皮照射治疗,结果肿瘤病灶缩小并消退。除定期随访外,没有进行血液学治疗。皮肤科随访 2 年,患者病情稳定,没有活动性皮损,MF 也没有进展。随后,患者失去了随访机会。这是一例罕见的同时发生大细胞癌和 B-CLL 的 MF 病例。20%-55% 的晚期 MF 患者会发生大细胞变异,就像我们的病例一样,医生必须注意这一点,因此建议反复进行活检(8)。我们还应该记住,MF 患者罹患第二种恶性肿瘤的风险较高。在这些第二种恶性肿瘤中,T 细胞和 B 细胞两系淋巴细胞增生性疾病(如 CTCL 和 B-CLL)并存的情况非常少见,目前仅有少数病例发表(6,7,10)。在大多数病例中,CTCL 发生在 B-CLL 之前,唯一确定的解释是 CTCL 患者发生第二次恶性肿瘤的风险增加(3,5,10)。其他解释性假说包括肿瘤干细胞、恶性遗传倾向、使用免疫抑制剂治疗首次肿瘤、病毒制剂以及单克隆 T 细胞增殖对 B 细胞系统的调节(1,5,6,9,10)。对所有 CTCL 和 MF 患者都必须进行定期随访,以确定疾病的进展情况,并及时发现二次恶性肿瘤。
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引用次数: 0
Upregulation of Anti-Desmocollin 3 Antibodies in Pemphigus Diseases: A Case-control Study. 天疱疮疾病中抗去疱疹素 3 抗体的上调:病例对照研究
Marwah Adly Saleh, Rana Mussa Said, Laila Ahmed Ahmed Rashed, Mona El-Kalioby

Background: Pemphigus diseases are a subgroup of autoimmune bullous diseases characterized by autoantibodies against desmogleins and occasionally desmocollins. Desmocollin 3 is the main desmocollin isoform that contributes to cell adhesion in the epidermis.

Objective: To evaluate the presence and level of anti-desmocollin 3 antibodies in pemphigus diseases, and to investigate whether their presence is associated with a specific type, presentation, or clinical pattern.

Methods: Forty patients with pemphigus diseases and forty healthy controls were enrolled. Medical history, clinical examination, and pemphigus disease area index (PDAI) scoring were recorded for all patients. Serum samples were collected from both groups for assessment of anti-desmocollin 3 antibody reactivity by ELISA.

Results: The presence of anti-desmocollin 3 antibodies was significant among patients with pemphigus compared with controls (P=0.003). The level of anti-desmocollin 3 antibodies was also significantly higher in patients with pemphigus compared with controls (P=0.01). There was no significant relationship between the presence of anti-desmocollin 3 antibodies and any of the clinical presentations of pemphigus (type, severity, duration, activity, presence of annular pattern, or site of affection - mucosal, cutaneous, on the scalp, palmoplantar, or flexural).

Conclusion: Anti-desmocollin 3 antibodies are upregulated in pemphigus diseases and can contribute to the pathogenesis of pemphigus. No specific clinical type, presentation, or pattern was found to be associated with the presence of anti-desmocollin 3 antibodies.

背景:丘疹性荨麻疹是自身免疫性大疱性皮肤病的一个亚组,其特征是针对去疱疹素(desmogleins)和偶尔针对去疱疹素(desmocollins)的自身抗体。去鳞屑球蛋白 3 是去鳞屑球蛋白的主要同工型,有助于表皮中的细胞粘附:评估丘疹性荨麻疹疾病中抗去鳞屑蛋白 3 抗体的存在和水平,并研究其存在是否与特定类型、表现或临床模式有关:方法:40 名丘疹性荨麻疹患者和 40 名健康对照者。记录所有患者的病史、临床检查和丘疹性荨麻疹疾病面积指数(PDAI)评分。采集两组患者的血清样本,用 ELISA 方法评估抗去疱疹病毒 3 抗体的反应性:结果:与对照组相比,丘疹性荨麻疹患者的抗去疱疹素 3 抗体存在显著差异(P=0.003)。与对照组相比,丘疹性荨麻疹患者的抗去疱疹素 3 抗体水平也明显更高(P=0.01)。抗去甲斑蝥素 3 抗体的存在与丘疹性荨麻疹的任何临床表现(类型、严重程度、持续时间、活动性、环状模式的存在或发病部位--粘膜、皮肤、头皮、掌跖或挠侧)之间均无明显关系:结论:抗去疱疹素 3 抗体在丘疹性荨麻疹中上调,可能是丘疹性荨麻疹的发病机制之一。结论:抗去鳞屑蛋白 3 抗体在丘疹性荨麻疹中上调,可能是丘疹性荨麻疹的发病机制之一。没有发现特定的临床类型、表现或模式与抗去鳞屑蛋白 3 抗体的存在相关。
{"title":"Upregulation of Anti-Desmocollin 3 Antibodies in Pemphigus Diseases: A Case-control Study.","authors":"Marwah Adly Saleh, Rana Mussa Said, Laila Ahmed Ahmed Rashed, Mona El-Kalioby","doi":"","DOIUrl":"","url":null,"abstract":"<p><strong>Background: </strong>Pemphigus diseases are a subgroup of autoimmune bullous diseases characterized by autoantibodies against desmogleins and occasionally desmocollins. Desmocollin 3 is the main desmocollin isoform that contributes to cell adhesion in the epidermis.</p><p><strong>Objective: </strong>To evaluate the presence and level of anti-desmocollin 3 antibodies in pemphigus diseases, and to investigate whether their presence is associated with a specific type, presentation, or clinical pattern.</p><p><strong>Methods: </strong>Forty patients with pemphigus diseases and forty healthy controls were enrolled. Medical history, clinical examination, and pemphigus disease area index (PDAI) scoring were recorded for all patients. Serum samples were collected from both groups for assessment of anti-desmocollin 3 antibody reactivity by ELISA.</p><p><strong>Results: </strong>The presence of anti-desmocollin 3 antibodies was significant among patients with pemphigus compared with controls (P=0.003). The level of anti-desmocollin 3 antibodies was also significantly higher in patients with pemphigus compared with controls (P=0.01). There was no significant relationship between the presence of anti-desmocollin 3 antibodies and any of the clinical presentations of pemphigus (type, severity, duration, activity, presence of annular pattern, or site of affection - mucosal, cutaneous, on the scalp, palmoplantar, or flexural).</p><p><strong>Conclusion: </strong>Anti-desmocollin 3 antibodies are upregulated in pemphigus diseases and can contribute to the pathogenesis of pemphigus. No specific clinical type, presentation, or pattern was found to be associated with the presence of anti-desmocollin 3 antibodies.</p>","PeriodicalId":94367,"journal":{"name":"Acta dermatovenerologica Croatica : ADC","volume":"31 4","pages":"178-183"},"PeriodicalIF":0.0,"publicationDate":"2023-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140854959","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Cutaneous T-cell Lymphomas: A Single-center Retrospective Analysis. 皮肤 T 细胞淋巴瘤:单中心回顾性分析
Till Kaemmerer, Anne Guertler, Benjamin M Clanner-Engelshofen, Corbinian Fuchs, Lars Einar French, Markus Reinholz

Introduction: Cutaneous T-cell lymphomas (CTCLs) are rare diseases characterized by infiltration of malignant T-cells into the skin. We evaluated the prevalence, epidemiology, and therapy of CTCLs, focusing on its most well-known subtypes, namely mycosis fungoides (MF) and Sézary syndrome (SS).

Patients and methods: We retrospectively analyzed the medical data of patients with a histologically confirmed diagnosis of CTCL presenting to our outpatient department during a 5-year period from January 2015 to December 2019.

Results: We evaluated the files of 102 patients, of whom 67% were men and 33% women. The overall mean age was 59.1±14.1 (24-86) years. Ninety-two patients (90%) were diagnosed with MF and ten patients (10%) with SS. According to ISCL/EORTC, the majority of patients initially classified as stage IA (34%) and IB (45%). Disease frequency decreased at advanced stages (II: 4%; III: 7%; IV: 10%). Forty-five patients (44.1%) received only skin-directed therapy (SDT). Twenty patients (19.6%) progressed from SDT to systemic therapy (ST). Thirty-seven patients (36.3%) received ST combined with SDT (TS) from the start of treatment. Overall, fifty different therapeutic approaches of TS were initiated due to lack of response to therapy or disease progression.

Conclusion: Management of CTCLs aims to maintain patient quality of life while minimizing side-effects. As CTCLs are usually incurable diseases, the focus of treatment is on symptom control and prevention of disease progression. Due to the large patient group and the long observation period, our study allows for a valid evaluation of the frequency and therapy of MF and SS in a university outpatient clinic in Germany. We favor topical therapies in early stages with more invasive therapies in advanced stages.

简介:皮肤 T 细胞淋巴瘤(CTCL皮肤T细胞淋巴瘤(CTCL)是一种罕见疾病,其特征是恶性T细胞浸润皮肤。我们评估了CTCL的发病率、流行病学和治疗方法,重点研究了其最著名的亚型,即真菌病(MF)和塞扎里综合征(SS):我们回顾性地分析了2015年1月至2019年12月这5年间在我院门诊部就诊并经组织学确诊为CTCL的患者的医疗数据:我们评估了102名患者的档案,其中67%为男性,33%为女性。总平均年龄为59.1±14.1(24-86)岁。92名患者(90%)被诊断为 MF,10名患者(10%)被诊断为 SS。根据ISCL/EORTC标准,大多数患者最初分为IA期(34%)和IB期(45%)。疾病发生率在晚期有所下降(II 期:4%;III 期:7%;IV 期:10%)。45 名患者(44.1%)只接受了皮肤导向疗法(SDT)。20名患者(19.6%)从SDT发展到全身治疗(ST)。37名患者(36.3%)从治疗一开始就接受了ST与SDT相结合的治疗(TS)。总的来说,有50种不同的TS治疗方法是由于缺乏治疗反应或疾病进展而启动的:CTCL的治疗旨在维持患者的生活质量,同时最大限度地减少副作用。由于 CTCL 通常是无法治愈的疾病,因此治疗的重点在于控制症状和预防疾病进展。由于患者群体大、观察时间长,我们的研究可以对德国一所大学门诊中 MF 和 SS 的发病频率和治疗方法进行有效评估。我们倾向于在早期阶段采用局部疗法,而在晚期阶段采用更具侵入性的疗法。
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引用次数: 0
The Association Between Hidradenitis Suppurativa and Diet: An Update. 化脓性扁桃体炎与饮食的关系:最新进展。
Marko Belamarić, Joško Miše, Zrinka Bukvić Mokos

Hidradenitis suppurativa (HS) is a chronic inflammatory skin condition characterized by painful inflammatory lesions, predominantly affecting areas of the skin rich in apocrine glands, such as inguinal, axillary, submammary, and anogenital regions, with an estimated global prevalence between 1%-4%. The treatment of HS is challenging with various treatment modalities employed to control the disease. Since the condition is chronic and life-impairing, many patients have looked for ways to complement their conventional treatment procedures with non-medical interventions, among which dietary interventions have been of particular interest. Researchers have looked for ways to connect the gastrointestinal system with the skin through the ˝skin-gut axis concept˝ introducing a strong association between the microbiome of the gastrointestinal system and the skin. In addition, diet stimulation of insulin and IGF-1 (insulin-like growth factor 1) may impact signaling pathways playing a role in HS pathogenesis. Patients have tried various dietary interventions to alleviate their symptoms of inflammation and suppuration. Among the different dietary approaches that have been described are paleo, autoimmune, Mediterranean, and elimination diet regimes. Dietary supplements have become the mainstay of lifestyle factors aimed at improving the clinical signs and symptoms of HS. This review aims to synthesize and present the current findings on diet as a modifiable factor in HS, helping the patients to navigate through the data and helping them make informed choices on their healthy lifestyles.

化脓性扁平湿疹(Hidradenitis suppurativa,HS)是一种慢性炎症性皮肤病,以疼痛性炎症性皮损为特征,主要累及腹股沟、腋窝、乳房下和肛门生殖器等分泌腺丰富的皮肤区域,全球发病率约为 1%-4%。HS的治疗具有挑战性,需要采用各种治疗方法来控制病情。由于该病是一种慢性病,会影响患者的生活,因此许多患者都在寻求用非医疗干预措施来补充常规治疗程序的方法,其中饮食干预措施尤其受到关注。研究人员通过˝皮肤-肠道轴概念˝寻找连接肠胃系统和皮肤的方法,该概念介绍了肠胃系统微生物群与皮肤之间的密切联系。此外,饮食对胰岛素和 IGF-1(胰岛素样生长因子 1)的刺激可能会影响在 HS 发病机制中发挥作用的信号通路。患者曾尝试过各种饮食干预措施来缓解炎症和化脓症状。在已描述的不同饮食方法中,包括古法饮食、自身免疫饮食、地中海饮食和消除性饮食。膳食补充剂已成为改善 HS 临床症状和体征的主要生活方式。本综述旨在总结和介绍目前有关饮食作为 HS 可改变因素的研究结果,帮助患者浏览相关数据,并帮助他们在健康的生活方式方面做出明智的选择。
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引用次数: 0
Platelet-rich Plasma Stimulates Collagen Type I Synthesis in the Human Skin: A Placebo-controlledin vivo Study. 富血小板血浆刺激人体皮肤中 I 型胶原蛋白的合成:安慰剂对照体内研究》。
Paweł Surowiak, Vladimir Tsepkolenko, Romuald Olszański

Platelet-rich plasma (PRP) is used in medicine as a source of autologous growth factors in different indications. At present, PRP is applied increasingly frequently in aesthetic medicine with the aim of skin revitalization. Until now, the mechanisms of PRP effects in healthy human skin treated with aesthetic goals have not been identified in detail. This study aimed to examine PRP effects on the synthesis of procollagen type I in human skin. This study was a prospective, single-center, single-dose, open-label, non-randomized controlled clinical study. The study was conducted on a group of 10 volunteers in whom forearm skin was injected with PRP, while the placebo control group received injections of 0.9% NaCl. Expression of procollagen type I was examined after 21 days using immunohistochemistry. The study demonstrated that skin fragments subjected therapy using PRP demonstrated a significantly higher expression of procollagen than that which was observed in placebo controls. The study demonstrated that PRP stimulated collagen expression in healthy human skin.

富血小板血浆(PRP)作为一种自体生长因子来源,被广泛应用于不同的医学领域。目前,PRP 在美容医学中的应用日益频繁,其目的是活化皮肤。到目前为止,PRP 在健康人皮肤上的作用机制尚未被详细确认。本研究旨在探讨 PRP 对人体皮肤 I 型胶原蛋白合成的影响。本研究是一项前瞻性、单中心、单剂量、开放标签、非随机对照临床研究。研究对象是 10 名志愿者,他们的前臂皮肤注射了 PRP,而安慰剂对照组则注射了 0.9% 氯化钠。21 天后,使用免疫组化方法检测了 I 型胶原蛋白的表达情况。研究表明,使用 PRP 治疗的皮肤碎片的胶原蛋白表达明显高于安慰剂对照组。研究表明,PRP 能刺激健康人皮肤中胶原蛋白的表达。
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引用次数: 0
he Influence of Academician Franjo Kogoj on Global Dermatology. Franjo Kogoj 院士对全球皮肤病学的影响。
Tomislav Duvančić, Mirna Šitum

Academician Franjo Kogoj graduated medicine in 1920 in Prague, where he then pursued training in dermatovenerology. During later years, he also visited other dermatology clinics in Europe, where he collaborated with renowned dermatologists of the time, such as in Breslau (present day Wroclaw in Poland) with Josef Jadassohn and in Strasbourg with Lucien-Marie Pautrier. He was also active in the famous Saint-Louis hospital in Paris. Academician Kogoj's scientific interests were especially focused on allergies, exanthemas, skin tuberculosis, and keratodermas. Kogoj was very active in defining a precise and useful terminology for various dermatological conditions, where the terminology was in many ways confusing and often overlapping, such as in cases of eczema and dermatitis. Kogoj performed experimental studies of allergic reactions in eczema and atopic dermatitis and introduced the term pruridermatitis (Pruridermatitis allergica chronica) into dermatological terminology instead of the name neurodermitis and other synonyms essentially describing atopic dermatitis (endogenous eczema, prurigo-asthma, prurigo Besnier). Academician Kogoj managed to define Mal de Meleda as a separate form of hereditary keratoderma and was engaged in the clinical symptomatology, serology, and therapy of syphilis, whereby he emphasized the so-called "critical moment" in the treatment of syphilis. Academician Kogoj's most famous scientific achievement was his histological definition of the spongiform pustule in the pathomorphology of psoriasis, which became a groundbreaking histological novelty in the classification of psoriasis, thus bearing Kogoj's name in the medical literature to this date. Academician Kogoj published many scientific and professional articles, books, monographs and contributions to manuals and textbooks. He was honored nationally as well as internationally as a leading expert in the field of medicine and dermatology, receiving many eminent awards and recognitions throughout his scientific career.

弗兰霍-科戈伊院士于 1920 年在布拉格获得医学学位,随后在那里接受了皮肤病学培训。在随后的几年里,他还访问了欧洲其他皮肤病诊所,与当时著名的皮肤病专家合作,如在布雷斯劳(今波兰弗罗茨瓦夫)与约瑟夫-雅达松合作,在斯特拉斯堡与卢西恩-玛丽-保特里尔合作。他还活跃在巴黎著名的圣路易医院。科戈伊院士的科研兴趣主要集中在过敏症、外感病、皮肤结核和角化病方面。科戈日非常积极地为各种皮肤病定义精确而有用的术语,因为这些术语在很多方面都很混乱,而且经常重叠,例如湿疹和皮炎。Kogoj 对湿疹和特应性皮炎的过敏反应进行了实验研究,并将瘙痒性皮炎(Pruridermatitis allergica chronica)这一术语引入皮肤病学术语中,取代了神经性皮炎(neurodermitis)和其他描述特应性皮炎的同义词(内源性湿疹、瘙痒性哮喘、prurigo Besnier)。科戈日院士成功地将 Mal de Meleda 定义为遗传性角化病的一种独立形式,并从事梅毒的临床症状学、血清学和治疗学研究,强调梅毒治疗中所谓的 "关键时刻"。科戈伊院士最著名的科学成就是他在银屑病病理形态学中对海绵状脓疱的组织学定义,这成为银屑病分类中一个开创性的组织学新发现,从而使科戈伊的名字在医学文献中留名至今。科戈吉院士发表了许多科学和专业文章、书籍、专著,并为手册和教科书撰稿。作为医学和皮肤病学领域的权威专家,他在整个科学生涯中获得了许多杰出的奖项和表彰,在国内和国际上都享有盛誉。
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引用次数: 0
Vitiligo as a First Sign of Vogt-Koyanagi-Harada Disease. 白癜风是 Vogt-Koyanagi-Harada 病的首发症状。
Marija Vukojević, Nenad Vukojevic, Ante Vuković, Borna Rupčić, Mislav Blažević, Ante Blažević

Vogt-Koyanagi-Harada (VKH) disease is a multisystem disorder characterized by bilateral granulomatous panuveitis resulting in serous retinal detachments, disk edema, and a sunset glow fundus development. Furthermore, it is associated with various extraocular findings, such as tinnitus, hearing loss, vertigo, poliosis, and vitiligo (1). VKH is considered to be an autoimmune disease mediated by T-cells targeting melanocyte antigen tyrosinase peptide (2). Moreover, VKH more often occurs in individuals with a genetic predisposition to the disease, including those of Asian and Hispanic heritage (3). Three disease categories have been recognized, including complete, incomplete, and probable VKH. Each category has different clinical features, varying from neurological and auditory manifestations to ophthalmologic and dermatologic findings (1). Herein, we present a case of chronic complete Vogt-Koyanagi-Harada disease, which started with vitiligo. CASE REPORT A forty-year-old female patient presented to the Department of Ophthalmology with photophobia, dull eye pain, and a gradual decrease in visual acuity over two months. In addition, at clinical examination, vitiligo spots were observed on the patient's hands and the periocular area. The patient's medical history revealed she had vitiligo from a young age. Additionally, she developed generalized epilepsy and headaches in adolescence. The neurologic symptoms had been treated, whereas dermatologic workup and treatment were never performed. It was also found that our patient was of Hispanic heritage, which later helped establish a diagnosis. Ophthalmologic examination revealed eye redness, hypotony, keratic precipitates, anterior chamber cells, and posterior synechiaes. Fundoscopy showed mild vitreous haze, optic disc and macular edema, chorioretinal thickening (also seen on eye ultrasound), and disturbance of retinal pigment epithelium (Figure 1). A standard diagnostic protocol for uveitis was performed. Serology for infectious causes was performed, and IgG for CMV and HSV 1 were positive. Tuberculosis testing was negative. HLA testing showed positive HLA-DR1, HLA B13/18, and HLA DQ-1 antigens. There were no cells in the intraocular fluid, and PCR of the fluid was negative for CMV and HSV 1 and 2. Considering the noninfectious uveitis, a history of neurological and dermatological disorders, and the Hispanic heritage of our patient, the diagnosis of Vogt-Koyanagi-Harada disease was established. Systemic methylprednisone in a 1.5 mg/kg dose was introduced during the first hospitalization. After slow tapering of the corticosteroid therapy, cyclosporine A in a 175 mg/day dose and azathioprine in a 100 mg/day dose were introduced for prolonged therapy. Although signs of eye inflammation were reduced, poor prognostic signs such as hypotony and optic disc edema were persistent. Therefore, the TNF-α inhibitor adalimumab was introduced. After the introduction of adalimumab, the disease was considered stable

Vogt-Koyanagi-Harada(VKH)病是一种多系统疾病,其特征是双侧肉芽肿性泛葡萄膜炎导致浆液性视网膜脱离、视盘水肿和日落辉光眼底发育。此外,它还伴有各种眼外症状,如耳鸣、听力下降、眩晕、脊髓灰质炎和白癜风 (1)。VKH 被认为是一种由靶向黑色素细胞抗原酪氨酸酶肽的 T 细胞介导的自身免疫性疾病(2)。此外,VKH 多发生在有遗传倾向的人身上,包括亚洲人和西班牙裔人(3)。目前已确认有三种疾病类别,包括完全性、不完全性和疑似 VKH。每种类型都有不同的临床特征,从神经系统和听觉表现到眼科和皮肤科发现都不尽相同(1)。在此,我们将介绍一例慢性完全性 Vogt-Koyanagi-Harada 病,该病以白癜风起病。病例报告 一位四十岁的女性患者因畏光、眼睛钝痛、视力在两个月内逐渐下降而到眼科就诊。此外,临床检查发现,患者的手部和眼周出现了白癜风斑点。病史显示,患者自幼患有白癜风。此外,她还在青春期患上了全身性癫痫和头痛。神经系统症状已经得到治疗,但皮肤科检查和治疗却从未进行过。我们还发现患者是西班牙裔,这有助于后来确诊。眼科检查发现患者眼睛发红、眼压过低、角膜沉淀物、前房细胞和后巩膜瘤。眼底镜检查显示轻度玻璃体混浊、视盘和黄斑水肿、脉络膜增厚(在眼部超声波检查中也能看到)以及视网膜色素上皮紊乱(图 1)。对葡萄膜炎进行了标准诊断。进行了传染病血清学检查,CMV 和 HSV 1 IgG 呈阳性。肺结核检测呈阴性。HLA检测显示HLA-DR1、HLA B13/18和HLA DQ-1抗原阳性。眼内液中没有细胞,眼内液的 PCR 检测结果为 CMV 和 HSV 1 和 2 阴性。考虑到患者患有非感染性葡萄膜炎、神经系统和皮肤病病史以及西班牙裔血统,Vogt-Koyanagi-Harada 病的诊断成立。在第一次住院期间,患者开始全身使用甲基强的松,剂量为 1.5 毫克/千克。在缓慢减少皮质类固醇治疗后,又开始使用环孢素 A(175 毫克/天)和硫唑嘌呤(100 毫克/天)进行长期治疗。虽然眼部炎症症状有所减轻,但低眼压和视盘水肿等预后不良的症状仍持续存在。因此,患者开始使用 TNF-α 抑制剂阿达木单抗。使用阿达木单抗后,患者的病情趋于稳定,视功能没有恶化,但白癜风斑点仍在继续发展(图 2)。讨论 我们的病例显示了一位西班牙裔患者的慢性 Vogt-Koyanagi-Harada 病。VKH 是一种罕见的自身免疫性疾病,累及多个器官系统,包括眼睛、皮肤、听觉和神经系统。在该病的发病机制中,存在一种由针对黑色素细胞特异性抗原的 T 淋巴细胞介导的潜在肉芽肿性炎症(4)。除免疫反应外,遗传也是该病病因的一个组成部分。HLA-DR1 和 HLA-DR4 与 VKH 疾病相关,特别是在西班牙裔和亚洲人群中(3,5)。其他研究发现,与白种人或非裔美国人相比,VKH 在亚裔和西班牙裔人群中更为常见(6)。在我们的病例报告中,患者的西班牙裔血统对疾病的诊断至关重要。VKH 疾病分为四个阶段。前驱期持续数天至数周,以头痛、眩晕、脑膜炎和恶心等眼外症状为特征(1)。前驱期过后是急性葡萄膜炎期,患者会突然出现视力模糊、结膜注射和畏光等症状(1,7)。数周至数月后进入恢复期,出现色素脱失症状,如白癜风、多形性白癜风和眼球边缘区白癜风,称为杉浦征。最后,在初期症状出现 6 至 9 个月后,进入慢性复发期,导致前葡萄膜炎加重(1)。尽管大多数患者在康复期会出现皮肤变化,但我们的患者在眼部受累前几年就出现了皮肤色素沉着。VKH 可以是完全性、不完全性或可能的。 我们的患者就是完全性 VKH 的一个例子,因为她符合完全性 VKH 的所有标准,包括:1)无眼部穿透性外伤或手术史;2)无其他眼部疾病的临床或实验室证据;3)双侧眼球受累;4)神经系统检查结果;5)全身检查结果(8)。VKH 的治疗包括口服或静脉注射大剂量全身性皮质类固醇激素,然后缓慢减量口服皮质类固醇激素。如果症状持续或恶化,可考虑使用环孢素和/或硫唑嘌呤进行免疫抑制治疗。如果症状没有改善,还可使用英夫利昔单抗和阿达木单抗等生物制剂(4)。
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Acta dermatovenerologica Croatica : ADC
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