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Adult-Onset Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis Syndrome on the Basis of Selective IgA Deficiency. 基于选择性 IgA 缺乏症的成人周期性发热、口腔炎、咽炎和宫颈腺炎综合征
Q3 Medicine Pub Date : 2024-07-31 eCollection Date: 2024-01-01 DOI: 10.1155/2024/9845501
Seda Altiner, Alper Ekinci

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome (PFAPA) is an autoinflammatory disease that is thought to occur with the contribution of genetic and environmental factors, but its etiology has not been clearly elucidated. It is characterized by recurrent attacks with fever, pharyngitis, oral aphthous lesions, and cervical lymphadenopathy, and an increase in the level of serum acute phase reactants is observed during the attacks. Although PFAPA usually begins in childhood, adult-onset cases are also reported in the literature. In the pathogenesis of PFAPA, an increase in the expression of various inflammatory cytokines, especially interleukin-1β (IL-1β), is observed as a result of the increase in inflammasome activity. Selective IgA deficiency (SIgAD) is the most prevalent primary immunodeficiency. Although most SIgAD cases remain asymptomatic and remain undiagnosed, it is known that the risk of mucosal infection is generally increased in SIgAD cases. In addition, the frequency of autoinflammatory diseases is increased in SIgAD cases compared with the general population. We aim to present a case of adult-onset PFAPA and SIgAD coexistence.

周期性发热、口腔炎、咽炎和颈腺炎综合征(PFAPA)是一种自身炎症性疾病,被认为与遗传和环境因素有关,但其病因尚未明确。其特点是反复发作,伴有发热、咽炎、口腔阿弗他病变和颈淋巴结病变,发作时可观察到血清急性期反应物水平升高。虽然 PFAPA 通常在儿童时期发病,但文献中也有成人发病的报道。在 PFAPA 的发病机制中,由于炎症小体活性增加,各种炎症细胞因子,尤其是白细胞介素-1β(IL-1β)的表达增加。选择性 IgA 缺乏症(SIgAD)是最普遍的原发性免疫缺陷病。虽然大多数 SIgAD 病例没有症状,也未被诊断出来,但众所周知,SIgAD 病例发生粘膜感染的风险通常会增加。此外,与普通人群相比,SIgAD 病例患自身炎症性疾病的频率也会增加。我们旨在介绍一例成人型 PFAPA 和 SIgAD 并存病例。
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引用次数: 0
Successful Use of Dupilumab in the Treatment of Acquired Perforating Dermatosis Associated with Atopic Dermatitis. 成功使用杜匹单抗治疗特应性皮炎相关的获得性穿孔性皮肤病。
Q3 Medicine Pub Date : 2024-06-19 eCollection Date: 2024-01-01 DOI: 10.1155/2024/6265608
Niccolò Gori, Eleonora De Luca, Andrea Chiricozzi, Stefania Sfregola, Alessandro Di Stefani, Ketty Peris

Acquired reactive perforating collagenosis is a rare cutaneous disorder characterised by the extrusion of abnormal connective tissue trough epidermidis and/or follicular units. Reactive perforating collagenosis is often associated with systemic diseases in which pruritus is a common symptom (e.g., diabetes and chronic kidney disease). Less commonly, it has been associated with chronic inflammatory dermatoses, including atopic dermatitis, as in this case. In this report, we describe the exceptional case of a 35-year-old man affected by acquired reactive perforating collagenosis associated with atopic dermatitis who was resistant to conventional topical and systemic treatment and experienced complete resolution of clinical signs and symptoms after 12 weeks of treatment with dupilumab. In our patient, the severe pruritus induced by atopic dermatitis likely contributed to the development of acquired perforating collagenosis lesions, which are thought to be a reactive response to chronic scratching and repetitive injury to the skin. Chronic pruritus in atopic dermatitis is known to be driven by type 2 cytokines, including IL-4 and IL-13, and dupilumab, a monoclonal antibody inhibiting IL-4 and IL-13 signalling, has been shown to be effective in the treatment of moderate to severe atopic dermatitis as well as other type 2-driven pruritic dermatological conditions. This case supports the potential use of dupilumab for the treatment of reactive perforating dermatosis.

获得性反应性穿孔胶原病是一种罕见的皮肤疾病,其特征是异常结缔组织通过表皮和/或毛囊单位挤出。反应性穿孔性胶原病通常与以瘙痒为常见症状的全身性疾病(如糖尿病和慢性肾病)有关。较少见的是,它与慢性炎症性皮肤病有关,包括特应性皮炎,本病例就是如此。在本报告中,我们描述了一例特殊病例:一名 35 岁的男性患者患有与特应性皮炎相关的获得性反应性穿孔性胶原病,对传统的局部和全身治疗均有抗药性,在使用杜必鲁单抗治疗 12 周后,临床症状和体征完全消失。在我们的患者中,特应性皮炎引起的严重瘙痒很可能导致了获得性穿孔性胶原病病变的发生,这种病变被认为是对慢性搔抓和皮肤反复损伤的反应性反应。众所周知,特应性皮炎的慢性瘙痒是由包括IL-4和IL-13在内的2型细胞因子驱动的,而抑制IL-4和IL-13信号传导的单克隆抗体dupilumab已被证明能有效治疗中度至重度特应性皮炎以及其他2型细胞因子驱动的瘙痒性皮肤病。本病例支持使用杜匹单抗治疗反应性穿孔性皮肤病。
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引用次数: 0
Successful Treatment of Refractory Generalized Granuloma Annulare with Upadacitinib. 乌达帕替尼成功治疗难治性全身性环状肉芽肿
Q3 Medicine Pub Date : 2024-06-05 eCollection Date: 2024-01-01 DOI: 10.1155/2024/8859178
Alexis Coican, Abigail Meckley, Nathan Sagasser, Melinda Greenfield, Eingun James Song, Jessica El-Bahri

Granuloma annulare is a poorly understood dermatosis that, when generalized, can occur in up to 15 percent of patients. In these cases, treatment is frustrating and experimental. We report a case of a 60-year-old woman and a 41-year-old woman who demonstrated resolution of recalcitrant, generalized granuloma annulare (GA) following oral treatment with upadacitinib. After showing little to no response to other various treatments, such as steroids, antibiotic regimens, and systemic therapies, each patient was started on 15 mg of daily upadacitinib. At 2 months, one patient had complete clearance of all lesions while the other patient experienced noticeable improvement. Within 4 months, the other patient reached total resolution of her lesions. These cases provide evidence of a therapeutic option that may shorten disease duration and provide relief from cutaneous disease.

环状肉芽肿是一种鲜为人知的皮肤病,如果泛发全身,多达 15% 的患者会发病。在这些病例中,治疗是令人沮丧和试验性的。我们报告了一例 60 岁女性和一例 41 岁女性在口服达帕替尼(upadacitinib)治疗后,顽固性、全身性环状肉芽肿(GA)得到缓解的病例。在对类固醇、抗生素方案和全身疗法等其他各种治疗几乎没有反应后,每位患者开始每天服用15毫克的达帕西替尼。2 个月后,其中一名患者的所有病灶完全清除,另一名患者的病灶明显好转。在 4 个月内,另一名患者的病灶完全清除。这些病例为一种可缩短病程并缓解皮肤疾病的治疗方案提供了证据。
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引用次数: 0
Reactive Angioendotheliomatosis in Association with Ulcerative Colitis. 与溃疡性结肠炎有关的反应性血管内皮瘤病
Q3 Medicine Pub Date : 2024-05-29 eCollection Date: 2024-01-01 DOI: 10.1155/2024/6505274
R Afiouni, G Abadjian, F Stephan

Reactive angioendotheliomatosis (RAE) is a rare benign skin condition characterized histologically by the proliferation of dermal vessels and endothelial cells that occurs secondary to an underlying disease such as infections or lymphoproliferative disorders. To our knowledge, no previous cases of RAE associated with ulcerative colitis (UC) were reported in the literature. Therefore, we report the case of a 46-year-old man with a history of UC presenting with RAE confirmed on histopathology and immunostaining.

反应性血管内皮细胞瘤病(RAE)是一种罕见的良性皮肤病,其组织学特征是真皮血管和内皮细胞增生,继发于感染或淋巴增生性疾病等潜在疾病。据我们所知,文献中从未报道过 RAE 与溃疡性结肠炎(UC)相关的病例。因此,我们报告了一例经组织病理学和免疫染色证实伴有 RAE 的 46 岁男性 UC 病例。
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引用次数: 0
Granuloma after the Injection of Poly-D,L-Lactic Acid (PDLLA) Treated with Triamcinolone 注射聚-D,L-乳酸 (PDLLA) 并用曲安奈德治疗后出现的肉芽肿
Q3 Medicine Pub Date : 2024-04-25 DOI: 10.1155/2024/6544506
Kateryn Michelle Perez Willis, Rosario Ramirez Galvez
Biostimulators are the latest trends in cosmetic procedures, substances such as PDLLA are used to induce collagen synthesis by a subclinical inflammatory reaction. We are describing a granuloma-like reaction case presentation 4 months after the application of PDLLA and its complete resolution with injections of triamcinolone. A 45-year-old female with any past medical history of allergies or immune diseases was injected with PDLLA on the mandibular border and cheek area to correct skin laxity. Four months after the application, the patient reported facial edema and granuloma-like reactions according to clinical examination on all the application areas. The ultrasound reports showed the presence of multiple nodules in the injection areas; therefore, we decided to apply triamcinolone to the granulomatous reaction areas 2 times a month and Prednisone 20 mg daily for 3 days followed by 10 mg for 2 days. After 4 applications, the adverse reaction was completely solved. Biostimulators are biocompatible and resorbable substances; however, nodules and/or granulomas have been reported as rare adverse events. Intralesional and oral steroids can allow us to treat this kind of adverse events.
生物刺激剂是美容手术的最新趋势,PDLLA 等物质可通过亚临床炎症反应诱导胶原蛋白合成。我们在此描述一例肉芽肿样反应病例,患者在使用 PDLLA 4 个月后出现肉芽肿样反应,注射曲安奈德后肉芽肿完全消退。一位 45 岁的女性既往没有过敏或免疫疾病病史,为了矫正皮肤松弛,她在下颌骨边缘和脸颊部位注射了 PDLLA。注射四个月后,根据临床检查,患者称所有注射区域都出现了面部水肿和肉芽肿样反应。超声波报告显示,注射区域存在多个结节;因此,我们决定在肉芽肿反应区域使用曲安奈德,每月 2 次,同时每天使用泼尼松 20 毫克,连用 3 天,然后再使用 10 毫克,连用 2 天。使用 4 次后,不良反应完全消除。生物刺激剂是生物相容性和可吸收的物质,但据报道,结节和/或肉芽肿是罕见的不良反应。局部注射和口服类固醇可以治疗这类不良反应。
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引用次数: 0
Treatment of Subcorneal Pustular Dermatosis without Dapsone: A Case Report and Review of the Literature 不使用达哌酮治疗角质层下脓疱性皮肤病:病例报告和文献综述
Q3 Medicine Pub Date : 2024-04-02 DOI: 10.1155/2024/8140483
Lindsey J Wanberg, B. Schultz, A. Goyal
Subcorneal pustular dermatosis (SPD) is a rare neutrophilic dermatosis characterized by pustules on the trunk and intertriginous areas. While oral dapsone is the first-line treatment for SPD, alternative options are necessary for patients with glucose-6-phosphate dehydrogenase deficiency, drug hypersensitivity reactions, or refractory disease. To date, no consensus exists regarding next-best agents for SPD. In this report, we present a patient with significant SPD who developed dapsone hypersensitivity syndrome and then was successfully treated with colchicine and adalimumab. We propose that colchicine should be considered as a second-line treatment for SPD and present a therapeutic algorithm for clinicians to utilize when patients are not candidates for dapsone, have side effects requiring drug discontinuation, or have refractory disease.
角膜下脓疱性皮肤病(SPD)是一种罕见的嗜中性皮肤病,其特征是在躯干和三叉神经间区域出现脓疱。虽然口服多塞酮是治疗脓疱性皮炎的一线药物,但对于患有葡萄糖-6-磷酸脱氢酶缺乏症、药物过敏反应或难治性疾病的患者来说,有必要选择其他药物。迄今为止,关于治疗 SPD 的次佳药物尚未达成共识。在本报告中,我们介绍了一名患有严重SPD的患者,该患者出现了地松超敏反应综合征,后经秋水仙碱和阿达木单抗治疗获得成功。我们建议将秋水仙碱作为SPD的二线治疗药物,并提出了一种治疗算法,供临床医生在患者不适合使用他松、出现副作用需要停药或病情难治时使用。
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引用次数: 0
A Case of Diltiazem-Induced Pustular Rash in an 83-Year-Old Female 一名 83 岁女性的地尔硫卓诱发脓疱性皮疹病例
Q3 Medicine Pub Date : 2024-04-02 DOI: 10.1155/2024/9547206
Ayrton I Bangolo, Karen Yelton-Torres, Atharv Mahajan, Vrushant Patel, Laiba Sajjad, Prince Ofori Ansong, Monisha Kashyap, Umesh Batura, Madhavi Ravulapalli, Ayodya Perera, Simcha I Weissman
Acute generalized exanthematous pustulosis (AGEP) is a rare, acute skin eruption characterized by the development of numerous nonfollicular sterile pustules. Most cases are caused by drug reactions, among which Diltiazem has been incriminated. Herein, we present an 83-year-old female who presented for evaluation of generalized skin rash 3 days after initiation of Diltiazem. She was eventually diagnosed with AGEP, Diltiazem was discontinued, and systemic steroids were administered with the resolution of symptoms. This case report has the objective of encouraging clinicians to include AGEP in the differential diagnosis of skin eruption following the initiation of Diltiazem.
急性全身泛发性脓疱病(AGEP)是一种罕见的急性皮肤疹,其特征是出现大量非叶状无菌脓疱。大多数病例是由药物反应引起的,其中地尔硫卓是罪魁祸首。在本文中,我们介绍了一名 83 岁的女性患者,她在开始服用地尔硫卓 3 天后因全身皮疹前来就诊。她最终被诊断为 AGEP,停用了地尔硫卓,并使用了全身性类固醇药物,症状得到缓解。本病例报告旨在鼓励临床医生在开始使用地尔硫卓后将 AGEP 纳入皮肤疹的鉴别诊断中。
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引用次数: 0
A Saudi Woman with Ceftriaxone Induced Fixed Drug Eruption. 一名沙特妇女因头孢曲松诱发固定药物喷发。
Q3 Medicine Pub Date : 2024-03-15 eCollection Date: 2024-01-01 DOI: 10.1155/2024/9975455
Rehab Y Al-Ansari, Leena Abdulrahman Almuhaish, Khaled Abdullah Hassan, Tawasoul Fadoul, Alexander Woodman

Background: A fixed drug eruption (FDE) is an immunological cutaneous adverse reaction, classified as a cutaneous adverse drug reaction (CADR) and characterized by well-defined lichenoid lesions that occur at the same site each time. Ceftriaxone is a third-generation antibiotic of cephalosporin antibiotics of the beta-lactam antibiotic family, which has typical in vitro activity against many Gram-negative aerobic bacteria. This is the first clinical case from Saudi Arabia and the fifth in the world to document a woman's experience with recurrent FDE after repeated ceftriaxone use. Case Report. A 25-year-old Saudi woman with a known case of sickle cell anemia (SCA) with a history of avascular necrosis of the right hip after replacement was hospitalized with a pain crisis triggered by an upper respiratory tract infection. The patient denied having a history of allergy previously. Due to fever, leukocytosis, and active follicular tonsillitis, ceftriaxone was started. However, a few hours later she developed lip edema and a fixed drug eruption measuring 7 × 11 cm on the left side of her back. The lesion reformed over a hyperpigmented lesion (4 × 8 cm) that the patient did not report upon initial examination. It turned out that this was due to the intravenous administration of ceftriaxone, a year ago in another hospital. An allergy to ceftriaxone was considered, and steroids and antihistamines were started. The case was labeled as ceftriaxone induced FDE.

Conclusion: Ceftriaxone induced FDE is an uncommon type of allergic reaction that has been reported infrequently. Understanding this condition and the mechanism by which FDE becomes recurrent with the same previous fixed lesion is of great importance for both academic and future research purposes.

背景:固定药物疹(FDE)是一种免疫性皮肤不良反应,被归类为皮肤药物不良反应(CADR),其特征是每次在同一部位出现界限清楚的苔藓样皮损。头孢曲松是β-内酰胺类抗生素家族中头孢菌素类抗生素的第三代抗生素,对多种革兰氏阴性需氧菌具有典型的体外活性。这是沙特阿拉伯的第一个临床病例,也是世界上第五个记录一名妇女在反复使用头孢曲松后复发胎盘早剥的病例。病例报告。一名 25 岁的沙特妇女已知患有镰状细胞性贫血(SCA),曾在右髋关节置换术后出现血管性坏死,因上呼吸道感染引发疼痛危机而住院治疗。患者否认之前有过敏史。由于发烧、白细胞增多和活动性滤泡性扁桃体炎,医生开始使用头孢曲松。然而,几小时后,她出现了嘴唇水肿,背部左侧出现了 7 × 11 厘米的固定药物疹。皮损在一个色素沉着的皮损(4 × 8 厘米)上重新形成,患者在初次检查时并未报告。原来,这是一年前在另一家医院静脉注射头孢曲松所致。考虑到患者对头孢曲松过敏,医生开始使用类固醇和抗组胺药物。该病例被诊断为头孢曲松诱发的FDE:结论:头孢曲松诱发的FDE是一种不常见的过敏反应,鲜有报道。了解这种情况以及 FDE 因先前的相同固定病灶而复发的机制,对于学术研究和未来研究都具有重要意义。
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引用次数: 0
Sporadic Pemphigus Foliaceus in a 3-Year-Old Vietnamese Girl: A Case Report and Literature Review 一名 3 岁越南女孩的散发性丘疹性红斑狼疮:病例报告和文献综述
Q3 Medicine Pub Date : 2024-02-16 DOI: 10.1155/2024/6748340
Trinh Thi Diem Nguyen, Trinh Ngoc To Chau, Phuong Thi Doan Vo, H. Nguyen
Pemphigus foliaceus is an uncommon autoimmune intraepidermal blistering disease characterized by immunoglobulin (Ig) G autoantibodies that attack desmoglein-1 in the epidermis. There are two predominant forms of pemphigus foliaceus, sporadic and endemic. Sporadic pemphigus foliaceus is known to be more prevalent in middle-aged and elderly people and to be extremely rare in children. Less than 40 nonendemic pediatric pemphigus foliaceus cases have been documented in the literature. This report documents a case of sporadic pemphigus foliaceus in a 3-year-old Vietnamese girl who presented with generalized scaling and crusted erosions over the body.
丘疹性荨麻疹是一种不常见的自身免疫性表皮内水疱病,其特征是免疫球蛋白(Ig)G自身抗体攻击表皮中的去疱疹素-1。叶面丘疹性荨麻疹有两种主要形式,即散发性和地方性。据了解,散发性叶面天疱疮多见于中老年人,在儿童中极为罕见。文献中记载的非流行性小儿丘疹性红斑狼疮病例不足 40 例。本报告记录了一例散发性丘疹性红斑狼疮病例,患者是一名 3 岁的越南女孩,全身出现鳞屑和结痂性糜烂。
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引用次数: 0
A Case Study of Furunculosis following an Ayurvedic Oil Massage, Sudation Therapy, and Lessons to Learn. 阿育吠陀精油按摩、须弥疗法后的疖病病例研究,以及应吸取的教训。
Q3 Medicine Pub Date : 2024-02-08 eCollection Date: 2024-01-01 DOI: 10.1155/2024/3660064
Satyajit Pandurang Kulkarni, Pallavi Satyajit Kulkarni, S Kumar

India has a long history of using sudation therapy and oil massage as Ayurvedic treatments. However, nothing is known about its side effects, and just two studies have identified side effects as cutaneous adverse drug reactions brought on by Ayurvedic oil massage. We are presenting the example of a 72-year-old adult man who visited our hospital and had his right knee massaged with Nirgudi oil followed by sudation therapy. Erythema, papules, itchiness, and scorching pain were some of his symptoms. However, these sensations only partially abated once we quit sudation therapy. Our investigation demonstrates that a Nirgudi oil massage or sudation causes the skin reaction associated with furunculosis. This case report illustrates the necessity of being aware of Panchakarma-related consequences and suggests that medical practitioners, patients, and product makers take into account the likelihood of such a reaction following Nirgudi oil massage and sudation therapy as a precaution.

印度使用涂油疗法和精油按摩作为阿育吠陀疗法的历史悠久。然而,人们对其副作用却一无所知,仅有两项研究发现阿育吠陀精油按摩带来的副作用是皮肤药物不良反应。我们以一名 72 岁的成年男子为例,他曾到我院就诊,并接受了 Nirgudi 油按摩右膝盖,随后又接受了涂抹疗法。他的症状包括红斑、丘疹、瘙痒和灼痛。然而,当我们停止熏蒸疗法后,这些感觉才部分减轻。我们的调查表明,尼尔古迪油按摩或熏蒸会导致与疖病相关的皮肤反应。本病例报告说明有必要了解与潘查卡玛疗法相关的后果,并建议医疗从业人员、患者和产品制造商在进行尼尔古地精油按摩和熏蒸疗法后考虑到出现这种反应的可能性,以防万一。
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引用次数: 0
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Case Reports in Dermatological Medicine
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