Neutrophilic dermatosis

嗜中性皮肤病
  • 文章类型: Case Reports
    坏疽性脓皮病(PG)是一种罕见的嗜中性皮肤病,其特征是快速发展和疼痛的皮肤溃疡,具有独特的特征。就我们而言,东亚以前没有面部PG的病例报告.在这种情况下,我们描述了一例79岁的男性,他的脸颊和上唇有3个月的进行性疼痛性溃疡病史。最初怀疑非典型分枝杆菌感染导致治疗方案无效。综合感染检测结果呈阴性,pathergy测试阳性提示PG的潜在诊断。皮肤活检证实了诊断,静脉注射甲基强的松龙和口服环孢素治疗后,患者显着改善。三个月后,病变完全消退,无复发.该案例突出了与PG相关的诊断挑战,由于与其他条件相似,经常被误诊。彻底的评估对于排除替代诊断至关重要,尤其是皮肤感染。临床形态学,组织活检,和文化对于准确诊断至关重要。Pathergy的存在,轻微创伤后新病变的发展,也可以是诊断线索。
    Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis characterized by rapidly developing and painful skin ulcers with distinctive features. As far as we are concerned, there is no previous case report on facial PG in East-Asia. In this case, we describe a case of a 79-year-old man with a 3-month history of progressive painful ulcers on his cheek and upper lip. Initial suspicion of atypical mycobacterium infection led to an ineffective treatment regimen. Comprehensive infectious testing yielded negative results, and a positive pathergy test indicated a potential diagnosis of PG. A skin biopsy confirmed the diagnosis, and the patient showed significant improvement with intravenous methylprednisolone and oral cyclosporine treatment. After three months, complete resolution of the lesions was achieved without recurrence. The case highlights the diagnostic challenges associated with PG, which is often misdiagnosed due to its resemblance to other conditions. Thorough evaluation is crucial to exclude alternative diagnoses, particularly cutaneous infections. Clinical morphology, tissue biopsy, and culture are essential for accurate diagnosis. The presence of pathergy, the development of new lesions following minor trauma, can also be a diagnostic clue.
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  • 文章类型: Journal Article
    Sweet综合征是硫唑嘌呤治疗的罕见并发症,其临床特征尚不清楚。目的探讨硫唑嘌呤诱导的Sweet综合征(AISS)的临床特点,为诊断提供参考,治疗和预后。我们通过检索1960年至2022年12月31日的中文和英文数据库收集了AISS的相关病例报告,提取数据并进行了回顾性分析。44名患者的中位年龄为50岁(范围9-89岁),其中包括32名男性(72.7%)。发热(86.4%)和关节痛(31.8%)是最常见的临床症状。皮损以脓疱为主(54.5%),丘疹(40.9%),斑块(40.9%)和结节(31.8%),主要分布在四肢(54.5%),面部(38.6%)和手部(36.4%)。实验室检查显示中性粒细胞减少(65.9%)以及C反应蛋白升高(63.6%)和红细胞沉降率(40.9%)。病变皮肤的组织病理学表现为中性粒细胞浸润(93.2%)和真皮水肿(38.6%)。所有患者停用硫唑嘌呤后,中位时间为7天(范围2-28天),症状缓解。9例患者(20.5%)的皮肤病变在再次服用硫唑嘌呤后24小时内复发。临床医生和药师应掌握AISS的规律和特点,不建议重新使用硫唑嘌呤,避免Sweet综合征的复发.
    Sweet syndrome is a rare complication of azathioprine treatment with unelucidated clinical features. The purpose of this study was to investigate the clinical characteristics of azathioprine-induced Sweet syndrome (AISS) and provide a reference for diagnosis, treatment and prognosis. We collected relevant case reports of AISS by searching Chinese and English databases from 1960 to December 31, 2022, extracted the data and carried out a retrospective analysis. The median age of the 44 patients was 50 (range 9-89) years, and they included 32 males (72.7%). Fever (86.4%) and arthralgia (31.8%) were the most common clinical symptoms. The skin lesions were mainly pustules (54.5%), papules (40.9%), plaques (40.9%) and nodules (31.8%), which were mainly distributed on the extremities (54.5%), face (38.6%) and hands (36.4%). Laboratory examination revealed neutropenia (65.9%) as well as elevated C-reactive protein (63.6%) and erythrocyte sedimentation (40.9%) rates. Histopathology of the lesioned skin showed neutrophil infiltration (93.2%) and dermal edema (38.6%). Symptom relief was achieved at a median time of 7 days (range 2-28 days) after azathioprine discontinuation in all patients. Nine patients (20.5%) had skin lesions that recurred within 24 h after taking azathioprine again. Clinicians and pharmacists should grasp the regularity and characteristics of AISS and should not recommend the readministration of azathioprine, to avoid the recurrence of Sweet syndrome.
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  • 文章类型: Journal Article
    自身炎性疾病(AID)是一组异质性疾病,其中复发性或持续性无菌性炎症主要通过先天免疫系统的抗原非依赖性过度激活而产生。皮肤经常涉及各种各样的皮肤表现,其中大多数是非特定的。识别AIDs中的皮肤病变有时可能为正确诊断提供线索。在这次审查中,根据其特征性表现,将>20种选定的AIDs的皮肤受累情况进行总结并分为不同类别,如荨麻疹皮肤病,嗜中性皮肤病,肉芽肿病,冻疮,脂肪营养不良,角化过度.有了这个分类方案,可以更容易地识别AIDs的皮肤表现,以便在临床实践中进行诊断.
    Autoinflammatory diseases (AIDs) are a heterogeneous group of disorders in which recurrent or continuous aseptic inflammation arises primarily through antigen-independent hyperactivation of the innate immune system. The skin is frequently involved with a wide variety of cutaneous manifestations, most of which are non-specific. Recognition of skin lesions in AIDs may sometimes provide clues for a correct diagnosis. In this review, the cutaneous involvements of >20 selected AIDs were summarized and organized into different categories based on their characteristic manifestations, such as urticarial dermatosis, neutrophilic dermatosis, granulomatosis, chilblain, lipodystrophy, and hyperkeratosis. With this classification scheme, cutaneous manifestations in AIDs could be more easily identified to facilitate diagnosis in clinical practice.
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  • 文章类型: Case Reports
    Pyoderma gangrenosum (PG) is a rare chronic neutrophilic dermatosis that causes undermining ulcers. Unfortunately, standardization of PG treatment remains a challenge. In this article, we describe a case in which a 69-year-old man presented with a painful ulcer on the right lower leg. The diagnosis of PG was made after excluding other diseases. He had a history of PG on his left lower leg 2 years earlier and was cured by the treatment of systemic corticosteroids and cyclosporin A for 43 days. However, such a treatment was not effective this time. Hence, we applied intravenous immunoglobulin and negative-pressure wound therapy, and the patient was cured. Altogether, this case supports the use of intravenous immunoglobulin as an effective adjuvant for refractory PG, and indicates negative-pressure wound therapy as a treatment option to advance ulcer healing under adequate immunosuppression.
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  • 文章类型: Case Reports
    BACKGROUND: We report a rare case of chronic brucellosis accompanied with myelodysplastic syndrome and neutrophilic dermatosis, which to the best of our knowledge, has never been reported.
    METHODS: A young man was admitted to our hospital complaining of recurrent fever, arthritis, rashes and anemia. He had been diagnosed with brucellosis 6 years prior and treated with multiple courses of antibiotics. He was diagnosed with myelodysplastic syndrome and neutrophilic dermatosis following bone marrow puncture and skin biopsy. After anti-brucellosis treatment and glucocorticoid therapy, the symptoms improved.
    CONCLUSIONS: Clinicians should consider noninfectious diseases when a patient who has been diagnosed with an infectious disease exhibits changing symptoms.
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