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Myelodysplasia cutis and VEXAS syndrome initially diagnosed as histiocytoid Sweet syndrome: A diagnostic pitfall 切面骨髓增生症和 VEXAS 综合征最初被诊断为组织细胞样斯威特综合征:诊断陷阱。
IF 1.6 4区 医学 Q3 DERMATOLOGY Pub Date : 2024-07-12 DOI: 10.1111/cup.14678
Serena J. Shimshak MD, Sion Jasmine MD, Mark D. P. Davis MD, Emma F. Johnson MD, Margot S. Peters MD, Gang Zheng MD, PhD, Olayemi Sokumbi MD, Nneka I. Comfere MD

Histiocytoid Sweet syndrome (H-SS) is a histopathological variant of Sweet syndrome (SS) defined by cutaneous infiltration of immature myeloid cells morphologically resembling histiocytes. The association of H-SS with underlying malignancy, particularly myelodysplastic syndromes, is well-established. Myelodysplasia cutis (MDS-cutis) has been proposed to describe cases historically diagnosed as H-SS but characterized by shared clonality of the myeloid infiltrate in skin and bone marrow. Therefore, identifying patients who might have MDS-cutis is critical for the management of the associated hematologic malignancy. VEXAS syndrome, an adult-onset autoinflammatory disease, should also be included in the histopathologic differential diagnosis of H-SS, as it shares clinical and pathologic features with MDS-cutis. Through the presentation of two cases, we aim to highlight the defining features and key clinical implications of MDS-cutis and VEXAS syndrome.

组织细胞样斯威特综合征(H-SS)是斯威特综合征(SS)的一种组织病理学变异型,其定义是形态类似组织细胞的未成熟髓系细胞的皮肤浸润。H-SS 与潜在的恶性肿瘤,尤其是骨髓增生异常综合征有密切关系。骨髓增生异常切面症(MDS-cutis)被提出来描述历史上诊断为 H-SS 的病例,但其特点是皮肤和骨髓中的骨髓浸润具有共同的克隆性。因此,识别可能患有 MDS-cutis 的患者对于治疗相关的血液恶性肿瘤至关重要。VEXAS综合征是一种成人发病的自身炎症性疾病,也应纳入H-SS的组织病理学鉴别诊断中,因为它与MDS-cutis具有相同的临床和病理特征。通过两个病例的介绍,我们旨在强调 MDS-cutis 和 VEXAS 综合征的定义特征和主要临床意义。
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引用次数: 0
Acantholytic PRIDE syndrome. 黄疽 PRIDE 综合征
IF 1.6 4区 医学 Q3 DERMATOLOGY Pub Date : 2024-07-10 DOI: 10.1111/cup.14684
Biswanath Behera, Ashish Kumar Nayak, Siddhartha Dash, Madhusmita Sethy, Pavithra Ayyanar
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引用次数: 0
Primary cutaneous EBV+ extranodal NK/T-cell lymphoma of gamma/delta T-cell lineage in the posttransplantation setting 移植后皮肤原发性EBV+结节外γ/δT细胞系NK/T细胞淋巴瘤。
IF 1.6 4区 医学 Q3 DERMATOLOGY Pub Date : 2024-07-10 DOI: 10.1111/cup.14677
Jessica F. Williams, Fabienne M. Lucas, Ruben D. Carrasco, Scott B. Lovitch, David C. Fisher, Thomas S. Kupper, Sam Sadigh

Posttransplantation primary cutaneous T-cell lymphomas (PT-CTCL) are a rare complication of sustained immunosuppression in the posttransplant setting. When present, PT-CTCLs are typically EBV− and exhibit features of mycosis fungoides/Sézary syndrome or CD30+ lymphoproliferative disorders. We present a case of a 75-year-old individual who developed skin lesions 30 years after liver transplantation. Pathologic evaluation of the skin biopsy revealed involvement by a clonal, EBV+ T-cell population of gamma/delta lineage with no evidence of systemic disease. Comprehensive genomic profiling was performed, confirming focal one-copy loss of 6q23.3, altogether consistent with the extremely rare and unusual diagnosis of primary cutaneous EBV+ extranodal NK/T-cell lymphoma of gamma/delta T-cell lineage in the posttransplantation setting.

移植后原发性皮肤T细胞淋巴瘤(PT-CTCL)是移植后持续免疫抑制的一种罕见并发症。PT-CTCL通常与EB病毒有关,并具有真菌病/塞扎里综合征或CD30+淋巴增生性疾病的特征。我们报告了一例 75 岁的患者,他在接受肝移植手术 30 年后出现皮肤病变。对皮肤活检的病理评估显示,γ/δ系的克隆、EBV+ T细胞群受累,但没有全身性疾病的证据。综合基因组图谱检查证实了 6q23.3 局灶性单拷贝缺失,这与原发性皮肤 EBV+结节外 NK/T 细胞淋巴瘤(γ/delta T 细胞系)这一极其罕见和不寻常的诊断一致。
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引用次数: 0
PRRX1-fused mesenchymal neoplasm: A novel PRRX1::NCOA1 fusion transcript PRRX1融合间充质肿瘤:一种新型的PRRX1::NCOA1融合转录本。
IF 1.6 4区 医学 Q3 DERMATOLOGY Pub Date : 2024-07-10 DOI: 10.1111/cup.14683
Xiao Cheng MD, Jian Wang MD, PhD, Rong Fang PhD, Jiayun Xu MS, Suying Wang MD, Ming Zhao MD

PRRX1-fused mesenchymal neoplasm is a recently identified, rare subcutaneous soft tissue neoplasm that is characterized by fusion of PRRX1 (exon 1) with NCOA1 (exon 13) in the majority of reported cases. Although initially considered to be fibroblastic, a possibility of neural or neuroectodermal differentiation has been suggested in a subset of cases. We report a 26-year-old female with a 4.0 cm painless mass located in the subcutis of the left thigh. Microscopically, the tumor was well-circumscribed and multinodular and was composed of relatively monomorphic ovoid to spindle cells arranged in loose fascicles, trabeculae, and cords within alternating myxoid and fibrous matrix, and vascularized stroma. Mitotic figures were scarce and necrosis was not observed. By immunohistochemistry, the neoplastic cells demonstrated focal co-expression of S100 protein and SOX10 and were negative for epithelial membrane antigen, smooth muscle actin, desmin, CD34, STAT6, HMB45, Melan-A, and MUC4. The expression of Rb1 was retained. Targeted RNA-sequencing identified a novel transcript fusion of PRRX1 (exon 1)::NCOA1 (exon 15), which was further confirmed by reverse transcription polymerase chain reaction and Sanger sequencing. The tumor was narrowly excised and no tumor recurrence or metastasis was identified after 13 months of follow-up. In summary, we report a new case of PRRX1-fused mesenchymal neoplasm, expanding the molecular genetic spectrum and providing further support for possible neural or neuroectodermal differentiation of this emerging soft tissue tumor entity.

PRRX1融合间充质肿瘤是最近发现的一种罕见的皮下软组织肿瘤,在大多数报道的病例中,其特征是PRRX1(外显子1)与NCOA1(外显子13)融合。虽然最初被认为是成纤维细胞性肿瘤,但也有一部分病例可能是神经或神经外胚层分化。我们报告了一名 26 岁女性的病例,她的左大腿皮下有一个 4.0 厘米的无痛性肿块。显微镜下,肿瘤呈环状和多结节状,由相对单形的卵圆形至纺锤形细胞组成,排列成松散的束状、小梁状和条索状,交替分布在肌层和纤维基质以及血管基质中。有丝分裂很少,也未观察到坏死。通过免疫组化,肿瘤细胞显示出 S100 蛋白和 SOX10 的灶性共表达,而上皮膜抗原、平滑肌肌动蛋白、desmin、CD34、STAT6、HMB45、Melan-A 和 MUC4 均为阴性。保留了 Rb1 的表达。靶向 RNA 测序确定了 PRRX1(外显子 1)::NCOA1(外显子 15)的新型转录本融合,并通过反转录聚合酶链反应和 Sanger 测序进一步证实了这一点。经过 13 个月的随访,肿瘤被勉强切除,未发现肿瘤复发或转移。总之,我们报告了一例新的 PRRX1 融合间充质肿瘤病例,扩大了分子遗传谱,并为这种新兴软组织肿瘤实体可能的神经或神经外胚层分化提供了进一步支持。
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引用次数: 0
Toxic epidermal necrolysis-like cutaneous toxicity following chimeric antigen receptor T-cell therapy in recurrent large B-cell lymphoma 嵌合抗原受体 T 细胞疗法治疗复发性大 B 细胞淋巴瘤后出现的中毒性表皮坏死溶解症样皮肤毒性。
IF 1.6 4区 医学 Q3 DERMATOLOGY Pub Date : 2024-07-09 DOI: 10.1111/cup.14687
Puneet K. Bhullar MD, Kiran Motaparthi MD, Daniel P. Zieman MD, Cassandra Johnson DO, Pooja Gurnani MD, Olayemi Sokumbi MD

Chimeric antigen receptor (CAR) T-cell therapy has demonstrated remarkable success in treating various B-cell malignancies, redirecting T-cell cytotoxicity toward cancer cells. Despite its efficacy, CAR-T therapy is associated with potential risks, including cytokine release syndrome (CRS) and cytopenia. We present a case of a 69-year-old man with diffuse large B-cell lymphoma treated with axicabtagene-ciloleucel CAR-T therapy, who developed a rare and severe cutaneous toxicity resembling toxic epidermal necrolysis (TEN). The patient exhibited persistent fevers, CRS, and subsequent development of a widespread erythematous macular eruption, progressing to vesiculation with bullae. Notably, allopurinol-induced TEN was considered with the patient's recent exposure to allopurinol, although the onset and minimal mucosal involvement did not align with typical presentations of allopurinol-induced cases. The cutaneous reaction, distinct from typical SJS/TEN, showed minimal mucosal involvement and coincided with the cytokine release storm, differing from allopurinol-induced TEN. Despite the absence of guidelines, the patient was managed with systemic steroids, achieving significant improvement. This case expands the spectrum of CAR-T therapy-related cutaneous toxicities, highlighting the need for early recognition of histopathology and tailored management by dermatologists. Further understanding of these reactions is crucial for optimizing the safety profile of this groundbreaking immunotherapy.

嵌合抗原受体(CAR)T 细胞疗法在治疗各种 B 细胞恶性肿瘤方面取得了显著的成功,它能将 T 细胞的细胞毒性重新导向癌细胞。尽管 CAR-T 疗法疗效显著,但也存在潜在风险,包括细胞因子释放综合征(CRS)和全血细胞减少症。我们报告了一例 69 岁男性弥漫大 B 细胞淋巴瘤患者接受阿昔单抗-伊洛琉醇 CAR-T 疗法治疗后出现类似中毒性表皮坏死(TEN)的罕见严重皮肤毒性的病例。患者表现出持续发热、CRS,随后出现广泛的红斑性糜烂,并发展为水疱。值得注意的是,患者最近接触过别嘌呤醇,因此被认为是别嘌呤醇诱发的 TEN,尽管其发病和最小的粘膜受累与别嘌呤醇诱发病例的典型表现并不一致。皮肤反应有别于典型的SJS/TEN,粘膜受累程度极低,且与细胞因子释放风暴同时发生,与别嘌呤醇诱发的TEN不同。尽管没有相关指南,但患者还是接受了全身类固醇治疗,病情得到了明显改善。该病例扩大了CAR-T疗法相关皮肤毒性的范围,强调了皮肤科医生早期识别组织病理学并进行针对性治疗的必要性。进一步了解这些反应对于优化这种突破性免疫疗法的安全性至关重要。
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引用次数: 0
A rare form of metastatic breast carcinoma mimicking lymphangioma circumscriptum 一种罕见的模仿周身淋巴管瘤的转移性乳腺癌。
IF 1.6 4区 医学 Q3 DERMATOLOGY Pub Date : 2024-07-09 DOI: 10.1111/cup.14688
Zacaria Jr. B. Pario, Ma Flordeliz Abad-Casintahan, Jonnie Rose Louise R. Wee, Melissa Rinne V. See, Karen Andrea D. Cadacio

Cutaneous metastasis is rare but may indicate an advanced internal malignancy or a recurrence of a previously treated one and is usually associated with a poor prognosis. They may also pose a diagnostic problem as the clinical manifestations are variable and non-specific, which could mimic other benign conditions. We report a case of a 48-year-old female who presented with a 4-year history of erythematous papules and vesicles on the trunk mimicking lymphangioma circumscriptum. Skin biopsy and immunohistochemistry were consistent with cutaneous metastasis from breast carcinoma. Cutaneous metastasis presents in a variety of patterns. A high index of suspicion and a low threshold for skin biopsy are paramount to the early diagnosis and treatment. A histopathologic evaluation will help identify the origin of the cutaneous metastasis and can significantly affect the outcome of the treatment.

皮肤转移很罕见,但可能预示着内部恶性肿瘤已到晚期,或之前治疗过的恶性肿瘤复发,通常预后较差。由于皮肤转移瘤的临床表现多变且无特异性,可能会与其他良性疾病相混淆,因此也会给诊断带来困难。我们报告了一例 48 岁女性的病例,她躯干上的红斑丘疹和水泡病史长达 4 年,模仿周身淋巴管瘤。皮肤活检和免疫组化结果与乳腺癌的皮肤转移一致。皮肤转移的表现形式多种多样。高怀疑指数和低皮肤活检门槛对早期诊断和治疗至关重要。组织病理学评估将有助于确定皮肤转移瘤的来源,并对治疗效果产生重大影响。
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引用次数: 0
Immunophenotyping and viral studies in pityriasis lichenoides et varioliformis acuta lesions 脓疱型苔癣和变异型脓疱病皮损的免疫分型和病毒研究。
IF 1.6 4区 医学 Q3 DERMATOLOGY Pub Date : 2024-07-07 DOI: 10.1111/cup.14679
Salih Mishlab, Emily Avitan-Hersh, Yaniv Zohar, Moran Szwarcwort-Cohen, Reuven Bergman

Background

The underlying pathogenesis of pityriasis lichenoides et varioliformis acuta (PLEVA) remains unclear, although immunologic injury and viral etiology have been suggested.

Objective

To evaluate and expand the immunophenotype of PLEVA and to search for possible viral pathogens.

Methods

Formalin-fixed, paraffin-embedded specimens of 20 patients with PLEVA and 9 patients with common inflammatory dermatoses (ID) were studied for immunophenotyping and for human herpesvirus (HHV) 1 and 2, cytomegalovirus (CMV), HHV-8, parvovirus B19, and Epstein–Barr virus (EBV) immunohistochemistry. The presence of HHV-6, HHV-7, and enteroviruses was assayed molecularly.

Results

The numbers of CD8+ T cells and T-cell intracellular antigen-1 (TIA-1)+ cells were statistically significantly higher in PLEVA compared to the ID group. Immunohistochemistry for human HHV-1 and HHV-2, CMV and HHV-8, parvovirus B19, and in situ hybridization for EBV were all negative. There was molecular evidence for HHV-7 in only one PLEVA case (5%). Molecular studies for HHV-6 and enterovirus involvement were negative in all the PLEVA specimens.

Conclusions

The predominant T-cell infiltrate in PLEVA is dominated by CD8+ cells, and by increased numbers of TIA1+ cells, which may indicate a cytotoxic T-cell damage to the epidermis. Viral presence was not detected.

背景:尽管免疫损伤和病毒病因已被提出,但脓疱性苔藓和变异性苔藓(PLEVA)的潜在发病机制仍不清楚:评估并扩展 PLEVA 的免疫表型,寻找可能的病毒病原体:方法:对20例PLEVA患者和9例常见炎症性皮肤病(ID)患者的福尔马林固定、石蜡包埋标本进行免疫分型和人类疱疹病毒(HHV)1和2、巨细胞病毒(CMV)、HHV-8、副病毒B19和爱泼斯坦-巴尔病毒(EBV)免疫组化研究。分子检测了 HHV-6、HHV-7 和肠道病毒的存在:结果:与 ID 组相比,PLEVA 组 CD8+ T 细胞和 T 细胞胞内抗原-1 (TIA-1)+ 细胞的数量在统计学上明显增加。人类HHV-1和HHV-2、CMV和HHV-8、副病毒B19的免疫组化以及EB病毒的原位杂交均为阴性。只有一个 PLEVA 病例(5%)有 HHV-7 的分子证据。在所有 PLEVA 标本中,HHV-6 和肠道病毒的分子研究均为阴性:结论:PLEVA 中主要的 T 细胞浸润以 CD8+ 细胞为主,TIA1+ 细胞数量增加,这可能表明表皮受到了细胞毒性 T 细胞的损伤。未检测到病毒的存在。
{"title":"Immunophenotyping and viral studies in pityriasis lichenoides et varioliformis acuta lesions","authors":"Salih Mishlab,&nbsp;Emily Avitan-Hersh,&nbsp;Yaniv Zohar,&nbsp;Moran Szwarcwort-Cohen,&nbsp;Reuven Bergman","doi":"10.1111/cup.14679","DOIUrl":"10.1111/cup.14679","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>The underlying pathogenesis of pityriasis lichenoides et varioliformis acuta (PLEVA) remains unclear, although immunologic injury and viral etiology have been suggested.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Objective</h3>\u0000 \u0000 <p>To evaluate and expand the immunophenotype of PLEVA and to search for possible viral pathogens.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Formalin-fixed, paraffin-embedded specimens of 20 patients with PLEVA and 9 patients with common inflammatory dermatoses (ID) were studied for immunophenotyping and for human herpesvirus (HHV) 1 and 2, cytomegalovirus (CMV), HHV-8, parvovirus B19, and Epstein–Barr virus (EBV) immunohistochemistry. The presence of HHV-6, HHV-7, and enteroviruses was assayed molecularly.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>The numbers of CD8<sup>+</sup> T cells and T-cell intracellular antigen-1 (TIA-1)<sup>+</sup> cells were statistically significantly higher in PLEVA compared to the ID group. Immunohistochemistry for human HHV-1 and HHV-2, CMV and HHV-8, parvovirus B19, and in situ hybridization for EBV were all negative. There was molecular evidence for HHV-7 in only one PLEVA case (5%). Molecular studies for HHV-6 and enterovirus involvement were negative in all the PLEVA specimens.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>The predominant T-cell infiltrate in PLEVA is dominated by CD8<sup>+</sup> cells, and by increased numbers of TIA1<sup>+</sup> cells, which may indicate a cytotoxic T-cell damage to the epidermis. Viral presence was not detected.</p>\u0000 </section>\u0000 </div>","PeriodicalId":15407,"journal":{"name":"Journal of Cutaneous Pathology","volume":null,"pages":null},"PeriodicalIF":1.6,"publicationDate":"2024-07-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141554917","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Trainee perceptions of digital dermatopathology implementation at a multisite academic institution 受训人员对多地点学术机构实施数字皮肤病理学的看法。
IF 1.6 4区 医学 Q3 DERMATOLOGY Pub Date : 2024-07-05 DOI: 10.1111/cup.14681
Jacob P. Reinhart MD, Sydney L. Proffer MD, MS, Jennifer L. Ridgeway PhD, Barbara Barry PhD, Celia Kamath PhD, Erin Wissler Gerdes MA, Derek Cervenka MS, Margot S. Peters MD, Nneka I. Comfere MD, Emma F. Johnson MD, Carilyn N. Wieland MD
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引用次数: 0
Coexistence of large cell transformed mycosis fungoides and diffuse large B-cell lymphoma in one patient 一名患者同时患有大细胞变异真菌病和弥漫大 B 细胞淋巴瘤。
IF 1.6 4区 医学 Q3 DERMATOLOGY Pub Date : 2024-07-05 DOI: 10.1111/cup.14673
Thomas Z. Rohan BS, Jayson Suriano BA, Volkan Tekmen BS, Safiyyah Bhatti MD, Sahithi Talasila BS, Daniel Joffe BS, Caleb Holtmeyer MD, Jason B. Lee MD, Onder Alpdogan MD, Neda Nikbakht MD, PhD

Diffuse large B-cell lymphoma (DLBCL) is the most common and aggressive subtype of non-Hodgkin lymphoma. The overall risk of developing DLBCL is increased in patients with other lymphomas, such as mycosis fungoides (MF). In this report, we present an 81-year-old female with early-stage MF who simultaneously progressed to tumor stage, large-cell transformed (LCT) MF and developed a primary DLBCL in a lymph node (LN). She presented with a tumor on her leg and new lymphadenopathy in her right axilla. Skin biopsy of the tumor revealed infiltration of large atypical CD3+, CD4+, and CD30+ cells, and a smaller portion of CD8+ cells in the dermis, consistent with LCT MF. Biopsy of the axillary LN revealed diffuse sheets of CD20+, BCL-2+, c-MYC+, and CD10 cells, highly suggestive of double expressor DLBCL. High-throughput sequencing revealed monoclonal T cells in the skin tumor and a monoclonal B-cell population in the LN. The above findings led to simultaneous diagnoses of LCT MF and nodal double expressor DLBCL. Our case demonstrates the importance of performing a full pathological workup in cutaneous T-cell lymphoma patients presenting with lymphadenopathy.

弥漫大 B 细胞淋巴瘤(DLBCL)是非霍奇金淋巴瘤中最常见、最具侵袭性的亚型。患有其他淋巴瘤(如放线菌病(MF))的患者罹患 DLBCL 的总体风险会增加。在本报告中,我们介绍了一名患有早期 MF 的 81 岁女性患者,她的病情同时发展为肿瘤分期、大细胞转化(LCT)MF,并在淋巴结(LN)中出现原发性 DLBCL。她出现腿部肿瘤和右腋窝淋巴结肿大。肿瘤的皮肤活检显示,真皮层有大量非典型CD3+、CD4+和CD30+细胞浸润,还有一小部分CD8+细胞,这与LCT MF一致。腋窝淋巴结活检发现CD20+、BCL-2+、c-MYC+和CD10-细胞弥漫成片,高度提示为双表达型DLBCL。高通量测序显示,皮肤肿瘤中有单克隆T细胞,而LN中有单克隆B细胞群。根据上述结果,我们同时确诊为 LCT MF 和结节双表达 DLBCL。我们的病例表明,对出现淋巴结病的皮肤T细胞淋巴瘤患者进行全面病理检查非常重要。
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引用次数: 0
Asymptomatic CD34+ plaque on the scalp of a child 儿童头皮上无症状的 CD34+ 斑块。
IF 1.6 4区 医学 Q3 DERMATOLOGY Pub Date : 2024-07-04 DOI: 10.1111/cup.14676
Tue F. Nguyen BS, Aeja Weiss MD, Emily Maxon DO, John C. Browning MD, Thomas Davis MD

Fibroblastic connective tissue nevus (FCTN) is a rare, benign dermal mesenchymal lesion of fibroblastic and myofibroblastic lineage. We report a case of a 2-year-old male who presented with an 18-month history of an erythematous, asymptomatic, unchanging dermal plaque on the right medial frontal scalp. A punch biopsy showed a disorderly, bland, dermal fibroblastic spindle cell proliferation extending to the superficial subcutis. It stained positive for CD34, and concern for dermatofibrosarcoma protuberans was raised. However, FISH was negative for PDGFB rearrangement, and the constellation of findings was most consistent with FCTN. This case underscores the importance of distinguishing CD34+ mesenchymal tumors for both dermatologists and dermatopathologists. As these represent a rather diverse group of lesions with different biological behaviors, a knowledge of the differential diagnosis of these entities is critical for proper patient management.

成纤维结缔组织痣(FCTN)是一种罕见的良性真皮间质病变,具有成纤维和成肌纤维的血统。我们报告了一例 2 岁男性患者的病例,他在 18 个月前发现右侧额头内侧头皮上有一个无症状、无变化的真皮红斑。打孔活检显示,真皮纤维母细胞纺锤形增生紊乱、平淡,一直延伸到皮下浅层。它的 CD34 染色阳性,引起了对原发性皮纤维肉瘤的担忧。然而,FISH 检测的 PDGFB 重排结果为阴性,而且这些结果与 FCTN 最为吻合。该病例强调了皮肤科医生和皮肤病理学家区分 CD34+ 间充质肿瘤的重要性。由于这些肿瘤代表了一组具有不同生物学行为的病变,因此了解这些实体的鉴别诊断对于正确处理病人至关重要。
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引用次数: 0
期刊
Journal of Cutaneous Pathology
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