Seung Hyo Cho, Su Min Lee, Jee-Bum Lee, Sook Jung Yun
{"title":"Cutaneous Langerhans Cell Sarcoma Harboring BRAF V600E Mutation Followed by Chronic Myelomonocytic Leukemia and EBV-Positive Diffuse Large B-Cell Lymphoma.","authors":"Seung Hyo Cho, Su Min Lee, Jee-Bum Lee, Sook Jung Yun","doi":"10.1111/cup.70208","DOIUrl":"https://doi.org/10.1111/cup.70208","url":null,"abstract":"<p><p>Langerhans cell sarcoma (LCS) is an extremely rare and aggressive neoplasm of histiocytic and dendritic cell lineage. We report a case of cutaneous LCS in a 74-year-old woman, harboring a BRAF V600E mutation and demonstrating an aggressive clinical course, including multiple metastatic lesions. During follow-up, the patient was diagnosed with chronic myelomonocytic leukemia and subsequently developed Epstein-Barr virus-positive diffuse large B-cell lymphoma. Additional molecular analyses demonstrated BRAF V600E in the primary LCS but not in the CMML bone marrow or the EBV-positive DLBCL, arguing against a common BRAF-driven clonal origin. Although an earlier shared founder alteration between LCS and CMML cannot be excluded, the subsequent EBV-positive DLBCL most likely developed independently in the setting of CMML-associated immune dysregulation. This case highlights the complexity of multiple hematologic neoplasms and emphasizes the importance of integrated clinicopathologic and molecular evaluation in rare histiocytic neoplasms.</p>","PeriodicalId":15407,"journal":{"name":"Journal of Cutaneous Pathology","volume":" ","pages":""},"PeriodicalIF":1.2,"publicationDate":"2026-09-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148900703","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}
V Pfefferle, A Vogelsberg, M Maaß, C M Schürch, S Forchhammer
{"title":"Aberrant Expression of Multiple T-Cell Markers in Primary Cutaneous Follicle Center Lymphoma: A Rare Case Report.","authors":"V Pfefferle, A Vogelsberg, M Maaß, C M Schürch, S Forchhammer","doi":"10.1111/cup.70207","DOIUrl":"https://doi.org/10.1111/cup.70207","url":null,"abstract":"<p><p>Aberrant expression of T-cell markers in B-cell lymphomas is rare and can pose diagnostic challenges. We report a case of a 65-year-old man presenting with newly developed scalp nodules. Histopathology revealed nodular lymphoid infiltrates composed of large centrocytes and centroblasts. Immunohistochemistry demonstrated CD20, BCL6, CD10, and MEF2B positivity with monotypic kappa light chain expression, confirming B-cell lineage. Notably, aberrant co-expression of multiple T-cell markers (CD2, CD3, CD4, and partial CD5), with weaker intensity than surrounding T-cells, was observed. Molecular studies showed clonal immunoglobulin heavy chain rearrangement with a polyclonal T-cell receptor pattern. These findings established the diagnosis of primary cutaneous follicle center lymphoma (PCFCL) with aberrant T-cell marker expression. This represents the first reported case of PCFCL with aberrant expression of multiple T-cell markers, emphasizing the importance of comprehensive immunophenotyping for accurate lymphoma classification.</p>","PeriodicalId":15407,"journal":{"name":"Journal of Cutaneous Pathology","volume":" ","pages":""},"PeriodicalIF":1.2,"publicationDate":"2026-09-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148900755","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}
Francisco Javier Díaz de la Pinta, Iván Prieto-Potín, Rebeca Manso Alonso, Juan Torre Castro, Cristina Serrano del Castillo, Jesús Frutos Díaz-Alejo, Rocío Nieves Salgado, Socorro María Rodríguez Pinilla
{"title":"Cutaneous-Only Relapse in T-Cell Prolymphocytic Leukemia With JAK2 Rearrangement","authors":"Francisco Javier Díaz de la Pinta, Iván Prieto-Potín, Rebeca Manso Alonso, Juan Torre Castro, Cristina Serrano del Castillo, Jesús Frutos Díaz-Alejo, Rocío Nieves Salgado, Socorro María Rodríguez Pinilla","doi":"10.1111/cup.70076","DOIUrl":"10.1111/cup.70076","url":null,"abstract":"","PeriodicalId":15407,"journal":{"name":"Journal of Cutaneous Pathology","volume":"53 10","pages":"874-877"},"PeriodicalIF":1.2,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147390139","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}
Jessica L. Muldoon, Laura M. Warmke, Ahmed K. Alomari, Rachel P. Kowal
{"title":"Superficial Ewing Sarcoma of the Rectum: A Case Report and the Utility of Molecular Diagnostics","authors":"Jessica L. Muldoon, Laura M. Warmke, Ahmed K. Alomari, Rachel P. Kowal","doi":"10.1111/cup.70128","DOIUrl":"10.1111/cup.70128","url":null,"abstract":"<p>Ewing sarcoma is an undifferentiated small round cell sarcoma that most commonly presents as a malignant bone tumor in pediatric and young adult patients. The diagnosis is typically confirmed by molecular genetic identification of a fusion protein, most commonly involving members of the FET and ETS gene families. The most frequent translocation is <i>EWSR1</i>::<i>FLI1</i> which occurs in approximately 85% of cases. Rare extraosseous cases of superficial Ewing sarcoma have been described, including one molecularly confirmed tumor involving the rectum. Herein, we report the second molecularly confirmed case of a superficial, rectal primary tumor arising in a 38-year-old male who initially presented with bleeding. Physical exam noted an ulcerated rectal polyp near the dentate line. Excisional biopsy of the lesion revealed a monotonous round cell malignancy involving the submucosal tissue with necrosis and marked mitotic activity. Immunohistochemical studies showed reactivity for CD99 and NKX2.2 with weak, focal expression of synaptophysin. Next-generation sequencing revealed an <i>EWSR1</i>::<i>FLI</i> fusion, confirming the diagnosis of Ewing sarcoma. The fusion variant junction was located in exon 7 for EWSR1 and exon 6 for FLI1. Subsequent imaging revealed no evidence of metastatic disease, and re-excision showed no residual tumor. Primary superficial Ewing sarcoma is extremely rare, and this is only the second molecularly confirmed case of rectal origin.</p>","PeriodicalId":15407,"journal":{"name":"Journal of Cutaneous Pathology","volume":"53 10","pages":"917-920"},"PeriodicalIF":1.2,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/cup.70128","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147838554","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Aubrey Nicole Montebello, Michael R. Lewin-Smith, Liesl Grenier, Stacy L. Strausborger, Michael Royer
{"title":"Photodistributed Hyperpigmentation in a Chronic Kratom User: Histologic and Elemental Characterization","authors":"Aubrey Nicole Montebello, Michael R. Lewin-Smith, Liesl Grenier, Stacy L. Strausborger, Michael Royer","doi":"10.1111/cup.70126","DOIUrl":"10.1111/cup.70126","url":null,"abstract":"<div>\u0000 \u0000 <p>Kratom (<i>Mitragyna speciosa</i>) is a plant-derived supplement increasingly used in the United States for chronic pain and mood elevation due to its opioid-like effects. While systemic side effects have been described, cutaneous manifestations have rarely been reported. We report a case of striking photodistributed dyspigmentation in a chronic kratom user, with a detailed description of the histologic and elemental analysis findings. A 37-year-old Soldier taking kratom for lower back pain presented with asymptomatic blue-gray pigmentation involving his face, neck, and dorsal hands. Biopsy revealed pigment-laden macrophages within the superficial dermis, a mild perivascular and periadnexal lymphohistiocytic infiltrate, and a slightly thickened basement membrane. Special stains confirmed the pigmented particles as melanin-like. Scanning electron microscopy with energy-dispersive X-ray analysis (SEM-EDXA) demonstrated sulfur-containing material, consistent with phaeomelanin and/or a drug metabolite melanin-like complex. This case expands the clinicopathologic understanding of kratom-associated dermatoses and illustrates the utility of SEM-EDXA in identifying exogenous or drug-induced pigmentation.</p>\u0000 </div>","PeriodicalId":15407,"journal":{"name":"Journal of Cutaneous Pathology","volume":"53 10","pages":"925-929"},"PeriodicalIF":1.2,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147868509","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}
Susan Pei, Maxwell A. Fung, Melissa Piliang, Adam I. Rubin
{"title":"Kratom Cutaneous Hyperpigmentation: An Emerging Dermatosis for Dermatopathologists and Dermatologists to Recognize","authors":"Susan Pei, Maxwell A. Fung, Melissa Piliang, Adam I. Rubin","doi":"10.1111/cup.70175","DOIUrl":"10.1111/cup.70175","url":null,"abstract":"","PeriodicalId":15407,"journal":{"name":"Journal of Cutaneous Pathology","volume":"53 10","pages":"871-873"},"PeriodicalIF":1.2,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148430535","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}
{"title":"Plasmablastic Lymphoma Presenting With Multiple Cutaneous Nodules Mimicking Primary Cutaneous Marginal Zone Lymphoma","authors":"Bo-Jung Chen, Feng-Jie Lai, Yin-Hsun Feng, Shih-Sung Chuang","doi":"10.1111/cup.70137","DOIUrl":"10.1111/cup.70137","url":null,"abstract":"<div>\u0000 \u0000 <p>Plasmablastic lymphoma (PBL) is a rare, high-grade large B-cell lymphoma characterized by large atypical cells with plasmablastic or immunoblastic morphology and a terminal B-cell differentiation phenotype. PBL often occurs in patients with immune deficiency or dysregulation, most commonly HIV infection. Cutaneous involvement is rare and occurs most frequently in those with immune deficiency. We report a 73-year-old immunocompetent female presenting with multiple cutaneous lesions and cervical lymphadenopathy. Nasopharyngeal and cutaneous biopsies confirmed EBV-associated PBL with plasmacytic differentiation, mimicking primary cutaneous marginal zone lymphoma (PC-MZL). The patient was treated with multiagent chemotherapy. Her disease progressed and she passed away in 9 months. We reviewed the literature for EBV-associated PBL occurring in the skin and those in immunocompetent patients. A comprehensive systemic evaluation integrating clinical, laboratory, and imaging findings is essential for excluding PC-MZL and plasmablastic multiple myeloma and establishing the correct diagnosis.</p>\u0000 </div>","PeriodicalId":15407,"journal":{"name":"Journal of Cutaneous Pathology","volume":"53 10","pages":"930-936"},"PeriodicalIF":1.2,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147868481","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}
Sydney Kenney, Andrea Acosta, Jason Castillo, David Cassarino, Aparche Yang
{"title":"Rare Presentation of TEN-Like Subacute Cutaneous Lupus Erythematosus Occurring in the Setting of Donepezil Exposure and COVID-19 Infection: A Diagnostic Challenge","authors":"Sydney Kenney, Andrea Acosta, Jason Castillo, David Cassarino, Aparche Yang","doi":"10.1111/cup.70109","DOIUrl":"10.1111/cup.70109","url":null,"abstract":"<div>\u0000 \u0000 <p>Toxic epidermal necrolysis (TEN) is a life-threatening mucocutaneous reaction characterized by rapidly progressive epidermal necrosis within hours to days, extensive skin detachment, and high mortality. Rarely, patients present with TEN-like eruptions that evolve slowly, over days to weeks, and have skin changes mostly confined to photodistributed areas, with possible mucosal involvement. The latter scenario is compatible with TEN-like lupus erythematosus. We report a 65-year-old man without prior lupus history who developed a widespread, photodistributed erythematous patchy eruption within 24 h of initiating donepezil for Parkinsonism-associated aphasia. The erythema rapidly progressed to erosions, bullae, and detachment involving 81% total body surface area (TBSA) and a Severity-of-Illness Score for Toxic Epidermal Necrolysis (SCORTEN) of 2. Initial differential diagnoses included TEN/Stevens Johnson Syndrome (SJS), bullous pemphigoid, generalized bullous fixed drug eruption, Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS), and reactive infectious mucocutaneous eruption (RIME). Histopathology revealed interface dermatitis with diffuse basilar apoptosis, focal full-thickness epidermal necrosis and robust dermal mucin deposits. Direct immunofluorescence study was negative. Serologies showed strongly positive anti-Ro/SSA and ANA titers. COVID-19 testing was positive. The constellation of clinical, pathologic and laboratory features supported a diagnosis of TEN-like subcutaneous lupus erythematosus (SCLE), likely triggered by donepezil and complicated by a concurrent COVID-19 infection. To our knowledge, this is a novel case of TEN-like SCLE associated with donepezil initiation.</p>\u0000 </div>","PeriodicalId":15407,"journal":{"name":"Journal of Cutaneous Pathology","volume":"53 10","pages":"946-954"},"PeriodicalIF":1.2,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147874380","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}
{"title":"Dermal-Based Spindle Cell Lipoma: A Case Series of 12 Patients","authors":"Luke J. Debenham, Gauri Panse, Christine J. Ko","doi":"10.1111/cup.70146","DOIUrl":"10.1111/cup.70146","url":null,"abstract":"<div>\u0000 \u0000 <p>Spindle cell lipoma (SCL) is a benign adipocytic neoplasm that typically presents as a subcutaneous mass in the “shawl” distribution of older men. This classic prototype is well recognized; however, the clinicopathologic features of dermal variants remain incompletely characterized. This case series includes 12 dermally located SCLs from our archives. Morphologic features were evaluated by consensus, and immunohistochemistry for RB1 and CD117 was performed on available tissue. The cohort included eight men and four women, with a mean age of 55 years. Half of the cases (50%) arose in atypical locations, most commonly the face. Histologically, tumors frequently deviated from the classic morphology: 50% were fat-free or fat-poor, 83% were poorly circumscribed, 33% showed myxoid change, and 16% displayed pseudoangiomatous features. All cases where immunohistochemistry could be performed showed RB1 loss (6 cases) and abundant CD117-positive mast cells (7 cases). Dermal SCL differs from the classic subcutaneous prototype. They are more likely to affect women, occur in atypical sites, be poorly circumscribed, and have fat-free or fat-poor morphologies. These deviations from the prototypical SCL illness script are diagnostically challenging, since they mimic other spindle cell neoplasms, such as neurofibroma, <i>PRRX1</i>-rearranged fibroblastic tumor, and a variety of spindle cell lesions that lack RB1 expression. CD117-positive mast cells may be helpful diagnostically.</p>\u0000 </div>","PeriodicalId":15407,"journal":{"name":"Journal of Cutaneous Pathology","volume":"53 10","pages":"955-960"},"PeriodicalIF":1.2,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147987761","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}
Ikuko Hirai, Joseph F. Sobanko, Tara C. Mitchell, Jacquelyn Roth, Rosalie Elenitsas, Emily Y. Chu
{"title":"Multicentric Asynchronous Endocrine Mucin-Producing Sweat Gland Carcinoma and Mucinous Carcinoma of the Skin","authors":"Ikuko Hirai, Joseph F. Sobanko, Tara C. Mitchell, Jacquelyn Roth, Rosalie Elenitsas, Emily Y. Chu","doi":"10.1111/cup.70122","DOIUrl":"10.1111/cup.70122","url":null,"abstract":"<p>Endocrine mucin-producing sweat gland carcinoma (EMPSGC) is an adnexal neoplasm which typically occurs on periorbital skin and demonstrates overlapping histopathologic features with primary mucinous carcinoma of the skin (MCS). Herein, we report a patient who developed five distinct lesions of EMPSGC and MCS over an eight-year period, some of which occurred on periorbital sites and others which occurred on the lower face. We further sought to better characterize these tumors on a molecular basis; targeted next generation sequencing analysis of two discrete tumors revealed identical missense variants within the <i>ATRX</i> gene. Our report expands on the rare prior reports of multicentric, asynchronous EMPSGC and MCS, and also provides a basis for additional genetic classification of these neoplasms.</p>","PeriodicalId":15407,"journal":{"name":"Journal of Cutaneous Pathology","volume":"53 10","pages":"901-906"},"PeriodicalIF":1.2,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/cup.70122","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147772809","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}