{"title":"Storiform Collagenomas With Unusual Morphology: Expanding the Histologic Spectrum in Two Molecularly Proven Cases.","authors":"Amanda Scopelliti, Pranav Dorwal, Alison Cheah","doi":"10.1111/cup.70218","DOIUrl":"https://doi.org/10.1111/cup.70218","url":null,"abstract":"<p><p>Storiform collagenoma is a rare benign dermal neoplasm recently shown to harbor concurrent PTEN and PDGFRB mutations. While most are sporadic, a subset occurs in the context of Cowden syndrome. We describe two storiform collagenomas with unusual morphology including infiltrative growth pattern and perivascular myoid differentiation. Confirmation of concurrent PTEN and PDGFRB mutations in both cases allowed for a more confident diagnosis of storiform collagenoma in these cases and their distinction from dermatofibrosarcoma protuberans.</p>","PeriodicalId":15407,"journal":{"name":"Journal of Cutaneous Pathology","volume":" ","pages":""},"PeriodicalIF":1.2,"publicationDate":"2026-09-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149012203","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":"Acute Myeloid Leukemia Involving the Skin.","authors":"Anna Sarah Erem, Alejandro A Gru","doi":"10.1111/cup.70219","DOIUrl":"https://doi.org/10.1111/cup.70219","url":null,"abstract":"<p><p>Leukemia cutis (LC) refers to cutaneous infiltration by leukemic cells, most often by acute myeloid leukemia (AML) in adults and by lymphoblastic leukemia in children. Although uncommon, LC is a clinically significant manifestation of extramedullary disease and may precede, coincide with, or follow bone marrow involvement. Recognition of AML-associated LC is essential, as it can be diagnostically challenging due to histopathologic overlap with inflammatory dermatoses and other cutaneous hematolymphoid neoplasms. Recent cohort-based studies have systematically characterized AML involving the skin, demonstrating frequent immunophenotypic discordance between cutaneous and marrow blasts and recurrent genetic associations, with important diagnostic and prognostic implications for dermatopathologists. This review provides a practical framework for current and historical nomenclature, the diagnosis of cutaneous AML, and key histopathologic patterns and pitfalls.</p>","PeriodicalId":15407,"journal":{"name":"Journal of Cutaneous Pathology","volume":" ","pages":""},"PeriodicalIF":1.2,"publicationDate":"2026-09-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149012201","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}
Arthur William Passos Raetsch, Richard C K Jordan, Steven D Billings, Laura B Pincus
{"title":"Pediatric Superficial Malignant Perineurioma/Perineuriomatous Malignant Peripheral Nerve Sheath Tumor of the Groin With NF1 Loss: A Case Report and Focused Review of the Literature.","authors":"Arthur William Passos Raetsch, Richard C K Jordan, Steven D Billings, Laura B Pincus","doi":"10.1111/cup.70215","DOIUrl":"https://doi.org/10.1111/cup.70215","url":null,"abstract":"<p><p>Malignant perineurioma, also referred to as perineuriomatous malignant peripheral nerve sheath tumor (MPNST), is an exceptionally rare neoplasm with limited clinicopathologic characterization, particularly in children. We report a 12-year-old boy with a 3.0-cm superficial right groin lesion. Histopathologic examination showed a spindle cell neoplasm with fascicular-to-swirling architecture in myxoid to collagenous stroma, moderate cytologic atypia and nuclear variability, mitotic activity, focal necrosis, and admixed inflammation. Epithelial membrane antigen, GLUT1, and claudin-1 positivity supported perineurial differentiation, while a broad negative immunohistochemical panel helped exclude potential mimics. Tumor-only sequencing demonstrated 17q loss with focal homozygous NF1 deletion, a BRCA2 frameshift, and low tumor mutational burden (3.4 mutations/Mb); RNA sequencing identified no pathogenic gene fusion. These molecular findings were interpreted as adjunctive rather than independently diagnostic. The tumor extended to the inked specimen edge, and subsequent management and follow-up information was unavailable. To our knowledge, this is the first reported superficial cutaneous malignant perineurioma/perineuriomatous MPNST in a pediatric patient.</p>","PeriodicalId":15407,"journal":{"name":"Journal of Cutaneous Pathology","volume":" ","pages":""},"PeriodicalIF":1.2,"publicationDate":"2026-09-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148990076","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}
Toan S Bui, Nicholas Urbanski, Damien Abreu, Jaroslaw Jedrych, Cole Sterling, Sima Rozati
{"title":"New Annular, Indurated Plaques During Mogamulizumab Therapy in Cutaneous T-Cell Lymphoma: A Clinicopathologic Pitfall.","authors":"Toan S Bui, Nicholas Urbanski, Damien Abreu, Jaroslaw Jedrych, Cole Sterling, Sima Rozati","doi":"10.1111/cup.70209","DOIUrl":"https://doi.org/10.1111/cup.70209","url":null,"abstract":"<p><p>New annular, indurated plaques arising during mogamulizumab therapy for cutaneous T-cell lymphoma (CTCL) present a diagnostic challenge, as they may raise concern for treatment-related eruption or disease progression. We report two patients with darker skin types who developed plaques that were morphologically distinct from their baseline presentations during mogamulizumab therapy. These morphologic changes may be subtle, manifesting as increased induration, textural change, or plaque thickening, and can be easily overlooked if not assessed in the context of prior disease morphology. Despite clinical changes suggestive of alternative pathology, histopathologic evaluation in both cases demonstrated persistent or progressive mycosis fungoides. Immunophenotyping and T-cell receptor gene rearrangement studies further supported lymphoma involvement rather than drug-induced eruptions. These cases illustrate clinicopathologic discordance, in which changes in clinical morphology do not reliably reflect the underlying pathology. Accurate classification requires a structured, algorithmic approach incorporating repeat biopsy, histopathologic assessment, immunophenotyping, molecular studies, and comparison with prior clinical and pathologic findings over time as well as appropriate restaging. Recognition of this diagnostic pitfall, particularly in patients with darker skin types, is essential to guide appropriate management during mogamulizumab therapy.</p>","PeriodicalId":15407,"journal":{"name":"Journal of Cutaneous Pathology","volume":" ","pages":""},"PeriodicalIF":1.2,"publicationDate":"2026-09-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148968632","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}
Hannah Moorman, Jennifer Crimmins, Joseph Gillam, Scott Turner, Mark C Mochel
{"title":"Epithelioid Fibrous Histiocytoma With Bulbous Subcutaneous Extension and SQSTM1::ALK Fusion.","authors":"Hannah Moorman, Jennifer Crimmins, Joseph Gillam, Scott Turner, Mark C Mochel","doi":"10.1111/cup.70201","DOIUrl":"https://doi.org/10.1111/cup.70201","url":null,"abstract":"","PeriodicalId":15407,"journal":{"name":"Journal of Cutaneous Pathology","volume":" ","pages":""},"PeriodicalIF":1.2,"publicationDate":"2026-09-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148963358","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}
Steven M Hrycaj, Joseph S Durgin, Johann E Gudjonsson, J Michelle Kahlenberg, Gaidaa R Majari, Carole Bitar, Catherine G Chung, Rosalynn M Nazarian, May P Chan
{"title":"Utility of IL-18 Immunohistochemistry in Distinguishing Dermatomyositis and Lupus Erythematosus in Skin Biopsies.","authors":"Steven M Hrycaj, Joseph S Durgin, Johann E Gudjonsson, J Michelle Kahlenberg, Gaidaa R Majari, Carole Bitar, Catherine G Chung, Rosalynn M Nazarian, May P Chan","doi":"10.1111/cup.70214","DOIUrl":"https://doi.org/10.1111/cup.70214","url":null,"abstract":"<p><strong>Background: </strong>Distinction of dermatomyositis (DM) and cutaneous lupus erythematosus (LE) in skin biopsies is often difficult due to significant histopathologic overlap. Prior transcriptional analysis has identified upregulation of IL-18 in DM compared with LE. We aimed to assess its utility as an immunohistochemical marker in this differential diagnosis.</p><p><strong>Methods: </strong>Skin biopsies classified as DM (n = 35) or LE (n = 32) were immunostained with an IL-18 antibody. A positive IL-18 result was defined as presence of full-thickness epidermal staining of medium or strong intensity. Absent, weak, or partial-thickness staining was considered negative. Results were compared between groups by Fisher's exact test.</p><p><strong>Results: </strong>IL-18 expression in keratinocytes, when present, was consistently cytoplasmic with or without nuclear positivity. Twenty-eight (80%) DM cases and 11 (34%) LE cases were positive for IL-18 (p = 0.0002). A positive result had a sensitivity of 80% and a specificity of 66% for DM. Specifically, strong full-thickness staining was only observed in DM (five cases; p = 0.05), whereas complete absence of epidermal staining was seen exclusively in LE (five cases; p = 0.02).</p><p><strong>Conclusions: </strong>IL-18 immunohistochemistry can be a useful diagnostic adjunct in distinguishing DM and LE in a small subset of skin biopsies when staining is strong or completely absent. Correlation with clinical and histopathologic findings remains imperative.</p>","PeriodicalId":15407,"journal":{"name":"Journal of Cutaneous Pathology","volume":" ","pages":""},"PeriodicalIF":1.2,"publicationDate":"2026-09-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148963387","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}
Seung-Soo Lee, Seok-Jong Lee, Weon Ju Lee, Dae-Lyong Ha
{"title":"Solitary Indeterminate Cell Histiocytosis Presenting as a Reddish Nodule on the Abdomen of a 4-Year-Old Boy: Clinicopathologic Correlation and Electron Microscopy Findings.","authors":"Seung-Soo Lee, Seok-Jong Lee, Weon Ju Lee, Dae-Lyong Ha","doi":"10.1111/cup.70210","DOIUrl":"https://doi.org/10.1111/cup.70210","url":null,"abstract":"<p><p>Indeterminate cell histiocytosis (ICH) is a clonal proliferative disorder of mononuclear phagocyte cells that shows features of both dendritic and histiocytic cells. Only around 100 cases of ICH were reported, and among them, only 24% of reported cases involve pediatric patients, with a mean age at diagnosis of 50 years. We report an exceptionally rare case of solitary ICH in a 4-year-old boy. A 4-year-old visited our department presenting a solitary asymptomatic bean-sized reddish nodule on upper abdomen for 1 year. Initially thought to be keloid or dermatofibroma, an excisional biopsy was performed. Histopathological examination revealed dense cellular infiltration in the dermis, characterized by medium-sized mononuclear histiocytes displaying abundant glassy eosinophilic cytoplasm and indented or folded nuclei. Immunohistochemistry studies confirmed positivity for S-100 protein and CD1a, coupled with negativity for CD207/Langerin. Electron microscopy revealed the absence of Birbeck granules, leading to the diagnosis of ICH. With no remarkable findings in laboratory and imaging studies, the patient is undergoing regular follow-up to monitor any potential recurrence or development of related conditions. While most cases of skin-restricted ICH follow an indolent course, recognizing the reported association between ICH and other hematologic neoplasms is crucial. Therefore, awareness of ICH is essential, and in cases of suspicion, a skin biopsy should be performed for an accurate diagnosis.</p>","PeriodicalId":15407,"journal":{"name":"Journal of Cutaneous Pathology","volume":" ","pages":""},"PeriodicalIF":1.2,"publicationDate":"2026-09-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148963459","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}
Ashley Gochoco, Sushila A Toulmin, Christopher Iriarte, Joshua F Goldsmith
{"title":"Non-Caseating Granulomatous Reaction to Red Tattoo Pigment in a Patient Receiving Immune Checkpoint Inhibitor Therapy.","authors":"Ashley Gochoco, Sushila A Toulmin, Christopher Iriarte, Joshua F Goldsmith","doi":"10.1111/cup.70213","DOIUrl":"https://doi.org/10.1111/cup.70213","url":null,"abstract":"<p><p>Cutaneous adverse reactions associated with tattoos exhibit various histopathologic patterns, ranging from allergic or interface dermatitis to sarcoid-like granulomatous inflammation. Immune checkpoint inhibitors (ICIs) are also well known to induce granulomatous cutaneous eruptions, usually with sarcoid-like features. Interestingly, ICIs can enhance immune responses at previous antigen exposure sites, including tattoos. Herein, we report a case of a non-caseating granulomatous tattoo reaction associated with red pigment in the context of ICI therapy. Histopathologically, the granulomas exhibited a tuberculoid architecture characterized by prominent peripheral lymphoplasmacytic inflammation rather than the relatively \"naked\" granulomas more typical of sarcoidal reactions. This case highlights the morphologic heterogeneity of granulomatous reactions arising within tattoos during ICI therapy and underscores the complex interaction between host immunity, tattoo pigment, and immunotherapy. Awareness of this pattern may help prevent misclassification as cutaneous sarcoidosis or infection and guide clinical care for patients on ICIs who develop such granulomatous reactions.</p>","PeriodicalId":15407,"journal":{"name":"Journal of Cutaneous Pathology","volume":" ","pages":""},"PeriodicalIF":1.2,"publicationDate":"2026-09-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148929619","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":"Delayed-Type Hypersensitivity With Prominent Langerhans Cell Microabscesses in Response to a Dendritic Cell/AML Fusion Vaccine.","authors":"Ashley Gochoco, Steven R Tahan","doi":"10.1111/cup.70212","DOIUrl":"https://doi.org/10.1111/cup.70212","url":null,"abstract":"","PeriodicalId":15407,"journal":{"name":"Journal of Cutaneous Pathology","volume":" ","pages":""},"PeriodicalIF":1.2,"publicationDate":"2026-09-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148912244","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}
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}