American Journal of Dermatopathology最新文献

{"title":"Combined Intraepidermal-Intranuclear IgG and \"Full-House\" Immunoreactant Deposition at the Dermoepidermal Junction in Patients With Bullous Systemic Lupus Erythematosus.","authors":"Jonathan D Ho, Jamee Charles, Janelle Welch, Michael Fitz-Henley, Lilly Paul, Natasha Richards, Althea East-Innis","doi":"10.1097/DAD.0000000000003098","DOIUrl":"https://doi.org/10.1097/DAD.0000000000003098","url":null,"abstract":"<p><strong>Abstract: </strong>Bullous systemic lupus erythematosus (BSLE) is a relatively uncommon autoimmune bullous dermatosis presenting with bullae, neutrophil-rich subepidermal blisters, deposition of IgG, and frequently IgA, IgM, and C3 at the dermoepidermal junction (DEJ) in a \"full-house\" pattern. We describe the combination of intraepidermal, intranuclear IgG (IEIN), along with full-house DEJ deposition in patients with BSLE. Fifty-seven direct immunofluorescence images were reviewed for 6 years. IEIN deposition of IgG was identified in 8 patients: all with concurrent IgG/IgM/IgA/C3 in a linear/continuous granular pattern at the DEJ and a diagnosis consistent with BSLE. Eight of 11 (72.7%) patients with BSLE had dual patterns. All patients with dual patterns had blisters and 50% had concurrent urticarial lesions. Three carried a pre-existing diagnosis of systemic lupus erythematosus (SLE), while histopathologic/immunofluorescence findings triggered investigation and confirmation of systemic lupus erythematosus in 5. IEIN IgG had a speckled appearance and correlated with antinuclear antibody positivity (100%). IEIN speckled appearance likely represents antibodies against extractable nuclear antigens (ENA, evaluable in 6/8, present in all of these). Variable combinations of anti-ENAs were seen. In autoimmune bullous dermatosis evaluation, combined IEIN/DEJ suggests BSLE even if patients do not have a known SLE diagnosis. Antinuclear antibody and ENA panels should be recommended.</p>","PeriodicalId":50967,"journal":{"name":"American Journal of Dermatopathology","volume":" ","pages":""},"PeriodicalIF":1.0,"publicationDate":"2025-09-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145034368","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":"Histopathologic and Clinical Characteristics of Cutaneous Toxicities of Tyrosine Kinase Inhibitors: Insights Into Pathologic Mechanisms From a Retrospective Cohort.","authors":"Ian Nykaza, Tania Platero-Portillo, Ellin Berman, Mark B Geyer, Jae H Park, Konstantinos Linos, Alina Markova","doi":"10.1097/DAD.0000000000003125","DOIUrl":"https://doi.org/10.1097/DAD.0000000000003125","url":null,"abstract":"<p><strong>Background: </strong>Dermatologic adverse events (dAEs) are prevalent with BCR-ABL tyrosine kinase inhibitors (TKIs), affecting quality of life and treatment adherence. Despite their prevalence, underlying mechanisms of toxicity remain unclear. We sought to characterize dAEs across TKI generations to elucidate mechanisms driving toxicities.</p><p><strong>Methods: </strong>Retrospective cohort study of patients receiving imatinib, dasatinib, nilotinib, bosutinib, ponatinib, and asciminib at Memorial Sloan Kettering Cancer Center between 2001 and 2023 with dermatologic biopsy for TKI-related dAEs. Clinical and histopathologic characteristics were analyzed. Fisher exact test, with Freeman-Halton extension, was used to determine statistically significant differences.</p><p><strong>Results: </strong>Among 28 patients with 32 unique dAEs, imatinib was implicated in 32.1% of patients, second-generation TKIs in 57.1%, and ponatinib in 10.7%. Imatinib-induced dAEs exhibited significantly higher rates of papular/plaque morphology (P = 0.02) with acanthosis (P = 0.001) and eosinophilic infiltrates (P = 0.02). Second-generation TKIs frequently caused folliculocentric eruptions with adnexal involvement. Ponatinib-related dAEs demonstrated significantly higher rates of ichthyosiform (P = 0.002) and pityriasiform (P = 0.02) morphologies with hyperkeratosis and fibrosis. No biopsy-confirmed asciminib dAEs were available.</p><p><strong>Conclusions: </strong>dAE pathogenesis varies across TKI generations, suggesting distinct patterns of inflammation and keratinocyte dysregulation. Imatinib-related dAEs demonstrated epidermal hyperplasia and eosinophilic inflammation, suggesting immune-mediated hypersensitivity as an important mechanism of toxicity. Second-generation TKI dAEs demonstrated prominent adnexal and perivascular involvement, possibly driven by off-target inhibition and proinflammatory mechanisms. Ponatinib dAEs reflected chronic epidermal alterations, possibly driven by off-target vascular endothelial growth factor receptor, fibroblast growth factor receptor, and Src-family kinase inhibition. Limited sample size, particularly for third-generation TKIs, limits generalizability. Understanding these differences may improve management strategies, optimizing patient quality of life and treatment continuation.</p>","PeriodicalId":50967,"journal":{"name":"American Journal of Dermatopathology","volume":" ","pages":""},"PeriodicalIF":1.0,"publicationDate":"2025-09-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145024723","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":"Lichen Scrofulosorum-Like Eruption Colocalizing to the BCG Site.","authors":"Arun Somasundaram, Siddhartha Siddeswara, Laxmisha Chandrashekar, Sreerekha Jinkala","doi":"10.1097/DAD.0000000000003101","DOIUrl":"https://doi.org/10.1097/DAD.0000000000003101","url":null,"abstract":"","PeriodicalId":50967,"journal":{"name":"American Journal of Dermatopathology","volume":" ","pages":""},"PeriodicalIF":1.0,"publicationDate":"2025-09-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145001815","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":"A Photodistributed Eruption With Ring Mitoses: Challenge.","authors":"Natalie Bourand, Susan Pei","doi":"10.1097/DAD.0000000000003091","DOIUrl":"https://doi.org/10.1097/DAD.0000000000003091","url":null,"abstract":"","PeriodicalId":50967,"journal":{"name":"American Journal of Dermatopathology","volume":" ","pages":""},"PeriodicalIF":1.0,"publicationDate":"2025-09-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145001846","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":"Cutaneous AL-Type Amyloidomas as a Variant of Primary Cutaneous Marginal Zone Lymphoma: A Series of 30 Patients.","authors":"Sergio García-González, Mar García-García, Clara Miguel Miguel, Alicia Córdoba Iturriagagoitia, Juan I Yanguas Bayona, Laura Nájera Botello, Rita Cabeza Martínez, Ángel Santos-Briz Terrón, Juan Torre Castro, Eva Sánchez Martínez, Socorro M Rodríguez Pinilla, Uwe Hillen, Celia Requena Caballero, Lorenzo Cerroni, Sara P Martínez Cisneros, Victoria Lezcano Biosca, Elena Carracedo Vega, Francisco J Diaz de la Pinta, Lucía Prieto-Torres","doi":"10.1097/DAD.0000000000003124","DOIUrl":"https://doi.org/10.1097/DAD.0000000000003124","url":null,"abstract":"<p><strong>Abstract: </strong>AL-amyloidomas, particularly those primarily localized to the skin, present diagnostic and clinical challenges. They predominantly arise from immunoglobulin light chains, often due to plasma cell proliferation. The relationship between this entity, AL-amyloidomas, and primary cutaneous marginal zone lymphoma remains a subject of scientific debate. We conducted a retrospective, multicenter observational study, including 30 patients diagnosed with AL-amyloidomas. Demographic data, clinical manifestations, and histopathologic findings were reviewed. Various diagnostic techniques, such as light chain restriction, immunohistochemical analysis, polymerase chain reaction-based clonality testing, and next-generation sequencing, were used to further delineate the nature of these lesions. The cohort consisted of 12 men and 18 women, with a median age of 64.7 years. The most common clinical presentation involved plaques and nodules on the extremities and face. Histopathologically, monoclonal plasma cell infiltrates and amyloid deposits in the dermis and subcutis were observed. Most patients exhibited lambda light chain restriction. Molecular analysis revealed the presence of gene mutations in SPEN and TNFRSF13B in 1 patient. No cases progressed to systemic disease. Sjögren syndrome was the most frequently associated comorbidity. Our findings suggest that cutaneous AL-amyloidomas are indolent lesions and may represent a variant of primary cutaneous marginal zone lymphoma. These lesions exhibit distinct histopathologic features, such as amyloid deposition, and have a favorable prognosis with minimal risk of progression to systemic amyloidosis. This study supports the hypothesis that AL-amyloidomas are part of the broader spectrum of indolent B-cell lymphomas.</p>","PeriodicalId":50967,"journal":{"name":"American Journal of Dermatopathology","volume":" ","pages":""},"PeriodicalIF":1.0,"publicationDate":"2025-09-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144994355","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":"PRAME Expression in Recurrent/Persistent Melanocytic Nevi.","authors":"Alison H Kucharik, Helana Ghali, Doniya Milani, Elizabeth Warbasse, Philippa Li, Sairekha Ravichandran, Paul Rodriguez-Waitkus, Wei-Shen Chen","doi":"10.1097/DAD.0000000000003104","DOIUrl":"https://doi.org/10.1097/DAD.0000000000003104","url":null,"abstract":"<p><strong>Background: </strong>Recurrent melanocytic nevi are benign melanocytic proliferations that occur after incomplete excision of a nevus. Their atypical clinical and histopathologic features complicate diagnosis, especially without knowledge of prior biopsy. The PRAME (PReferentially expressed Antigen in MElanoma) immunohistochemical stain has been increasingly used to support a diagnosis of melanoma, however, its utility in recurrent melanocytic nevi is not well established.</p><p><strong>Methods: </strong>We analyzed PRAME expression in paired original and recurrent melanocytic nevi from 22 patients. Tissue sections underwent PRAME immunohistochemistry, evaluated by 2 dermatopathologists using 3 criteria: (1) staining intensity, (2) percentage of melanocytes with nuclear staining, and (3) a composite score.</p><p><strong>Results: </strong>Among original nevi, 81.8% had an intensity score of 0, 9.1% scored 1, 4.5% scored 2%, and 4.5% scored 3. Percentage positivity was 81.8% at score 0 and 18.2% at score 1. All had a negative composite score. Recurrent nevi showed similar results, with 81.8% intensity score 0, 9.1% score 1, 4.5% score 2, and 4.5% score 3. Percentage positivity was 81.8% at score 0 and 18.2% at score 1, with all patients scoring negative on the composite score.</p><p><strong>Conclusions: </strong>PRAME expression remained consistent between original and recurrent nevi, with all patients showing negative composite scores. These findings support PRAME's utility in differentiating benign recurrent nevi from melanoma, even when histologic or clinical features raise concern. PRAME staining may enhance diagnostic confidence, reducing unnecessary procedures.</p>","PeriodicalId":50967,"journal":{"name":"American Journal of Dermatopathology","volume":" ","pages":""},"PeriodicalIF":1.0,"publicationDate":"2025-09-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144994313","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":"Xanthogranulomatous Epithelial Tumor in a Pediatric Patient. A Potential Diagnostic Pitfall With Non-Langerhans Cell Histiocytic Neoplasms, Particularly Juvenile Xanthogranuloma.","authors":"Danielle V de Stefano, Jennifer Picarsic, Somak Roy, Eduardo Zambrano Tola","doi":"10.1097/DAD.0000000000003120","DOIUrl":"https://doi.org/10.1097/DAD.0000000000003120","url":null,"abstract":"<p><strong>Abstract: </strong>Xanthogranulomatous epithelial tumor is a recently described neoplasm harboring recurrent HMGA2::NCOR2 fusions, which, owing to its extensive xanthogranulomatous appearance with abundant foamy histiocytes and frequent Touton-type giant cells, may be confused with other inflammatory and neoplastic entities. We present a case arising in the right arm subcutaneous tissues of a 10-year-old boy, which was initially interpreted as a non-Langerhans cell histiocytic neoplasm compatible with Juvenile xanthogranuloma.</p>","PeriodicalId":50967,"journal":{"name":"American Journal of Dermatopathology","volume":" ","pages":""},"PeriodicalIF":1.0,"publicationDate":"2025-09-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144977662","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":"Lupus Panniculitis of the Scalp Presenting as a Patchy Nonscarring Alopecia: Report of an Uncommon Case.","authors":"Biswanath Behera, Madhusmita Sethy, Bhini Ameta, Farhan S R A Khatib, Pavithra Ayyanar","doi":"10.1097/DAD.0000000000003115","DOIUrl":"https://doi.org/10.1097/DAD.0000000000003115","url":null,"abstract":"<p><strong>Abstract: </strong>Diagnosing a case of patchy alopecia in the setting of lupus erythematosus (LE) can be clinically challenging. Of the various causes of LE-specific alopecias, lupus panniculitis of the scalp is rarely reported. A 40-year-old woman presented with a nonscarring patch of alopecia over the scalp. Trichoscopy showed multiple follicular plugging, multiple thin and dystrophic hair shafts, empty follicles, and regularly distributed pinpoint white dots within the lesion. The clinical diagnoses of alopecia areata or early discoid LE were considered. However, the histopathological examination of the scalp biopsy showed typical hyaline-type fat necrosis of the subcutis along with moderate perivascular and perifollicular inflammatory infiltrate without any interface dermatitis. On direct immunofluorescence, staining for IgG, IgA, IgM, and C3 was negative. A diagnosis of lupus panniculitis of the scalp, presenting as patchy nonscarring alopecia, was rendered. Treatment with oral prednisolone and methotrexate led to complete recovery of alopecia. In conclusion, we report a rare case of lupus panniculitis of the scalp and discuss its differential diagnosis in the setting of LE.</p>","PeriodicalId":50967,"journal":{"name":"American Journal of Dermatopathology","volume":" ","pages":""},"PeriodicalIF":1.0,"publicationDate":"2025-09-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144977628","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":"Methotrexate-Associated Primary Cutaneous Epstein-Barr Virus-Positive Polymorphic B-Cell Lymphoproliferative Disorder With Metachronous T-Cell Clonally Related Angioimmunoblastic T-Cell Lymphoma: A Case Report and Review of the Literature.","authors":"Tiffany Liu, Patrick M Brunner, Olga Marushchak, Chrisanna Dobrowolski, Christian Salib, Cynthia Magro","doi":"10.1097/DAD.0000000000003116","DOIUrl":"https://doi.org/10.1097/DAD.0000000000003116","url":null,"abstract":"<p><strong>Abstract: </strong>Primary cutaneous Epstein-Barr virus (EBV)-positive polymorphic B-cell lymphoproliferative disorder (LPD) is a rare LPD associated with iatrogenic and endogenous immune dysregulation with the commonest risk factor being immunosuppressive therapy. We present a 55-year-old woman with rheumatoid arthritis, previously on methotrexate, who developed a waxing and waning papulonodular eruption on the chest and neck. Histopathology revealed a lymphohistiocytic infiltrate with atypical EBV+/CD30+ B cells, consistent with EBV+ polymorphic B-cell LPD. Although discontinuation of methotrexate initially resulted in complete lesional resolution, recurrence occurred 2 years later while the patient was on rituximab therapy. The patient subsequently developed lymphadenopathy, and biopsy confirmed angioimmunoblastic T-cell lymphoma. Notably, earlier skin samples harbored the same T-cell clone found in the lymph node. This case underscores the dynamic evolution of EBV+ polymorphic B-cell LPD, where an initial clonal T-cell response to infected atypical B cells eventuated into angioimmunoblastic T-cell lymphoma.</p>","PeriodicalId":50967,"journal":{"name":"American Journal of Dermatopathology","volume":" ","pages":""},"PeriodicalIF":1.0,"publicationDate":"2025-09-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144994286","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":"A Case of Sarcoidal Granulomatous Dermatitis and Vasculitis in a 28-Year-Old Woman.","authors":"Allison Bai, Saman Namazian, Alejandro Gru, Erika Elliott","doi":"10.1097/DAD.0000000000003121","DOIUrl":"https://doi.org/10.1097/DAD.0000000000003121","url":null,"abstract":"<p><strong>Abstract: </strong>Granulomatous vasculitis represents a rare cutaneous manifestation of sarcoidosis, a multisystem disease characterized by noncaseating granulomas. We report the case of a 28-year-old woman with new-onset tender, nonpruritic, erythematous papules coalescing into plaques and subcutaneous nodules on her lower legs, accompanied by anterior uveitis, exertional dyspnea, and constitutional symptoms including fatigue and night sweats. Punch biopsy revealed non-necrotizing epithelioid granulomas with vasculitic changes, consistent with cutaneous sarcoidal granulomatous vasculitis. Although granulomatous vasculitis is well recognized in pulmonary sarcoidosis, its presence in the skin can complicate diagnosis and delay appropriate therapy. This report highlights the value of histopathologic evaluation of suspicious lesions in patients with possible sarcoidosis. Collaborative care between dermatology, rheumatology, ophthalmology, and pulmonology is key for timely intervention and improved patient outcomes.</p>","PeriodicalId":50967,"journal":{"name":"American Journal of Dermatopathology","volume":" ","pages":""},"PeriodicalIF":1.0,"publicationDate":"2025-09-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144977553","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}