Angela Mei, Benjamin Allanson, Dustin Hall, Nima Mesbah Ardakani, Nathan Tobias Harvey
{"title":"Cutaneous Involvement by Refractory Celiac Disease Type 2 Histologically Mimicking Mycosis Fungoides.","authors":"Angela Mei, Benjamin Allanson, Dustin Hall, Nima Mesbah Ardakani, Nathan Tobias Harvey","doi":"10.1097/DAD.0000000000002793","DOIUrl":"10.1097/DAD.0000000000002793","url":null,"abstract":"<p><strong>Abstract: </strong>Refractory celiac disease (RCD) is a rare condition characterized by persistent malabsorptive symptoms and villous atrophy despite a gluten-free diet. While RCD type 1 has a normal intraepithelial lymphocyte phenotype, RCD type 2 is defined by the presence of immunophenotypically aberrant and monoclonal intraepithelial T lymphocytes, with a high propensity to transform to enteropathy-associated T-cell lymphoma (EATL). Although dermatological manifestations of celiac disease are common, presentation with cutaneous involvement by abnormal lymphocytes of RCD type 2 or EATL is rare, with few histologic descriptions in the literature. We describe the case of a 66-year-old man with a history of celiac disease presenting with a generalized, erythematous papular rash over his torso, upper arms, and legs. Biopsy of his skin lesions showed prominent hyperkeratosis with underlying spongiosis and interface change. Increased intraepithelial (epidermotropic) lymphocytes were observed, out of proportion to the level of spongiosis, but not overly atypical in appearance. Immunohistochemistry revealed an aberrant T-cell immunophenotype (CD3/2/7 positive; CD5/4/8 negative), raising suspicion for a cutaneous T-cell lymphoproliferative disorder. A duodenal biopsy demonstrated total villous atrophy with a morphologically bland population of epitheliotropic T lymphocytes showing the same aberrant immunophenotype. Similar cells were also identified by flow cytometry in the peripheral blood. In conjunction with the history of celiac disease, a diagnosis of RCD type 2 or 'EATL in situ' with cutaneous involvement was made. Cutaneous RCD type 2 or EATL should be considered as differential diagnoses in patients with a history of celiac disease and histopathology reminiscent of epidermotropic forms of cutaneous T-cell lymphoma.</p>","PeriodicalId":50967,"journal":{"name":"American Journal of Dermatopathology","volume":null,"pages":null},"PeriodicalIF":1.1,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141621748","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}
Özge Zorlu, Sevil Karabağ, Kıvılcım E Erdoğan, Merve Aksın, Batuhan Üstün
{"title":"Immunoexpression Patterns of Adhesion Molecules (E-cadherin, β-catenin, CD56) and Cytokeratins (CK19, CK20, HMWCK, CAM5.2) During Hair Development in Human Fetuses Compared With Adults.","authors":"Özge Zorlu, Sevil Karabağ, Kıvılcım E Erdoğan, Merve Aksın, Batuhan Üstün","doi":"10.1097/DAD.0000000000002741","DOIUrl":"10.1097/DAD.0000000000002741","url":null,"abstract":"<p><strong>Abstract: </strong>Abnormalities in the expression of cytokeratins or adhesion molecules have been associated with hair disorders. The expression patterns of these molecules in the hair follicles of developing human fetuses are not obvious. We aimed to investigate the expression patterns of some cytokeratins and adhesion molecules in the hair follicle of human fetuses and compared them with adults. Forty-eight fetuses of >16 gestational weeks and 22 adult cases with total excisions of benign nevi or cysts were enrolled. The skin samples were taken from both the scalp and back of the fetuses. The histopathologically normal skin areas were evaluated in adults. CK19, CK20, CAM5.2, high-molecular-weight cytokeratin, E-cadherin, β-catenin, and CD56 immunohistochemical stainings were performed. In the fetus group, the staining scores declined in the third trimester but elevated and reached the highest level in adults, except for CD56, which did not stain any adult samples. All stainings were mostly observed in the outer root sheath, except CD56 that stained the perifollicular dermal sheath only in fetuses. E-cadherin, β-catenin, and high-molecular-weight cytokeratin strongly and diffusely stained all adult samples. CAM5.2 and CK19 scores were correlated in fetuses (scalp scores: r s = 0.405, P = 0.004; back scores: r s = 0.422, P = 0.003) and adults (back scores: r s = 0.562, P = 0.046). CD56 negativity indicated the immune-privilege feature of adult hair follicles. As CK19, CAM5.2 may be used to find the regions of stem cells or transient amplifying cells.</p>","PeriodicalId":50967,"journal":{"name":"American Journal of Dermatopathology","volume":null,"pages":null},"PeriodicalIF":1.1,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141263108","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 Perivascular Hemophagocytosis: A Report of 2 Cases With Emphasis on Clinical and Histological Features.","authors":"Gerardo Cazzato, Franco Rongioletti","doi":"10.1097/DAD.0000000000002732","DOIUrl":"10.1097/DAD.0000000000002732","url":null,"abstract":"<p><strong>Abstract: </strong>Cutaneous perivascular hemophagocytosis (CH) is a histological manifestation that manifests as systemic hemophagocytic syndrome, also known as hemophagocytic lymphohistiocytosis, when accompanied by fever, hepatosplenomegaly, liver dysfunction, and cytopenia, and may rarely manifest independently of hemophagocytic lymphohistiocytosis. CH typically presents as purpuric or brownish macules and patches on the extremities, abdomen, and trunks. Histopathologically, the hallmark of CH includes extravasated erythrocytes and karyorrhectic debris phagocytized by histiocytes, associated with dermal capillary ectasia, perivascular infiltration of neutrophils, nuclear dust, and histiocytes without atypia. In this study, we report 2 cases of CH encountered in routine diagnostic practice and elucidate their significant clinical and histologic features. Our first patient had leukocytoclastic vasculitis with CH in the setting of Yersinia enterocolitis, and the second case represents CH in association with non-Hodgkin lymphoma. This study highlights the importance of considering CH as a potential indicator of underlying systemic pathology, including infectious and hematological disorders, in clinical practice.</p>","PeriodicalId":50967,"journal":{"name":"American Journal of Dermatopathology","volume":null,"pages":null},"PeriodicalIF":1.1,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140892928","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":"Rare Presentation of Melanoma Recurrences With Diffuse Subcutaneous Intravascular Lesions: A Novel In-Transit Metastases Pattern.","authors":"Shitong Su, Jie Liu, Yu Jiang","doi":"10.1097/DAD.0000000000002735","DOIUrl":"10.1097/DAD.0000000000002735","url":null,"abstract":"<p><strong>Abstract: </strong>In 4%-11% of cases, melanoma recurrences present as in-transit (IT) metastases, and their prognosis is quite poor. Consequently, an early diagnosis and treatment of IT metastasis assume paramount significance. Despite this, the diagnosis of cutaneous IT metastases persistently presents a formidable challenge due to the diversity in clinical and dermoscopic characteristics. We provide a novel melanoma IT metastases pattern with interesting dermoscopic features and magnetic resonance imaging via presenting an unusual case characterized by diffuse subcutaneous intravascular lesions to supplement the understanding of cutaneous melanoma IT metastases.</p>","PeriodicalId":50967,"journal":{"name":"American Journal of Dermatopathology","volume":null,"pages":null},"PeriodicalIF":1.1,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140892929","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":"Diagnostic Accuracy of ChatGPT for Textbook Descriptions of Epidermal Tumors: Correspondence.","authors":"Hinpetch Daungsupawongm, Viroj Wiwanitkit","doi":"10.1097/DAD.0000000000002829","DOIUrl":"https://doi.org/10.1097/DAD.0000000000002829","url":null,"abstract":"","PeriodicalId":50967,"journal":{"name":"American Journal of Dermatopathology","volume":null,"pages":null},"PeriodicalIF":1.1,"publicationDate":"2024-08-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141983805","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}
Kristan Schiele, Veronica Voronina, Arthur Marka, Julianne Mann, Robert E LeBlanc
{"title":"Clinicopathologic Correlation and Routine Immunostains Help Distinguish Congenital Disseminated Pyogenic Granuloma From Other Cutaneous Disseminated Vascular Proliferations of Infancy: Case Report and Literature Review.","authors":"Kristan Schiele, Veronica Voronina, Arthur Marka, Julianne Mann, Robert E LeBlanc","doi":"10.1097/DAD.0000000000002830","DOIUrl":"https://doi.org/10.1097/DAD.0000000000002830","url":null,"abstract":"<p><strong>Abstract: </strong>The presence of multiple cutaneous vascular lesions in infancy can signal the possibility of visceral involvement. Dermatopathologists must appreciate how the differential diagnosis includes entities that have distinct therapeutic and prognostic implications. Fortunately, these rare entities can be distinguished histopathologically with the help of clinicopathologic correlation and immunohistochemistry. In this article, we discuss congenital disseminated pyogenic granuloma, multifocal infantile hemangioma, and multifocal lymphangioendotheliomatosis with thrombocytopenia. Subtle morphologic and immunophenotypic features permit their distinction, which in turn is important for identifying extracutaneous manifestations and effective treatments. We present a case of a 3-week-old infant with congenital disseminated pyogenic granuloma involving the skin and the liver whose lesions regressed without therapeutic intervention over 6 months of close follow-up. We review the literature on these rare, overlapping entities and present an approach to resolving the differential diagnosis.</p>","PeriodicalId":50967,"journal":{"name":"American Journal of Dermatopathology","volume":null,"pages":null},"PeriodicalIF":1.1,"publicationDate":"2024-08-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141983803","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}
Donald E Neal, Emma F Johnson, Shruti Agrawal, Austin Todd, Michael J Camilleri, Carilyn N Wieland
{"title":"Comparison of Digital Pathology and Light Microscopy Among Dermatology Residents: A Reappraisal Following Practice Changes.","authors":"Donald E Neal, Emma F Johnson, Shruti Agrawal, Austin Todd, Michael J Camilleri, Carilyn N Wieland","doi":"10.1097/DAD.0000000000002805","DOIUrl":"https://doi.org/10.1097/DAD.0000000000002805","url":null,"abstract":"<p><strong>Background: </strong>Following transition to digital pathology for primary diagnosis at our institution, dermatology residents have reduced exposure to light microscopy. This study compares resident competency with light microscopy versus digital pathology following practice changes.</p><p><strong>Methods: </strong>Twenty-one dermatology residents were administered a dermatopathology examination composed of 32 diagnoses evaluated using digital slides and 32 with light microscopy. Case difficulty was graded and balanced between modalities. Diagnostic accuracy was measured using the number of correct diagnoses for each modality. Participants were surveyed regarding their experience and preferences.</p><p><strong>Results: </strong>Diagnostic accuracy was higher with digital pathology than light microscopy (22/32 vs. 18/32, P < 0.001). Diagnostic accuracy with digital pathology increased with years of training, but accuracy with light microscopy did not. Residents with previous light microscopy experience achieved an average score of 19/32 on glass, as compared with 10/32 for those without experience (P = 0.039). Digital pathology was preferred over light microscopy (18/21, 85.7%).</p><p><strong>Conclusions: </strong>Trainees had better diagnostic proficiency with digital pathology and preferred this modality. Most practices at this time continue to use light microscopy. Therefore, we need to maintain proficiency in microscopy during training while concurrently preparing trainees for a digital future.</p>","PeriodicalId":50967,"journal":{"name":"American Journal of Dermatopathology","volume":null,"pages":null},"PeriodicalIF":1.1,"publicationDate":"2024-08-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141983804","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}
Rohan Mital, Kristopher Fisher, Abraham M Korman, Jose A Plaza, Benjamin H Kaffenberger, Catherine G Chung
{"title":"Histopathologic Findings of Evolving Mpox Lesions.","authors":"Rohan Mital, Kristopher Fisher, Abraham M Korman, Jose A Plaza, Benjamin H Kaffenberger, Catherine G Chung","doi":"10.1097/DAD.0000000000002709","DOIUrl":"https://doi.org/10.1097/DAD.0000000000002709","url":null,"abstract":"<p><strong>Abstract: </strong>This article reports two cases of the 2022 mpox virus with notable histopathology, and includes a novel description of mpox pseudotumor in the perianal region which is not previously described. This article additionally includes literature review of characteristic histopathology through evolving lesions, as it is sparsely described in relation to the 2022 mpox outbreak. Case one describes a 42-year-old man who presented with umbilicated, smooth papules on the trunk and extremities, and milia-like papules on the face. Histopathology of an umbilicated lesion revealed epidermal acanthosis with keratinocyte pallor, ballooning degeneration, keratinocyte necrosis, and neutrophilic epitheliotropism. Case two describes a 51-year-old man who presented with scattered eroded papules as well as a perianal mass. Histopathology of the mass revealed ulceration with keratinocyte enlargement and pallor with a mixed inflammatory cell infiltrate. It additionally revealed rare multinucleated keratinocytes with nuclear molding. These cases are remarkable and contribute to literature as reports of the histopathology of the atypical 2022 mpox outbreak are rare. A combination of clinical, laboratory, and histopathologic evidence is useful in diagnosing mpox, and these cases contribute to describing the evolution of viral lesions.</p>","PeriodicalId":50967,"journal":{"name":"American Journal of Dermatopathology","volume":null,"pages":null},"PeriodicalIF":1.1,"publicationDate":"2024-08-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141983807","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}
Spencer Ng, Katie C Hall, Klaus J Busam, Cecilia Lezcano, Andrea P Moy, Melissa Pulitzer, Aravindhan Sriharan, Shaofeng Yan, Konstantinos Linos
{"title":"Superficial Wnt-Activated Melanocytic Nevi/Melanocytomas With a Junctional Component: A Case Series.","authors":"Spencer Ng, Katie C Hall, Klaus J Busam, Cecilia Lezcano, Andrea P Moy, Melissa Pulitzer, Aravindhan Sriharan, Shaofeng Yan, Konstantinos Linos","doi":"10.1097/DAD.0000000000002804","DOIUrl":"https://doi.org/10.1097/DAD.0000000000002804","url":null,"abstract":"<p><strong>Abstract: </strong>The conventional morphological characteristics of Wnt-activated deep penetrating/plexiform melanocytomas/nevi (DPN) are those of large spindled or epithelioid melanocytes with distinctive voluminous amphophilic cytoplasm, fine pigmented granules, and surrounding melanophages. The central molecular hallmark is the activation of the Wnt-pathway predominantly driven by mutations in the beta-catenin (CTNNB1) gene. Although typically lacking a junctional component, a lesser-known superficial variant with a junctional component has been identified, which could potentially lead to diagnostic challenges. This study presents a cohort of 11 such cases displaying a junctional component of DPN from 10 patients (5 women and 5 men; age range: 27-78 years; median age: 51 years). The nevi were distributed as follows: 1 conjunctival, 1 scalp, 2 lower limb, and 6 truncal lesions. Eight cases were combined with a conventional nevus, 2 cases displayed pure DPN cytology exhibiting only a junctional element, and 9 cases exhibited some degree of lentiginous architecture. All cases demonstrated a low mitotic index (<1 mitosis/mm2). Immunohistochemistry revealed positive BRAF V600E staining in 8 cases (8/11), whereas all cases tested (11/11) were PRAME negative. Nuclear beta-catenin and LEF1 staining was consistently strong and diffuse with DPN cytology (11/11), along with robust cyclin D1 staining in all cases tested (11/11). By contrast, all 9 conventional nevi showed an absence of nuclear beta-catenin staining (0/9) and weaker, mosaic-type LEF1 and cyclin D1 staining was observed. This study emphasizes the diagnostic challenge these nevi can pose in the absence of a conventional, deeper DPN component, which can potentially be misdiagnosed as melanoma.</p>","PeriodicalId":50967,"journal":{"name":"American Journal of Dermatopathology","volume":null,"pages":null},"PeriodicalIF":1.1,"publicationDate":"2024-08-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141983851","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}
Jinpyo Hong, Joshua Shoemaker, Jennifer Scott, Klaus Helm
{"title":"Adding \"Unlikely\" as a Clinical Qualifier Is Unlikely to Be of Help: A Retrospective, Single-Center, Cross-Sectional Cohort Study.","authors":"Jinpyo Hong, Joshua Shoemaker, Jennifer Scott, Klaus Helm","doi":"10.1097/DAD.0000000000002814","DOIUrl":"https://doi.org/10.1097/DAD.0000000000002814","url":null,"abstract":"<p><strong>Abstract: </strong>Clinical correlation is essential to accurate and efficient diagnosis and differential diagnosis in dermatopathology. Poor-quality clinical information can lead to failures and delays in patient care. Some clinicians use the term \"unlikely\" when submitting a specimen. How frequently the \"unlikely\" clinical diagnosis correlates with the final pathologic diagnosis is unknown. We studied 203 dermatopathology reports from December 8, 2020, to July 1, 2021, that included the qualifier \"unlikely\" on the requisition sheet. Samples were stratified into either an inflammatory or neoplastic cohort based on final histopathologic diagnosis, with the neoplastic cohort being further stratified into pigmented and nonpigmented cohorts. Statistical analyses were conducted. The \"unlikely\" diagnosis in the clinical differential diagnosis and the final histologic diagnosis were the same in 7.9% of the 203 samples studied. This occurred in 8.5% of the inflammatory cohort and 7.6% of the neoplastic cohort. We concluded that the use of the qualifier \"unlikely\" is not helpful. We acknowledge the limitations of our study because of a small sample.</p>","PeriodicalId":50967,"journal":{"name":"American Journal of Dermatopathology","volume":null,"pages":null},"PeriodicalIF":1.1,"publicationDate":"2024-08-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141983800","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}