Journal of Cutaneous Pathology最新文献

{"title":"Thymoma-Associated Multiorgan Autoimmunity (TAMA) Presenting as a Graft Versus Host Disease-Like Erythroderma.","authors":"Noah Hewitt, Brooke Bertus, William Farmer, Katrin Kiavash, Colleen Beatty, Roxann Powers","doi":"10.1111/cup.14743","DOIUrl":"https://doi.org/10.1111/cup.14743","url":null,"abstract":"<p><p>Thymoma-associated multiorgan autoimmunity (TAMA) is a rare paraneoplastic disorder that presents similarly to graft versus host disease (GVHD). We report a unique case of TAMA presenting as a GVHD-like erythroderma in an elderly male with a history of benign thymoma. Cutaneous histopathological findings demonstrated vacuolar interface dermatitis with numerous dyskeratotic keratinocytes, exocytosis of lymphocytes, and a mildly acanthotic epidermis, which can be seen in several different disease processes. Thus, careful consideration of clinical presentation, historical information, laboratory testing, and histopathologic findings led to the correct diagnosis and treatment.</p>","PeriodicalId":15407,"journal":{"name":"Journal of Cutaneous Pathology","volume":null,"pages":null},"PeriodicalIF":1.6,"publicationDate":"2024-10-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142501222","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":"Compound Follicles in Folliculitis Decalvans Do Not Contain Vellus and Retained Telogen Follicles-A Pilot Histopathologic Series on Polytrichia.","authors":"Juwon Lee, Mariya Miteva","doi":"10.1111/cup.14735","DOIUrl":"https://doi.org/10.1111/cup.14735","url":null,"abstract":"<p><strong>Background: </strong>Polytrichia is a common clinical and trichoscopic feature in folliculitis decalvans (FD) that morphologically corresponds to compound follicular structures (CFS) of six or more follicles sharing a single infundibulum, surrounded by fibrosis.</p><p><strong>Objectives: </strong>To characterize the type of follicles in the CFS in scalp biopsy specimens from FD.</p><p><strong>Methods: </strong>We retrospectively reviewed 10 scalp biopsy specimens obtained by using the trichoscopy-guided approach from the affected scalp of patients diagnosed with early-stage FD between 2018 and 2023. We assessed the number and type of follicles within the CFS on horizontal sections.</p><p><strong>Results: </strong>The total number of assessed follicles was 205, out of which 159 were part of CFS. Of those, 156 were terminal follicles (146 anagen and ten telogen), three intermediate, and zero vellus follicles. Other common findings included dense mixed cell infiltrate consisting of histiocytes, plasma cells, lymphocytes, neutrophils, and eosinophils; psoriasiform hyperplasia; and fragmented hair shafts.</p><p><strong>Conclusions: </strong>We show that CFS in FD are composed largely of terminal anagen follicles. This may help with further understanding of the disease.</p>","PeriodicalId":15407,"journal":{"name":"Journal of Cutaneous Pathology","volume":null,"pages":null},"PeriodicalIF":1.6,"publicationDate":"2024-10-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142466588","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":"Pacinioma of Lumbosacral Skin in Closed Spinal Dysraphism.","authors":"Bryan Johnston, Katelynn Campbell","doi":"10.1111/cup.14733","DOIUrl":"https://doi.org/10.1111/cup.14733","url":null,"abstract":"<p><p>Closed spinal dysraphism (CSD) is a congenital condition caused by a failure in secondary neurulation during embryogenesis. CSD is associated with characteristic cutaneous stigmata often identified clinically. Rarely, such stigmata have been reported to occur with complex congenital intraspinal lipomas containing Pacinian corpuscle hyperplasia. Herein, we present our case of an asymptomatic 8-month-old female who presented with a midline lumbosacral acrochordon-like lesion. MRI revealed a midline lipomatous lesion at S2-S3 with a fibrovascular stalk likely continuous with the acrochordon. Given the absence of neurological symptoms concerning tethered cord syndrome (TCS), only the skin lesion was removed for cosmesis. Subsequent histopathological examination revealed numerous Pacinian corpuscles in a background of dermal collagen and subcutaneous adipose tissue. No features suggested a co-existent peripheral nerve sheath lesion (such as a neurofibroma). Given the spinal cord abnormalities, and the histologic features of a pure proliferation of Pacinian corpuscles, a diagnosis of the so-called \"Pacinioma\" was made. To the best of our knowledge, only four other cases of complex intraspinal lipomatous lesions containing Pacinioma in the setting of CSD with a tethered cord have been previously reported. This case highlights the complexity of the cutaneous stigmata of CSD.</p>","PeriodicalId":15407,"journal":{"name":"Journal of Cutaneous Pathology","volume":null,"pages":null},"PeriodicalIF":1.6,"publicationDate":"2024-10-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142466603","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":"Multisystem ALK-Positive Histiocytosis With DCTN1::ALK Fusion in an Adult, Responsive to Alectinib: Case Report and Literature Review.","authors":"Gregory S Phillips, Maxwell Knapp, Keith C Olsen, William Martin, Brandon Hayes-Lattin, Jina Chung","doi":"10.1111/cup.14732","DOIUrl":"https://doi.org/10.1111/cup.14732","url":null,"abstract":"<p><p>Anaplastic lymphoma kinase (ALK)-positive histiocytosis has emerged as a clinically relevant diagnosis featuring a wide span of clinical presentations, which are unified by the presence of ALK-positive histiocytes on histopathology and molecular drivers involving the ALK kinase gene. This report presents an adult case of multisystem ALK-positive histiocytosis with xanthogranuloma-like features on histopathology that was responsive to ALK inhibition, and includes a review of ALK-positive histiocytoses with cutaneous involvement reported in the literature. A 56-year-old male developed a widespread eruption of red-brown papules on the face, trunk, and upper extremities. Histopathological evaluation revealed a well-circumscribed, nodular dermal infiltrate of epithelioid histiocytes with Touton giant cells, rare bizarre multinucleated cells, and focal emperipolesis. The lesional cells were positive for CD68 and ALK1 immunohistochemical stains, and negative for CD1a. Next-generation sequencing identified a DCTN1::ALK fusion. On imaging, he was found to have bone, lung, soft tissue, and salivary gland involvement. ALK inhibition was initiated with alectinib, resulting in rapid improvement of cutaneous lesions and eventual complete resolution of abnormal imaging findings, which was sustained at 24 months of follow-up. This case adds to the spectrum of ALK-positive histiocytoses and further demonstrates the positive response with targeted therapy.</p>","PeriodicalId":15407,"journal":{"name":"Journal of Cutaneous Pathology","volume":null,"pages":null},"PeriodicalIF":1.6,"publicationDate":"2024-10-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142466602","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":"Hunting for Early Melanomas With a Maximum Clinical Surface Diameter up to 6 mm: A Prospective Study of Reflectance Confocal Microscopy Features in 68 Consecutive Cases.","authors":"J Pyne, S MacDonald, S Beale, E Myint, S Clark, A Trang","doi":"10.1111/cup.14731","DOIUrl":"https://doi.org/10.1111/cup.14731","url":null,"abstract":"<p><strong>Background: </strong>Early diagnosis of melanoma and prompt effective therapy optimizes prognosis. Reflectance confocal microscopy (RCM) facilitates diagnosis by providing immediate 3D single cell resolution down into the papillary dermis.</p><p><strong>Methods: </strong>Consecutive cases were examined using a Vivascope 1500 confocal microscope at a single referral medical practice in Sydney, Australia 2019-2023. Melanoma clinical surface diameters were recorded by 0.1 mm increments up to 6.0 mm. The RCM features recorded were: pagetoid single cells or nests, pleomorphic cell shape, atypical dendritic cells, non-edged papillae, variation in melanocyte size and confluent sheets of cells. All cases required diagnostic agreement by two dermatohistopathologists using hematoxylin and eosin staining followed by SOX 10 and/or PRAME stains if required.</p><p><strong>Results: </strong>Total cases were 68: 38 males (mean age 57) and 30 females (mean age 64). Melanoma in situ (n = 65) compared to invasive melanoma (n = 3), all males, invasion depth (0.4-0.5 mm). Most frequent RCM features found in 50% or more of cases within all diameter increments were: pagetoid single cells n = 64/68 (94%), pleomorphic cell shape n = 63/68 (93%), epidermal disarray n = 58/68 (85%), and atypical dendritic cells n = 45/68 (66%). Non-edged dermal papillae were n = 42/68 (62%).</p><p><strong>Conclusion: </strong>Melanoma RCM features were found throughout the diameter ranges. Confocal examination may facilitate early melanoma recognition in these ranges.</p>","PeriodicalId":15407,"journal":{"name":"Journal of Cutaneous Pathology","volume":null,"pages":null},"PeriodicalIF":1.6,"publicationDate":"2024-10-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142466589","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":"An Unexpected Finding of a PTPN11 Germline Mutation in a Patient With a Melanocytic Lesion With a Somatic MAP2K1 Mutation. Coincidence or Not?","authors":"Sven van der Woude, J S Klein Wassink-Ruiter, Joost Kluiver, Marthe de Jonge, Gilles F H Diercks","doi":"10.1111/cup.14730","DOIUrl":"https://doi.org/10.1111/cup.14730","url":null,"abstract":"<p><p>Melanocytic tumors are a diverse group of lesions and are traditionally classified based on a combination of clinical presentation as well as histological examination. More recently, molecular diagnostics has become an increasingly important part of differentiating different melanocytic lesions in the current WHO standards. This molecular testing, however, can result in unexpected findings. In this report, we describe that molecular testing of a clinical atypical melanocytic lesion showed a mutation in the MAP2K1 gene as well as an unexpected germline mutation in PTPN11, indicative of Noonan syndrome. Based on these findings we concluded that the patient had a MAP2K1 associated melanocytic lesion with Noonan syndrome as an incidental finding. Melanomas are classically not associated with Noonan syndrome. However, we hypothesized that the germline mutations of PTPN11 and the somatic second hit mutation in the MAP2K1 genes might be involved in the formation of the aforementioned lesion. As they are both part of the RAS-MAPK pathway. Furthermore, with the expansion of molecular diagnostics in melanomas, we expect to find an increase in unexpected (germline) mutations.</p>","PeriodicalId":15407,"journal":{"name":"Journal of Cutaneous Pathology","volume":null,"pages":null},"PeriodicalIF":1.6,"publicationDate":"2024-10-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142400353","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":"The Association of Interleukin-36 Staining Intensity and Response to Biologic Therapy in Patients With Psoriasis: A Retrospective Immunohistochemical and Chart Review Pilot Study.","authors":"William R Zhang, Tina Bhutani, Jeffrey P North","doi":"10.1111/cup.14729","DOIUrl":"https://doi.org/10.1111/cup.14729","url":null,"abstract":"<p><strong>Background: </strong>There are limited surrogate biomarkers to identify the active inflammatory pathway in psoriasis to direct treatment with targeted biologic therapies. We investigated the association of interleukin (IL)-36 epidermal expression, a diagnostic marker of psoriasis, with response to biologic therapy in patients with psoriasis.</p><p><strong>Methods: </strong>Retrospective immunohistochemical and chart review pilot study.</p><p><strong>Results: </strong>Patients with psoriasis with low (scores 0-2) vs. high (scores 3-4) IL-36 expression did not have significantly different response rates to tumor necrosis factor α (TNFα), IL-17, and IL-12/23 or IL-23 inhibitors; and similarly, mean IL-36 expression scores did not significantly differ among responders vs. non-responders to each treatment mechanism. However, in patients with psoriasis treated with IL-12/23 or IL-23 inhibitors, there was a marked absolute difference in response rates in those with high vs. low IL-36 (84% vs. 50%, p = 0.12) and in mean IL-36 scores in responders vs. non-responders (3.35 vs. 2.57, p = 0.19).</p><p><strong>Conclusions: </strong>Patients with psoriasis with high IL-36 expression were more likely to respond to IL-12/23 and IL-23 inhibition than those with low IL-36, though these findings were not statistically significant. Additional studies with larger sample sizes are needed to validate and expand upon these findings.</p>","PeriodicalId":15407,"journal":{"name":"Journal of Cutaneous Pathology","volume":null,"pages":null},"PeriodicalIF":1.6,"publicationDate":"2024-10-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142390923","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":"Acquired Perforating Dermatosis After Herpes Zoster: Wolf Isotopic Response.","authors":"Xiao H Du, Su Y Huang, Xiao F Zeng, Si J Lu, Zhe Gao","doi":"10.1111/cup.14728","DOIUrl":"https://doi.org/10.1111/cup.14728","url":null,"abstract":"<p><p>Wolf isotopic response (WIR) is a phenomenon in which a second, unrelated skin disease arises at the same site as a previously healed dermatosis. WIR most commonly occurs in healed herpes zoster but has also been described in other conditions, such as herpes simplex virus, varicella-zoster virus, and skin tumors. Acquired perforating dermatosis (APD) is characterized by transepidermal elimination of collagen bundles that lead to the development of ulcerative papules, which are often associated with systemic conditions such as diabetes or renal failure. This report documents a rare occurrence of APD after WIR and reviews related published works.</p>","PeriodicalId":15407,"journal":{"name":"Journal of Cutaneous Pathology","volume":null,"pages":null},"PeriodicalIF":1.6,"publicationDate":"2024-10-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142365408","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":"Evaluation of Multiple Tissue Levels Frequently Upstages Patients With Clinically Localized Thin Primary Cutaneous Melanoma.","authors":"Louise A Jackett, James P Gullifer, Richard A Scolyer","doi":"10.1111/cup.14726","DOIUrl":"https://doi.org/10.1111/cup.14726","url":null,"abstract":"<p><strong>Background: </strong>Breslow thickness (BT), ulceration, and microsatellitosis are critical prognostic parameters for cutaneous melanoma staging. These parameters can vary depending on the number of tissue levels examined from individual paraffin blocks. We sought to evaluate all prognostic histopathologic parameters in melanoma for their variations between levels, taken at regular intervals, in a single study.</p><p><strong>Methods: </strong>We analyzed 40 consecutive cases of primary cutaneous (nonacral) melanoma through five hematoxylin and eosin sections, taken at 100 μm intervals, for staging and prognostic parameters.</p><p><strong>Results: </strong>Examination of additional levels resulted in (a) an increase in BT in 47.5% (19 out of 40) of cases and (b) detection of ulceration in a further 5% (2/40). This resulted in upstaging for 20% (8 out of 40) of patients (15% because of BT, 2.5% because of ulceration, and 2.5% because of BT and ulceration). The upstaging effect was incremental, with approximately 5% of patients upstaged with each additional 100 μm interval (up to 400 μm). Incipient ulceration and epidermal consumption were infrequent (10% of cases); however, when present, ulceration was subsequently observed in half of cases. We encountered no cases where microsatellitosis was detected at deeper levels.</p><p><strong>Conclusion: </strong>The performance of additional tissue levels is a simple and inexpensive procedure that can improve the accuracy of staging for patients with thin (pT1) primary cutaneous melanomas. It may be pertinent for pathologists to consider additional levels for thin melanomas when a BT measurement is close to a staging threshold (e.g., within 0.1-0.3 mm for pT1a vs. pT1b, or pT1b vs. pT2a), or when incipient ulceration is encountered.</p>","PeriodicalId":15407,"journal":{"name":"Journal of Cutaneous Pathology","volume":null,"pages":null},"PeriodicalIF":1.6,"publicationDate":"2024-10-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142365409","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":"The Prognostic Significance of Tumoral Melanosis.","authors":"Alison J Potter, Peter M Ferguson, Serigne N Lo, Tasnia Ahmed, Robert V Rawson, John F Thompson, Georgina V Long, Richard A Scolyer","doi":"10.1111/cup.14727","DOIUrl":"https://doi.org/10.1111/cup.14727","url":null,"abstract":"<p><strong>Background: </strong>Tumoral melanosis (TM) is a histological term to describe a nodular aggregation of macrophages containing melanin pigment (melanophages) that is devoid of viable melanocytes. It is most often identified in skin, where it may be appreciated clinically as a pigmented lesion; however, it can also be found in other organs such as lymph nodes. The presence of TM is usually thought to signify the presence of a regressed melanoma or other pigmented tumor. Until recently, it was a relatively uncommon finding; however, with the use of effective systemic therapies against melanoma, its occurrence in histological specimens is more frequent.</p><p><strong>Methods: </strong>We identified and reviewed all histopathological diagnoses of TM at any organ site reported at a single institution from 2006 to 2018. TM cases were paired with non-TM cases of cutaneous melanoma through propensity score matching at a 1:2 ratio, and their survival outcomes were compared. The clinical outcomes examined included recurrence-free survival (RFS), distant disease-free survival (DDFS), melanoma-specific survival (MSS), and overall survival (OS).</p><p><strong>Results: </strong>TM was reported in 79 patients. Their median age was 65 years (range 22-88), with a 2:1 male predominance (51 out of 79, 65%). The most common organ involved was the skin (67%), with a third of all cases localized to a lower limb (36%). TM had a strong association with the presence of melanoma (91%) and regression at other sites of melanoma (54%), suggesting that it is part of a systemic immune response against melanoma. Most patients with TM either previously or subsequently developed histologically confirmed melanoma in the same anatomical region as the TM (89%). Thirty-five TM patients were matched with 70 non-TM cases. Patients with melanoma who developed TM without prior regional or systemic therapy showed improved MSS (p = 0.03), whereas no statistically significant differences were observed in terms of RFS, DDFS, and OS.</p><p><strong>Conclusions: </strong>TM usually occurs in the context of a previous or subsequent cutaneous melanoma and is associated with improved MSS. It is important that TM is recognized by pathologists and documented in pathology reports.</p>","PeriodicalId":15407,"journal":{"name":"Journal of Cutaneous Pathology","volume":null,"pages":null},"PeriodicalIF":1.6,"publicationDate":"2024-10-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142365410","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}