Archival Institutions Research Articles

Metastatic Renal Cell Carcinoma with Fibromyomatous Stroma associated with Tuberous Sclerosis or MTOR, TSC1/TSC2-Mutations: A Series of 4 Cases and a Review of the Literature

Renal cell carcinoma with fibromyomatous stroma (RCCfms) are characterized by a constellation of morphologic findings that include elongated tubules lined by cells with clear to pale eosinophilic cytoplasm and intersecting bands of smooth muscle stroma. Consistent immunohistochemistry findings in RCCfms include diffuse positivity for carbonic anhydrase 9 and variable expression of keratin 7. Molecular profiling of such tumors show either pathogenic alterations of the ELOC (TCEB1) gene, or alterations of MTOR, TSC1, and TSC2. MTOR, TSC1/TSC2-altered RCCfms (M/TSC-RCCfms) has been reported both in the sporadic setting and in association with tuberous sclerosis complex (TSC). The importance of accurate diagnosis of M/TSC-RCCfms includes prompting germline testing in the appropriate clinical context. In addition, it can lead to patient management strategies that are focused on the preservation of renal function, as TSC patients often have multifocal and bilateral disease. As diagnostic criteria for M/TSC-RCCfms have only been recently established, additional data are needed to understand the natural history of this disease. Herein, we report 6 patients with metastatic M/TSC-RCCfms, including four patients from our institutional archives (four males, aged 36 to 58 years at nephrectomy), and two additional cases reported in the literature. Five patients had TSC, and the sixth had an MTOR-altered RCCfms. The majority of patients (5/6, 83%) presented with regional lymph node involvement and one patient developed metastases to the lung. All patients were alive at last follow up (median follow-up of 85 months). Our report is intended to raise awareness regarding rare instances of metastatic behavior for M/TSC-RCCfms.

Open-Source Solutions in Archival Institutions

Open-source solutions offer a cost-effective alternative for archival institutions facing budget constraints. The absence of licensing fees and the ability to modify and customize the software without vendor lock-in make open source particularly appealing. This financial flexibility allows institutions to allocate resources to other critical aspects of preservation and access. In the article the author presents various advantages and disadvantages of using open-source software.

‘The melody that’s sweetly played in tune’ #—the heritage ecologies of the Ardeer Peninsula, North Ayrshire, Scotland

This photo essay explores the ways in which archaeology and photography work together and intervene in the world, to focus on landscapes that have been overlooked or actively ignored. It considers the site of a dynamite factory that was built by Alfred Nobel on the Ardeer Peninsula, Scotland, which has now been designated as a brownfield. The prevailing narrative is of industrial progress followed by ruination; where institutional archives memorialise Alfred Nobel, the technology of dynamite manufacture, and the built environment, they ignore the lives of those who worked there. This narrative sits alongside the way the present landscape is viewed as derelict and in need of redevelopment, ignoring the fauna and flora that thrive there. We take an other-than-human approach and consider how multiple species and processes come together at Ardeer. A vibrant and lively landscape is brought to the fore in terms of an archaeology of factory workers and a heritage site as an ecology.

Yakov Denisovich Petrov: Smolensk Teacher at the Head of the Regional Pedagogical University during the Great Patriotic War

In this publication, the authors continued their work on the study of the personality of the director of the Sverdlovsk State Pedagogical Institute, Yakov Denisovich Petrov, who held this post more than anyone else who headed this university, and his role in the development of the system of higher pedagogical education in the Sverdlovsk region. Based on the materials identified by the authors and previously unknown materials deposited in archival institutions of the Sverdlovsk and Smolensk regions, the authors analyzed in detail the features of the early biography of the director of the institute. This allowed us to fully answer questions related to Petrov's social background, which in the 1920s and 1930s was one of the main criteria for evaluating the political and business qualities of a communist and an employee for party and Soviet officials. And his gradual advancement up the career ladder: from a rural teacher to an experienced manager. At the same time, the authors abandoned the historical-party approach in consecrating the work of the Sverdlovsk Pedagogical Institute in the early years of Ya.D. Petrov's official activity, which is characteristic of Soviet historians. The personal participation of the director in solving the personnel and material problems of the university is specially investigated. The authors draw readers' attention to the fact that Petrov's solution of state tasks for the development of higher pedagogical education in the Sverdlovsk region and improving its quality in the most difficult conditions of the war years were quite successful. In many ways, this was the result of the rich work experience that Yakov Denisovich had accumulated during his years working in the public education system of the Smolensk region and in the People's Commissariat of Education of the RSFSR.

MALTomas of the lung: A Clinicopathologic Review

Abstract Introduction/Objective MALTomas of the lung are the most common primary lymphoma of the lung. They are rare in clinical practice and have an indolent nature with a good prognosis and are often asymptomatic upon diagnosis. We undertook a retrospective study to assess the clinical and pathologic features of primary MALTomas of the lung. Methods/Case Report A search for lymphomas of the lung was done in our institutional archive of pathology from 1993-2023. The clinical and pathological data were reviewed in detail. Results (if a Case Study enter NA) There were a total of 51 cases of MALTomas recorded. Ages ranged from 35-88 years with an average age of 65 years old. 32 of the patients were female and 19 were male. Twenty eight of these cases involved the right lung, 18 affected the left lung, 5 cases had synchronoous involvement bilaterally. Thirteen (13/51) cases had extrapulmonary involvement, including the bone marrow, stomach, lymph nodes, thyroid, endometrium, eye, small bowel and pleura. 1/51 had synchronous follicular lymphoma and 2/51 had metachronous lymphoma (one large cell and one small lymphocytic lymphoma). 15/51 patients had bone marow biopsies, one of which developed multiple myeloma 7 years later. 10/51 patients had synchronous gastrointestinal epithelial lesions (9 tubular adenomas and 1 carcinoid tumor). 1/51 patients had a synchronous adenocarcinoma of the lung. 18 of the cases showed plasmacytic differentiation (8 kappa restricted and 10 lambda restricted). Conclusion Primary MALTomas of the lung are low grade malignancies that can have a more complex clinical scenario, such as multifocality or synchronous/metachronous lesions which can complicate clinical management.

“Ka Aina No Ka Poe o Hawaii”

Abstract This article examines the independent newspaper Huli, published at the birth of the modern Hawaiian sovereignty movement from 1971 to 1973 by the grassroots political organization Kokua Hawaii. Reading through the surviving issues of Huli available in institutional archives, the article considers the variety of perspectives promoted within its pages to position Kokua Hawaii in relation to international understandings of anticolonial liberatory politics of the Global South. Revisiting prominent critiques of Kokua Hawaii, it argues that insights provided by Huli trouble lasting understandings of the organization and underscore the periodical’s relevance for navigating rising waves of political struggle in Hawaiʻi today. Moreover, the article contends that Huli—a timely revival of Hawaiʻi’s politically engaged print culture that flourished during the nineteenth century—offers one foundation for constructing a counterhegemonic lineage of socially concerned artists and cultural workers in Hawaiʻi. Through an analysis of Huli’s content, imagery, and context of production, the article uses the name of the periodical and its call to “huli” as a means of tracking the influence of Kokua Hawaii’s politics and aesthetics into the present, with Huli acting as a medium for insurgent inspiration.

ALK-rearranged, CD34-positive spindle cell neoplasms resembling dermatofibrosarcoma protuberans: a study of seven cases.

The majority of dermatofibrosarcoma protuberans (DFSP) harbour PDGFB or PDGFD rearrangements. We encountered ALK expression/rearrangement in a PDGFB/D-negative CD34-positive spindle cell neoplasm with features similar to DFSP, prompting evaluation of ALK-rearrangements in DFSP and plaque-like CD34-positive dermal fibroma (P-LDF). We searched the archives of academic institutions for cases previously coded as DFSP and P-LDF. NGS-naïve or PDGFB-negative DFSP were screened for ALK (clone D5F3) expression by immunohistochemistry. NGS or ALK FISH was performed on ALK-positive cases. Methylome profiling studies were performed and compared with conventional DFSP. One case of "DFSP" and two "P-LDF" with ALK expression were identified from the archives, while four cases were detected prospectively. These seven cases (6F:1M; 8 months to 76 years) arose in the dermis of the arm (two), scalp, eyelid, thigh, abdomen, and shoulder and ranged from 0.4 to 4.2 cm. Tumours were composed of spindled cells and displayed a storiform growth pattern. Cytologic atypia was absent, and mitotic figures were scarce (0-2/10 HPFs, high power fields). The lesional cells were diffusely positive for CD34 and ALK and negative for S100 protein. By NGS (n = 5), ALK fusion partners included DCTN1 (2), PLEKHH2, and CLIP2 in DFSP-like cases and FLNA in P-LDF-like lesions. ALK FISH was positive in one (of two) cases previously labelled P-LDF. Methylome profiling of two (of three) ALK-rearranged DFSP-like tumours showed clustering with conventional DFSP in the UMAP dimension reduction plot. To date, no tumour has recurred (n = 2; 26, 27 months). We describe a cohort of novel ALK-rearranged tumours with morphologic features similar to DFSP.

Modeling Memory Institutions: The Ainu Case Study

ABSTRACTThis study aimed to construct a model of memory institutions (libraries, archives, and museums) as a lens for analyzing contemporary collective memory practices related to the Ainu people. First, we developed a pilot model to organize the current phenomena and practices of collective memories and suggest a respective circulation mechanism consisting of three core elements: media, collective representations, and collective memory. Additionally, employing this pilot model, we conducted close readings of 449 pieces of literature and illustrated the characteristics of collective memory practices and phenomena in a case study of the Ainu. Our findings indicate that 1) the traditional collective memory of the Ainu has gradually shifted toward recognizing them as a unique ethnic group, and 2) by translating their oral literature into diverse media, the Ainu have reshaped their cultural identity.

Navigating the Vatican Library repositories

An institution of ancient origin, the Vatican Apostolic Library holds a diverse and complex collection of documents that reflect its rich institutional history, distributed among its various collections and institutional archives. This multifaceted heritage, comprising traditional and digital resources, stands as a valuable testimony to the active role of the Library in the international cultural context. This article aims to provide a general overview of these resources and their potential historical significance, focusing on the last century and presenting some significant episodes in the institution’s history.

Characterisation of Kürsteiner canals of parathyroid: imparting relevance to a one-and-a-quarter-century-old concept.

Kürsteiner canals (KC) were described at least 125 years ago as pharyngeal pouch embryological remnants of parathyroid and thymic development. While considered precursors for a subset of parathyroid cysts and salivary heterotopias (SH), they remain enigmatic. We now define a comprehensive phenotype of KC remnants and investigate their role in a spectrum of parathyroid lesions. `Sixty-two cystic and 22 non-cystic parathyroid lesions (73 patients) were retrieved from our institutional archive (2011-23) and evaluated for the presence of KC and prevalence of KC phenotype in parathyroid hormone (PTH)-positive and PTH-negative cysts. KC phenotype was defined as: cysts and tubules with surrounding sclerosis; bland, unilayered lining with frequent nuclear indentation of lumina; vesicular chromatin relative to chief cells; attenuated eosinophilic to 'hyper-cleared' cytoplasm; and staining pattern PTH-negative, SOX-10-positive, CK7-positive, GATA-3-positive and PAX-9 dim, a subset with oestrogen/progesterone receptor (ER/PR) positivity. Thirty PTH-negative cysts were identified in the neck/mediastinum; 14 of this group also showed SH. Thirty-two PTH-positive cysts included: 11 cystic parathyroid adenomas, 17 hyperplastic parathyroids, and four carcinomas. KC showed two distinct subtypes and were often found near PTH-negative cysts. PTH-negative cysts were associated with inferior parathyroids, SOX-10 positivity, fibrosclerosis, vesicular nuclei indenting cyst lumina and hyper-cleared or attenuated eosinophilic cytoplasm. KC are common in parathyroids and show a distinct histological and immunohistochemical profile, with an inferior predilection favouring branchial cleft III distribution. Diagnostically, the high prevalence of this phenotype in PTH-negative cysts and salivary heterotopia supports derivation of non-functioning cysts from KC. Conversely, PTH-positive cysts are more compatible with cystic change within hyperfunctioning glands.

Stratified Mucin-producing Lesions of the Anus

Primary anal cancers are rare and typically driven by high-risk human papillomavirus (HPV) infection. Though squamous cell carcinoma is most common, a spectrum of HPV-related nonsquamous anogenital neoplasms with similarities to cervical stratified mucin-producing carcinoma has been reported. In this study, we mined our institutional archives to characterize the clinicopathologic features of this emerging entity. Six cases were identified from the files at 2 institutions, including 4 cases of invasive stratified mucin-producing carcinoma and 2 stratified mucin-producing intraepithelial lesions (SMILE). Four patients were women, and the mean age was 70 years. Patients presented with rectal/anal mass or polyp, rectal bleeding or pain, weight loss, or at the time of screening colonoscopy. Tumors displayed histologic features as described in the gynecologic tract. Cases of invasive stratified mucinous carcinoma showed infiltrative tumor nests with variable intracytoplasmic mucin, peripheral palisading, prominent apoptosis, and neutrophilic infiltrate. One invasive stratified mucinous carcinoma associated with high grade glandular dysplasia, whereas 1 SMILE was next to conventional low-grade squamous intraepithelial lesion. All lesions stained with p16 showed block-like p16 expression. HPV in situ hybridization was performed in 5 cases, 4 of which were positive; one was interpreted as equivocal. Follow-up information, available in 4 patients, revealed 1 local recurrence followed by death due to unrelated causes in a patient with invasive stratified mucin-producing carcinoma. We report the first series of HPV-associated primary anal stratified mucin-producing neoplasms analogous to those seen in the gynecologic tract, further broadening the spectrum of HPV-related anal neoplasia.

What are the Main Complications When it Comes to the Restitution of the Objects Stolen During the Holocaust in Bosnia and Herzegovina?

Abstract During World War II, Bosnia and Hercegovina was occupied by the Ustashe-led Independent State of Croatia, a Nazi collaborator par excellence. Ustashe, mostly Croats, Muslims-Bosniaks, and domestic Germans, overwhelmingly participated in the annihilation of more than 85 % of the Bosnian Jewish population during the Shoah. Beside the physical destruction of the community, these Nazi collaborators plundered Jewish assets in an estimated value of over one billion US dollars and robbed priceless cultural artifacts along with the communal archives. While witness accounts agree that looting of most movable property (books, artwork, and other valuables) was carried out in the first days of occupation by the Nazis themselves, the robbery of Jewish property (apartments, houses, businesses) as well as torture and killings of domestic Jews was committed by the Ustashe. What complicates the restitution in this country is the state and memory politics, but also the inexistence of a central registry of stolen items that could be claimed. Moreover, it is of the essence that the GLAM institutions (galleries, libraries, archives, and museums) within Bosnia and the former Yugoslavia region engage in conducting detailed provenance research of their respective collections.

Educational and Cultural Role of Arab Virtual Document Exhibitions

Archival institutions are legally authorized to preserve educational, historical, or archival documents. Under this context, this research explores the role and interest of Arab archival institutions in displaying official documents on their websites. This study delves into focusing on the service provision of archival institutions by providing a virtual exhibition of their documents. Method of content analysis helps this research analyze the exhibition’s characteristics, for instance, display of document type, size, metadata availability, research tools and instruments, and engraving the possibility to copy, download, or print documents. The sample size of the research included ten archival institutions in Arab, including Oman, Jordan, UAE, Tunisia, KSA, Iraq, Qatar, Libya, and Morocco. These institutes were selected based on the presence of archival document websites that provide text documents, photographs, and visual recordings. The analysis of the research uncovered the aspect that Arab archival institutes differ in their interest in exhibiting virtual documents and the type/number of documents available to the public. However, almost 50% of archival institutes provide metadata for displayed documents. They provide advanced characteristics, such as enlargement of images, search, download, and sharing, available in 33.3% of virtual exhibitions. This study recommends the establishment of control to select displayed documents compatible with different users. Moreover, the display of images and documents should include descriptive data that explains the nature and context of document creation and preservation. Virtual exhibition visitors must be surveyed with. their aspirations under consideration of the archival institute's policies and country-specific document management laws.Educational Service

P104 MEDICAL TREATMENT IN PATIENTS WITH HEART FAILURE WITH REDUCED EJECTION FRACTION HOSPITALIZED AT RESEARCH INSTITUTE OF CARDIOLOGY DURING 1 YEAR (FROM SEPTEMBER 2022 TO SEPTEMBER 2023)

Objective: Patients with heart failure remain most challenging group of patients with cardiovascular diseases. Hospital period of these patients is important period of their life. Therefore evaluation of the level of in- hospital care and treatment options represents valuable information. Design and method: 477 patients (pts), 13% female,87% male with HFrEF hospitalized at Research Institute of Cardiology during 1 year (September 2022 – September 2023) represented study population. Consecutive patients’ data was extracted from the archive of Institute and evaluated. Results: Majority of pts had NYHA III-IV and mean ejection fraction (EF) of 35% (26-43%).Atrial fibrillation (AF) observed in 17,8% out of 477 pts. Myocardial infarction (MI) in anamnesis was in 82%. Systolic Blood Pressure (SBP) – mean 137 mm.Hg(min 80-max 240),Diastolic Blood Pressure(DBP) – mean 84 mm.Hg(min 50 – max 140).Arterial Hypertension(AH) had 61,4%. Type 2 Diabetes (T2D) – 51%.Chronic Kidney Disease had 22,9%.Pulmonary artery hypertension – 33%.Hb<12 g/l had 22,4%.ACE inhibitors or ARB mainly in form of fix combination with thiazide diuretics received 34% out of 477pts. ARNI were used in 27,7% of pts.B-blockers-55%. MRA – 89%. SGLT2 inh – 17,4%.Loop diuretics-86,2%.Digoxin-28,8%.Amiodarone – 22,9%.Ivabradine – 6%. Lab, tests: К+ mean 4,4 (2,9-6,2). Na+, mean 140,4 (133- 155). NT-proBNP, mean 2246.39 pg/ml (10.0-30000.0). Conclusion: In significant number of patients with HFrEF hospitalized to Research Institute of Cardiology during the period from September 2022- September 2023 medical therapy was almost adequately adherent to modern international guidelines on management of heart failure. In some cases’ limitations for indication because of low blood pressure, low eGFR and high serum kalium level took place.

“We are the forerunners in Southern Europe”: Experimenting with Business Improvement Districts in Greater Barcelona

ABSTRACT This paper uses the experimentation with Business Improvement Districts in Greater Barcelona to examine its relational re-making in a rather over-looked Southern-Mediterranean urban, socio-spatial and political-institutional context. Drawing on semi-structured interviews and institutional archives, it offers three intellectual contributions to urban policy mobilities studies. First, the paper argues that the territorial adaptation, mediation and translation of urban entrepreneurial policies hinges upon the differential and inherited nature of welfare regimes, state-market constellations and existing political infrastructures. Second, the paper outlines that policymakers have followed open-ended and multilateral learning approaches through space and time, in which some policy features were (re-)learned, circulated and modified to fit more centralized regimes. Third, and finally, it sketches out the role of government-funded pilot programs as instances through which policies are showcased, experimented and ultimately constituted before and after their institutionalization in specific socio-legal and socio-spatial contexts.

Decentralisation as a marketing strategy for archival services in Zimbabwe

Purpose Archival institutions must make their holdings and services known to the public to ensure increased usage. It is the obligation of archival institutions to adopt strategies to market their services to increase the usage of their holdings. Therefore, this study aims to assess the National Archives of Zimbabwe’s (NAZ) decentralisation drive as a strategy to market its archival services. Design/methodology/approach The multiple case study research design, which is interpretive and qualitative, was used for the study. The study used interviews, questionnaires and document review as data collection tools. The collected data were presented, analysed and discussed using the thematic data analysis approach. Findings Findings revealed that the NAZ decentralised provincial records centres were actively involved in the marketing of archival services offered by the NAZ. The study also established that the decentralised provincial records centres perform activities such as records surveys, training, oral history, issuance of brochures, guided tours and career guidance, which increase their interactions with communities they serve. The decentralised offices took advantage of these interactions to market archival services offered by the NAZ. Originality/value The study illustrated that decentralisation of archival institutions and services to the lower tiers of government is a powerful strategy for the marketing of archival services. Therefore, there is need for archival institutions to adopt or enhance the use of this strategy to increase the usage of archives.

The Emergence of Anthroposophical Curative Education in Israel

Abstract This paper tracks anthroposophical social initiatives in Israel in the mid-20th century. It follows the migration of people involved in the anthroposophical movement, the transfer of anthroposophical ideas and the transnational translation of social work knowledge and practice from Europe to Mandatory Palestine and Israel. Archival material was collected from official, institutional, and private archives in Israel, Switzerland, Britain, and Germany and some data was obtained through oral history interviews. Transnational correspondence between anthroposophists in Palestine, later Israel, and those in Europe and Britain up to the late 1960s reveals efforts to tackle the newly founded state’s most burning struggles to adjust, realize, and embed an anthroposophical care practice. The paper suggests that the transnational translation of anthroposophical curative education played a significant role in the reception of anthroposophy in Israel through a process in which European cultural aspects of anthroposophy were adjusted, and its universal human aspects were prioritized.

Religious Publishing in 17th-Century Geneva

The objective of this article is to shed light on the history of the Reformation in 17th-century Geneva. The lens through which this study is conducted is that of religious publishing activity, which was significantly managed by the Company of Pastors and Professors. The role of the Company in religious publishing is inextricably linked to the unique status of the Church of Geneva within the broader context of the Reformation. The Company’s institutional archives offer insight into the issues at stake in the printed book matters. This article focuses on the role of the Company in local censorship, which diminished over the period under study. The Company’s censorship function enabled it to exert concrete influence on the global scale of Reformed publishing. This influence was the consequence of the Company’s ecclesiastical and theological authority. This authority derived from the status of the Church of Geneva as the principal church and birthplace of the Reformation in the 16th century. An analysis of the metaphors signifying and symbolizing this role in the printed books themselves underlines the pre-eminence of the Church of Geneva in 17th-century Reformation.

Evaluating Microsoft Bing with ChatGPT-4 for the assessment of abdominal computed tomography and magnetic resonance images.

To evaluate the performance of Microsoft Bing with ChatGPT-4 technology in analyzing abdominal computed tomography (CT) and magnetic resonance images (MRI). A comparative and descriptive analysis was conducted using the institutional picture archiving and communication systems. A total of 80 abdominal images (44 CT, 36 MRI) that showed various entities affecting the abdominal structures were included. Microsoft Bing's interpretations were compared with the impressions of radiologists in terms of recognition of the imaging modality, identification of the imaging planes (axial, coronal, and sagittal), sequences (in the case of MRI), contrast media administration, correct identification of the anatomical region depicted in the image, and detection of abnormalities. Microsoft Bing detected that the images were CT scans with 95.4% accuracy (42/44) and that the images were MRI scans with 86.1% accuracy (31/36). However, it failed to detect one CT image (2.3%) and misidentified another CT image as an MRI (2.3%). On the other hand, it also misidentified four MRI as CT images (11.1%) and one as an X-ray (2.7%). Bing achieved an 83.75% success rate in correctly identifying abdominal regions, with 90% accuracy for CT scans (40/44) and 77.7% for MRI scans (28/36). Concerning the identification of imaging planes, Bing achieved a success rate of 95.4% for CT images and 83.3% for MRI. Regarding the identification of MRI sequences (T1-weighted and T2-weighted), the success rate was 68.75%. In the identification of the use of contrast media for CT scans, the success rate was 64.2%. Bing detected abnormalities in 35% of the images but achieved a correct interpretation rate of 10.7% for the definite diagnosis. While Microsoft Bing, leveraging ChatGPT-4 technology, demonstrates proficiency in basic task identification on abdominal CT and MRI, its inability to reliably interpret abnormalities highlights the need for continued refinement to enhance its clinical applicability. The contribution of large language models (LLMs) to the diagnostic process in radiology is still being explored. However, with a comprehensive understanding of their capabilities and limitations, LLMs can significantly support radiologists during diagnosis and improve the overall efficiency of abdominal radiology practices. Acknowledging the limitations of current studies related to ChatGPT in this field, our work provides a foundation for future clinical research, paving the way for more integrated and effective diagnostic tools.

Acantholytic Dyskeratoses of the Vulva: Clinicopathologic Characterization of 16 Cases and Review of the Literature.

The vulva and perineum are rarely involved by acantholytic dyskeratoses, including Hailey-Hailey disease, Darier disease, papular acantholytic dyskeratosis of the genitocrural area, acantholytic dyskeratotic acanthoma, and warty dyskeratoma. These entities show broad histomorphologic overlap, generally requiring clinical correlation for definitive classification. This institutional series aims to better characterize vulvar acantholytic dyskeratoses and provide a practical literature review and diagnostic aid for gynecologic pathologists. Our institutional archives contained 16 vulvar acantholytic dyskeratoses diagnosed between 1990 and 2023. Affected patients were 36 to 79 (mean, 58) years old and presented with one or more asymptomatic (n = 9) or pruritic (n = 6) lesions involving the vulva (predominantly the labia majora), with additional perineal involvement in 2. Four patients have known Hailey-Hailey disease. Eleven cases comprised singular, raised, erythematous, or skin-colored papules, measuring 0.2 to 0.6 (mean, 0.3) cm. Two patients had oligofocal (both with known Hailey-Hailey disease) vulvar lesions, and 2 had multifocal vulvar lesions (one with known Hailey-Hailey disease). Histologically, all showed acantholysis and dyskeratoses (abundant in 8, focal in 8, with corps ronds generally more conspicuous than corps grains). Additional features included suprabasal clefting (n = 14), dermal papillomatosis (n = 12), and acanthosis (n = 8). Adnexal involvement was rare (n = 1). No histologic features reliably distinguished sporadic versus syndromic acantholytic dyskeratoses. Sporadic lesions were cured by local excision. Patients with Hailey-Hailey disease were variably responsive to corticosteroids. Neither our series nor the literature indicate a significant correlation between sporadic or syndromic acantholytic dyskeratosis and squamous cell carcinoma. Important differential diagnoses include pemphigus vulgaris and pemphigus vegetans, for which direct immunofluorescence may be performed, when indicated.