TEMPI syndrome: A clinical, light-microscopic and phenotypic evaluation with review of the literature.

BACKGROUND AND OBJECTIVES: TEMPI (telangiectasias, elevated erythropoietin and erythrocytosis, monoclonal gammopathy, perinephric fluid collections, and intrapulmonaryshunting) syndrome is a rare multisystemic disease classified as a monoclonal gammopathy of cutaneous significance. The pathogenesis and etiology of TEMPIare not well known because of the rarity of this disorder. Although telangiectasias are the hallmark of this syndrome, skin biopsies are rarely performed. We aim to further characterize TEMPI syndrome through the evaluationof a skin biopsy. METHODS: We reviewed the histopathology and immunophenotypic profile of a skin biopsy from a 53-year-oldwoman diagnosed with TEMPI syndrome. Other components of her syndromic complex included an IgA myeloma, elevated vascular endothelial growth factor (VEGF), and erythrocytosis. RESULTS: A biopsy showed prominent vascular ectasia with some degree of microvascular basement membranezone thickening. Our patient had a reduction in neoplastic plasma cell burdenand clearing of her telangiectasias following myeloma directed treatment. CONCLUSIONS: TEMPI can beviewed as a reactive vascular paraneoplastic syndrome in the setting of a plasma cell dyscrasia. Elaboration of VEGF from neoplastic plasma cells is likely pathogenetically implicated and appears to be a common link that explains other vascular lesions associated with monoclonal gammopathy syndromes.

Primary cutaneous acral CD8-positive T-cell lymphoproliferative disorder: A clinical and histologic retrospective cohort study.

Clonally restricted, non-epidermotropic, low-grade, CD8-positive T-cell infiltrates of the skin was recognized as a unique form of indolent CD8-positive lymphoproliferative disease in 2007 when it was first called primary cutaneous indolent CD8-positive lymphoid proliferation. More recently, the designation of primary cutaneous acral CD8-positive T-cell lymphoproliferative disorder has been used. It is unique as a cutaneous lymphoproliferative disorder because of relative uniformity in its clinical presentation and histomorphology. It has been recognized as having an interesting predilection for the ear and acral sites, characteristically presenting as a solitary lesion. The basic morphology is one characterized by a non-epidermotropic, tumefactive infiltrate of well-differentiated, noncerebriform, atypical, small- to intermediate-sized lymphocytes that exhibit a specific phenotype characterized by CD8 and TIA positivity in concert with a distinct perinuclear Golgi staining pattern for CD68. The typical presentation is in the context of a solitary lesion, which can be treated surgically or with local irradiation. We describe in detail two very unusual cases that expand the clinical spectrum of this condition given the non-acral localization, the multiplicity of lesions to involve the trunk and extremities, and, in one case, the stable but recalcitrant course over 30 years. In addition, the second patient developed paraneoplastic dermatomyositis. We also retrospectively review our database for other cases that represent the entity of primary cutaneous acral CD8-positive T-cell lymphoproliferative disorder and review the literature focusing on non-acral cases. Nomenclature evolution from its first recognition in 2007 to the present is discussed.

Complete Neurological Recovery After Emergency Burr Hole Placement Utilizing EZ-IO® for Epidural Hematoma.

BACKGROUND: Post-traumatic epidural hematoma (EDH) accounts for 1-3% of pediatric closed head injury admissions. There is a 2.5:1 male predominance. Etiology varies by age; motor vehicle collisions are the primary cause of EDH in adolescents. Post-traumatic EDH accompanies up to 4% of adult head injuries, and is associated with 10% mortality in adults and 5% mortality in children. In North America, standard of care for post-traumatic EDH includes decompressive craniotomy or trepanation via burr hole. Such lifesaving care is typically provided in the operating room by consulting neurosurgery teams or other personnel trained in the use of burr hole equipment. CASE REPORT: The case of a 17-year-old female patient who presented to a community emergency department (ED) after being involved in a motor vehicle collision is discussed. At the scene of the accident, she refused emergency medical services transport and was brought to the ED via private vehicle. She quickly decompensated in the ED and required intubation. Neurosurgical services were not available and transport to the nearest pediatric trauma center was delayed due to weather. Decompression and drainage of her EDH was accomplished with an EZ-IO® driver and intraosseous needle under virtual guidance of a pediatric neurosurgeon until definitive care could be obtained. The patient made a full neurologic recovery. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS: EDHs have high morbidity and mortality. In settings without access to neurosurgical services, and where ED access to or familiarity with burr hole equipment is limited, the EZ-IO® device may be a temporizing and lifesaving intervention until definitive neurosurgical care can be obtained.

Human Polyomavirus 9-An Emerging Cutaneous and Pulmonary Pathogen in Solid Organ Transplant Recipients.

IMPORTANCE: We describe the first report to our knowledge of cutaneous and systemic pathogenicity of human polyomavirus 9 in solid organ transplant recipients. OBJECTIVE: Three solid organ transplant recipients developed a widespread, progressive, violaceous, and hyperkeratotic skin eruption. All died from pulmonary and multiorgan failure around 1 year from onset of the rash. Routine clinical diagnostic testing could not identify any causative agent; therefore, samples and autopsies were investigated for novel pathogens using high-throughput sequencing. DESIGN, SETTING, AND PARTICIPANTS: This case series, including 3 solid organ transplant recipients who developed characteristic pink, violaceous, or brown hyperkeratotic papules and plaques throughout the body, was conducted at the Columbia University Medical Center. Lesional skin biopsies were collected from all 3 patients and subjected to high-throughput illumina sequencing for identification of microbial pathogens. Human polyomavirus 9 was identified in lesional skin biopsies. We subsequently collected ocular swabs, oral swabs, urine samples, and blood samples from patients, and organ tissues at autopsy in 1 patient. We investigated these samples for the presence of human polyomavirus 9 using in situ hybridization and quantitative polymerase chain reaction (PCR) assays. MAIN OUTCOMES AND MEASURES: A description of the clinical and pathologic findings of 3 patients. RESULTS: This case series study found that human polyomavirus 9 was detected in the skin biopsies of all 3 patients by a capture-based high-throughput sequencing method platform (VirCapSeq-VERT). Human polyomavirus 9 was also detected in blood, oral, ocular swabs, and urine by real-time polymerase chain reaction (PCR) assay. In situ hybridization and quantitative PCR assays were performed on the skin biopsies from 3 patients and lung autopsy of 1 patient, which showed the presence of human polyomavirus 9 messenger RNA transcripts, indicating active viral replication and pathogenesis in the skin and lungs. CONCLUSIONS AND RELEVANCE: Human polyomavirus 9 was associated with the widespread cutaneous eruption. All 3 patients had progression of cutaneous disease, accompanied by clinical deterioration, pulmonary failure, and death. One patient underwent autopsy and human polyomavirus 9 was identified in the lungs and paratracheal soft tissue. These findings suggest that human polyomavirus 9 may be associated with cutaneous and possibly pulmonary infection and death in solid organ transplant recipients.

Post-COVID-19 vaccination IgA vasculitis in an adult.

Leukocytoclastic vasculitis has been reported in the setting of COVID-19 infection and post-COVID-19 vaccination. We report a case of IgA vasculitis (IgAV) post-COVID-19 vaccination, with immunoglobulin A (IgA) immune deposits in the skin and renal involvement. SARS-CoV spike protein immunohistochemical staining was negative. IgAV with skin and renal involvement is a potential reaction to COVID-19 vaccination.

Cutaneous collagenous vasculopathy: A report of three cases.

Cutaneous collagenous vasculopathy is a rare pauci-inflammatory, superficial, cutaneous vasculopathy characterized by progressive fine-branching telangiectasias clinically, while light microscopically one observes dilated venules and capillaries within the superficial dermis exhibiting excessive Type IV collagen within the vessel wall. We present three cases of collagenous vasculopathy. Two cases were associated with certain autoimmune stigmata, including a positive serologic anti-endothelial cell antibody assay and positive lupus anticoagulant in one, while the third case had positive anti-ribonucleoprotein (RNP) antibodies. The latter case was associated with chronic hydroxyurea therapy for an underlying myeloproliferative disorder. We explore the role of immune- and non-immune-based endothelial cell injury in the pathogenesis of collagenous vasculopathy.

The use of drug calendars for the diagnosis of cutaneous drug eruptions in the age of electronic medical records.

A morbilliform drug eruption is the most common condition leading to a dermatology consultation for a patient in the hospital. Timing is an important diagnostic tool since the onset of a skin rash usually takes place within days-to-weeks of the start of the implicated drug. A comprehensive, thorough, and reliable drug history by the clinician is essential. Therefore, to assist in the task of determining the causative medication of a new skin rash in a hospitalized patient, the creation of a drug calendar is recommended. The development of an electronic version of the drug calendar offers several benefits over the manual version. As the use of electronic medical records continues to become the standard in medicine, the electronic drug calendar will serve as an invaluable tool for the diagnosis of drug hypersensitivity.

Epidermotropic CXCR3 positive marginal zone lymphoma: a distinctive clinical histopathological entity potentially originating in the skin; it does not always indicate splenic marginal zone lymphoma.

Epidermotropic B cell lymphoma represents a rare form of marginal zone lymphoma presenting as a disseminated skin rash resembling pityriasis rosea. To date there are 8 reported cases. In addition to the widespread nature of the skin rash, there is a proclivity for spleen and bone marrow involvement raising consideration regarding its categorization as a systemic lymphoma. We present an 89-year-old man with epidermotropic B cell lymphoma, who presented with a pityriasis rosea-like skin rash. An initial diagnosis of diffuse large cell B cell lymphoma was made based on the extent of dermal-based large cell infiltration. However, after recognizing the epidermotropic component and the distinctive clinical presentation, a diagnosis of epidermotropic B cell lymphoma was rendered. There was minimal bone marrow involvement based only on flow cytometric analysis, but there was no apparent bone marrow or splenic involvement on routine light microscopic assessment. Remission was = achieved with single agent rituximab chemotherapy and the patient remained symptom free. The neoplastic CD20 positive epidermotropic B lymphocytes expressed CXCR3. Similar to the prior reported cases by the authors, the neoplastic cells expressed CXCR3, a chemokine whose organ and tissue specific ligands could contribute to its relatively indolent clinicalcourse.

Monoclonal plasma cell infiltrates in the setting of cutaneous follicular helper T cell lymphoproliferative disorders.

There is a growing recognition that some primary cutaneous T cell lymphomas of the skin exhibit a follicular helper T cell phenotype best exemplified by primary cutaneous CD4+ small/medium sized pleomorphic T cell lymphoma. The follicular helper T cells is an evolutionary function in a common TH1 cell under the influence of other cell types most notably monocyte derived dendritic cells but also plasma cells. In addition, the skin defines a characteristic organ site of involvement for angioimmunoblastic T-cell lymphoma (AITL); the first recognized form of follicular helper T cell lymphoma. One of the hallmarks of the follicular helper T cell lymphomas a significant degree of post germinal center B cell hyperplasia. We encountered 7 cases of primary cutaneous follicular helper T cell and four cases of AITL, in which the biopsies contained a light chain restricted plasma cell infiltrate in the skin. There were no features that suggested an atypical or more aggressive clinical course in association with the identification of this light chain restricted plasmacytic infiltrates except one case of AITL in whom a diffuse large cell B cell lymphoma subsequently developed. There was no association with Epstein-Barr virus (EBV) infection light chain restricted plasma cell infiltrate in any of the eleven cases. The basis of these infiltrates is likely a reciprocal functional one reflecting the role of follicular helper T cells in the induction of B cell hyperplasia and the role of plasma cells as a countercheck balance controlling the extent of follicular helper T cell hyperplasia. B cell clonality, plasma cell atypia and blastic B cell transformation can occur without implying a malignant transformation.

The Role of Magnetic Resonance Imaging in Optimizing Injury Management in Air Force Pararescuemen, Combat Rescue Officers, and Survival Specialists.

Operators perform physically demanding jobs associated with a variety of overuse and acute musculoskeletal injuries. The current management of musculoskeletal complaints in the Air Force includes plane radiographs and 6 weeks of physical therapy (PT) before consideration of orthopedic consultation and magnetic resonance imaging (MRI); however, MRI shows a clear advantage compared with plane radiographs. We conducted a performance improvement project and conclude that (1) MRI allowed for definitive diagnosis as well as definitive triage for care in a timely manner, (2) guidelines for ordering lumbosacral MRIs should be followed and not ordered for pain that is not progressive and severe or not associated with a neurological finding, and (3) because of the risk of X-ray exposure in patients in their 20 and 30s, X-rays should be avoided in this setting unless definitely indicated.

Cutaneous Presentation of Mesothelioma With a Sarcomatoid Transformation.

Malignant pleural mesothelioma is a rare neoplasm of mesodermal origin. Cutaneous involvement of malignant pleural mesothelioma is a very rare entity, with only 11 cases reported in the literature. Here, we describe the case of a 75-year-old man with stage IV epithelioid pleural mesothelioma, presenting with a cutaneous eruption 5 months after initial diagnosis, which revealed sarcomatoid features on skin biopsy. Histological analysis of malignancy progression through immunohistochemical staining of the pleural, lymph node, and skin tissue revealed gradual loss of calretinin and gain of desmin, supporting a transformation from epithelioid to sarcomatoid tissue. To our knowledge, this is the first reported case of an epithelioid to sarcomatoid transformation of malignant pleural mesothelioma manifesting in a cutaneous presentation.