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Магнитная резонация, изображающая осаждение внутриутробного жира в клубнеобразном склерозе

Клубничный склерозный комплекс (TSC) представляет собой редкий автосомный доминирующий нейрокутановый синдром. Фенотип сильно изменяется и может влиять на несколько систем органов, при этом симптомом заболевания является широко распространенная гамартома или аномальное развитие нормальных тканей. Хотя сердечные хабдомиомы являются наиболее распространенным сердечным проявлением ТСК, разрабатываемым довольно рано, даже в течение второго семестра беременности, они, как правило, спонтанно регрессируют в течение нескольких месяцев или лет. С другой стороны, наличие внутрикардиального осаждения жир

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… Manifestations of the disease in this patient included facial angiofibromas (Adenoma sebaceum), large bilateral renal angiomyolipomas, subependymal giant cell astrocytomas resected 20 and 2 years earlier, focal epilepsy, patches of retinal pigment epithelial hypopigmentation and retinal hamartomas. …

Pathogenesis of Oral Toxicities Associated with Targeted Therapy and Immunotherapy.

Targeted therapy and immunotherapy have redefined cancer treatment. While they have enhanced tumor response and improved survival rates in many cancer types, toxicities continue to occur, and these often involve the oral cavity. Broadly reported as "mucositis" or "stomatitis," oral toxicities induced by targeted therapies differ clinically and mechanistically from those associated with conventional chemotherapy. Manifesting primarily as mucosal lesions, salivary gland hypofunction, or orofacial neuropathies, these oral toxicities may nonetheless lead to significant morbidity and impact patient

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… Hyperpigmentation ( Table 1 ) Pigmentary changes are a well described AE of imatinib mesylate, with hypopigmentation being reported far more often than hyperpigmentation [ 83 ]. … Imatinib is thought to inhibit ligand binding to specific receptors on the surface of human melanocytes, which reduces cellular activity and may cause hypopigmentation and, less frequently, hyperpigmentation of the skin and mucosa [ 90 ]. …

Evaluation of toceranib for treatment of apocrine gland anal sac adenocarcinoma in dogs.

BackgroundThere is no widely accepted standard medical treatment for apocrine gland anal sac adenocarcinoma (AGASACA) in dogs. Targeted agents such as toceranib may be effective in treatment of AGASACA, but the number of clinical reports investigating its efficacy is limited.Hypothesis/aimTo evaluate the efficacy of toceranib treatment of AGASACA in dogs, and to assess prognostic factors in the study population. Our hypothesis was that toceranib would provide a clinical benefit in the treatment of dogs with AGASACA.AnimalsThirty-six client-owned dogs with either a cytologic or histologic diagn

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… Table 3 Adverse events Adverse event | Grade 1 | Grade 2 | Grade 3 | Grade 4 | Grade 5 Diarrhea | 9 | 6 | 0 | 0 | 0 Anorexia | 6 | 4 | 0 | 0 | 0 Vomiting | 1 | 1 | 0 | 0 | 0 Nausea | 1 | 0 | 0 | 0 | 0 Hypertension | 0 | 1 | 1 | 0 | 0 Proteinuria | 1 | 0 | 0 | 0 | 0 Dehydration | 0 | 1 | 0 | 0 | 0 Hypopigmentation …

Skin-Directed Therapies in Mycosis Fungoides: An Update.

Mycosis fungoides (MF) is the most common subtype of cutaneous T cell lymphoma (CTCL), characterized by monoclonal proliferation of malignant T cells primarily involving the skin. The management of MF, especially in the early stages, involves skin-directed therapies (SDTs) to achieve disease control, alleviate symptoms, and enhance the quality of life (QoL). This review provides an updated overview of SDTs in MF treatment, including topical agents such as corticosteroids, retinoids, mechlorethamine, carmustine, imiquimod, as well as photodynamic therapy (PDT); phototherapy modalities including

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… For patients with higher Fitzpatrick skin phototypes, chlormethine gel may be a more suitable option compared to certain other skin-directed therapies, especially topical steroids, which can lead to hypopigmentation in individuals with darker skin tones. … Long-term adverse effects include hypopigmentation, alopecia, telangiectasias, and, rarely, radiation-induced secondary malignancies. In the Piccinno et al. study, no serious complications were reported, and cosmetic outcomes were acceptable or good in over 90% of patients [ 134 ]. …

Mucocutaneous Findings in Hematolymphoid Neoplasms: An Observational Study.

BackgroundCutaneous manifestations of hematological neoplasms can be divided into three broad categories - direct infiltration, paraneoplastic conditions, and those due to the treatment of hematological cancers.ObjectivesTo study the frequency and patterns of mucocutaneous manifestations in patients with hematolymphoid neoplasms and those due to chemotherapy.Materials and methodsThis was an observational study done with 172 patients. Categorization of mucocutaneous manifestations was done into malignancy-associated and chemotherapeutic drugs-associated and data was analyzed.ResultsOut of a tot

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… cell infiltrates | 1 (0.6%) | Acanthosis nigricans | 2 (1.2%) | Cutaneous hyperpigmentation | 54 (31.4%) | Pruritus without skin lesions | 2 (1.2%) | Oral findings | 42 (24.4%) | Acquired perforating dermatosis | 1 (0.6%) | Xerosis/Dry skin | 32 (18.6%) | Sweet’s syndrome | 1 (0.6%) | Cutaneous hypopigmentation …

Imatinib and dasatinib as salvage therapy for sclerotic chronic graft-vs-host disease.

AimTo assess the toxicity, tolerance, steroid-sparing capacity, effectiveness, and response rate to imatinib and dasatinib for the treatment of severe sclerotic chronic graft-vs-host disease (scGVHD).MethodsThis retrospective study analyzed 8 consecutive patients with severe refractory scGVHD who received salvage therapy with imatinib. Patients intolerant and/or refractory to imatinib received dasatinib treatment.Results7 patients discontinued imatinib treatment (1 achieved complete response, 5 were resistant and/or intolerant, and 1 developed grade IV neutropenia) and 1 patient achieved prolo

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… | Yes | Yes | | Yes | | Yes | | Yes | Yes hidebound | Yes | Yes | Yes | | Yes | | Yes | Yes | Yes | | Yes | | Yes | | Yes | Yes superficial sclerosis | | | | Yes | | Yes | | | | | | Yes | | Yes | | hyperpigmentation | | | | | Yes | | Yes | Yes | | | | | | | Yes | Yes hypopigmentation …

Cutaneous Skin Toxicities of Oncological Targeted Therapies: Comprehensive Overview of Mechanisms, Management, and Research Gaps-A Narrative Review.

Targeted therapies in oncology have become a mainstay in therapy of numerous types of cancers. They work by targeting and utilizing (activating, inhibiting, or modulating) molecular pathways that are crucial in pathogenesis of certain cancers and can be divided into numerous classes according to their mechanism. However, since the antigens and molecules they target can take part in homeostasis of the skin and the integument, cutaneous toxicities are a common occurrence. The frequency of certain cutaneous adverse events (CAEs) depends on the causal drug and varies even between drugs of the same

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… Rash maculopapular Macules/papules < 10% BSA with/without symptoms (e.g., pruritus) Macules/papules 10–30% BSA with/without symptoms; limits instrumental ADL Rash covering > 30% BSA with or without mild symptoms Macules/papules > 30% BSA with moderate/severe symptoms; limits self-care ADL – – Skin hypopigmentation … maculopapular | Macules/papules < 10% BSA with/without symptoms (e.g., pruritus) | Macules/papules 10–30% BSA with/without symptoms; limits instrumental ADL Rash covering > 30% BSA with or without mild symptoms | Macules/papules > 30% BSA with moderate/severe symptoms; limits self-care ADL | – | – Skin hypopigmentation …

Oral Graft-Versus-Host Disease: A Pictorial Review and a Guide for Dental Practitioners.

IntroductionGraft-versus-host disease (GVHD) is a complication of haematopoietic stem cell transplantation (HSCT). GVHD may also develop following solid transplants or blood transfusions if white blood cells are transferred. GVHD affects multiple organs, including the oral tissues.ObjectiveThis pictorial review provides a background of GVHD to dental practitioners, describes the most common oral manifestations of GVHD and highlights the main treatment modifications needed to deliver dental care to patients with GVHD.MethodsA narrative review enhanced with clinical photographs.ResultsAcute GVHD

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… entities | Common manifestations Skin | • Poikiloderma | • Depigmentation | • Sweat impairment | • Erythema | • Lichen planus-like features | • Papulosquamous lesions | • Ichthyosis | • Maculopapular rash | • Sclerotic features | | • Keratosis pilaris | • Pruritus | • Morphea-like features | | • Hypopigmentation …

Integumentary System

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… Hypopigmentation refers to decreased melanin in the epidermis. …

Treatments for Morton's neuroma.

BACKGROUND: Morton's neuroma (MN) is a painful neuropathy resulting from a benign enlargement of the common plantar digital nerve that occur … icipants; very low-certainty evidence). Adverse events for CS+LA included mild skin atrophy (3.9%), hypopigmentation of the skin (3.9%) and plantar fat pad atrophy (2.6%); no adverse events were observed with LA. Ultrasound-guided (UG) CS+LA versus non-ultrasound-guided (NUG) CS+LA Two RCTs compared UG CS+LA versus NUG CS+LA. At six months: • UG CS+LA probably red …

The Role of Platelet Dysfunctions in the Pathogenesis of the Hemostatic-Coagulant System Imbalances.

Platelet dysfunction plays a critical role in the pathogenesis of various disorders affecting the hemostatic-coagulant system. This review aims to explore the mechanisms by which platelet dysfunctions contribute to the disruption of hemostasis, leading to an increased risk of both thrombosis and bleeding. Platelets, traditionally known for their role in clot formation, can exhibit altered functionality under pathological conditions such as cardiovascular diseases, metabolic disorders, and autoimmune diseases, impacting their interaction with coagulation factors and vascular endothelium. The re

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… HPS mouse models closely resemble human disease phenotypes, including hypopigmentation, platelet granule deficiency, and genotype-specific macrophage activation. …

Усовершенствованная последовательность транскриптомов и искусственный интеллект/машинное обучение (AI/ML) в имитируемом псориазом воспалении кератиноцитов человека.

ВВЕДЕНИЕ: Несмотря на то, что модель Keratinocyte HaCAT использовалась в предыдущих исследованиях для изучения воздействия антипсориатических агентов, по-прежнему отсутствует полное понимание механизма имитируемого имимодом распространения и трансдукции сигналов в псориазных кератиноцитах. ЦЕЛИ: Данное исследование было направлено на изучение молекулярных механизмов и путей, связанных с воспалением, подобным псориазу, в результате IMQ в кератиноцитах человека. МАТЕРИАЛЫ И МЕТОДЫ: Клетки HACAT подвергались воздействию различных концентраций IMQ, чтобы вызвать воспаление, аналогичное тому, котор

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… Immune dysregulation and inflammation causing hypopigmentation in post kala-azar dermal leishmaniasis: partners in crime? Trends Parasitol. 2023;39:822–36. doi: 10.1016/j.pt.2023.07.005. 74. Nunez R, Dona I, Cornejo-Garcia JA. …

Kabuki Syndrome-Clinical Review with Molecular Aspects.

Kabuki syndrome (KS) is a rare developmental disorder principally comprised of developmental delay, hypotonia and a clearly defined dysmorphism: elongation of the structures surrounding the eyes, a shortened and depressed nose, thinning of the upper lip and thickening of the lower lip, large and prominent ears, hypertrichosis and scoliosis. Other characteristics include poor physical growth, cardiac, gastrointestinal and renal anomalies as well as variable behavioral issues, including autistic features. De novo or inherited pathogenic/likely pathogenic variants in the KMT2D gene are the most c

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… A KS girl whose parents are first cousins presented with multiple congenital abnormalities, including anorectal malformation—specifically imperforate anus with recto-vestibular fistula—diaphragmatic defect, lower lip pits, hypopigmentation, hypogammaglobulinemia as well as cleft palate. …

Минимально инвазивное плазменное устройство для борьбы с множественным раком кожи Бенигена, связанное с сериями Rare Gendermatoses-Cases, и обзор терапевтических методов.

Фон: Немеланоцитические доброкачественные опухоли кожи включают в себя разнообразную группу повреждений, классифицированных по признаку их клеточного происхождения, таких, как эпидермальные, сосудистые, волокнистые, нейронные, мышечные и аднексальные опухоли. Хотя они часто обнаруживают повреждения в одиночестве, многочисленные опухоли кожи сосредотачиваются на генодерматозах. Каждый синдром обладает различными клиническими характеристиками и потенциальными осложнениями, включая категорические и внекачественные недуги, некоторые из которых могут представлять опасность для жизни. Диагнозные ген

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… The adverse effects include persistent erythema, hyperpigmentation, hypopigmentation, infections, scarring, acneiform eruptions, milia, and ectropion [ 72 , 126 , 127 , 128 ]. …

Glycoprotein Non-Metastatic Protein B: An Emerging Biomarker for Lysosomal Dysfunction in Macrophages.

Several diseases are caused by inherited defects in lysosomes, the so-called lysosomal storage disorders (LSDs). In some of these LSDs, tissue macrophages transform into prominent storage cells, as is the case in Gaucher disease. Here, macrophages become the characteristic Gaucher cells filled with lysosomes laden with glucosylceramide, because of their impaired enzymatic degradation. Biomarkers of Gaucher cells were actively searched, particularly after the development of costly therapies based on enzyme supplementation and substrate reduction. Proteins selectively expressed by storage macrop

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… In humans, a truncated version of GPNMB is associated with hyper- and hypopigmentation of the skin in an autosomal recessive variant of Amyloidosis cutis dyschromica (ACD) [ 125 ]. Unlike its homologue PMEL17, GPNMB expression is not restricted to melanocytes. GPNMB has received multiple names. …

Skin manifestations associated with systemic diseases - Part II.

The skin, by reflecting internal processes, externalizes what happens inside the body in many diseases. Thus, the skin, as an organ, extrapolates its functions of protection, barrier and signals the existence of systemic diseases, expanding the importance of the dermatologist beyond the skin surface. Thus, the dermatologist investigates diagnostic hypotheses for conditions related to all systems and refers patients to the appropriate specialty. Combined with examination by a trained eye, the skin, due to its easy access, is still the ideal place for performing biopsies, which often clarify the

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… Cutaneous changes secondary to pruritus: excoriations, prurigo nodularis, post-inflammatory hyper or hypopigmentation, chronic lichen simplex. Xerosis: 50%–80% of patients on hemodialysis. The pathophysiology of skin surface dryness is not well understood and causes pruritus. …

PPAR-γ in Melanoma and Immune Cells: Insights into Disease Pathogenesis and Therapeutic Implications.

Changes in skin pigmentation, like hyperpigmentation or moles, can affect appearance and social life. Unlike locally containable moles, malignant melanomas are aggressive and can spread rapidly, disproportionately affecting younger individuals with a high potential for metastasis. Research has shown that the peroxisome proliferator-activated receptor gamma (PPAR-γ) and its ligands exhibit protective effects against melanoma. As a transcription factor, PPAR-γ is crucial in functions like fatty acid storage and glucose metabolism. Activation of PPAR-γ promotes lipid uptake and enhances sensitivi

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… The common changes include two main categories: hyperpigmentation and hypopigmentation. … In turn, hypopigmentation, which is a reduced biosynthesis of melanin, typically refers to vitiligo, albinism, and tinea versicolor [ 3 ]. …

Chronic pruritus: a narrative review.

Chronic pruritus encompasses a manifestation of several cutaneous, allergic, infectious, neurological, psychological, and systemic conditions, whose etiological investigation and therapeutic strategy can be challenging. This comprehensive review aims to enhance the understanding of pruritus by highlighting important elements in its pathogenesis, including keratinocytes, Merkel cells and mast cells, nerve fibers, histaminergic and nonhistaminergic pathways, and the interaction of itch signals with the central nervous system. Diagnostic evaluation of chronic pruritus may require a meticulous app

Skin manifestations associated with systemic diseases - Part I.

The skin demonstrates what is happening in the body in many diseases, as it reflects some internal processes on the surface. In this sense, skin as an organ, goes beyond its protective and barrier functions, as it provides clues for the identification of some systemic diseases. The dermatologist then raises diagnostic hypotheses for conditions related to all systems and refers them to the appropriate specialty. With easy access to examination by trained eyes and biopsies, the skin can present specific or non specific alterations on histopathology. In the first case this combination establishes

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… High phototypes may present clinically with hyperpigmentation or hypopigmentation, even after resolution of inflammation. It is commonly associated with SLE with positive anti-Ro (SSA) antibodies. Photosensitivity is an important factor in LE in all skin types. …

Navigating Stomatologic Complications Secondary to Antineoplastic Agents-A Comprehensive Review.

This review systematically examines the oral complications associated with conventional and novel anti-cancer therapies. It highlights that while molecularly targeted agents including monoclonal antibodies targeting the vascular endothelial growth factor and its receptor, the epidermal growth factor receptor, tyrosine kinase inhibitors, and immune checkpoint inhibitors tend to exhibit a lower overall toxicity profile compared to traditional cytotoxic chemotherapeutics, they are nonetheless linked to significant oral adverse events. These complications encompass inflammatory mucosal reactions k

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… Pigmentations Pigmentary alterations represent a well-documented adverse effect of imatinib mesylate, with hypopigmentation being reported far more frequently than hyperpigmentation [ 87 ]. …