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Specific (leukemia cutis) and nonspecific (reactive or secondary) skin lesions are associated with systemic leukemia. The following categories of leukemia cutis are discussed in this article: myelogenous (granulocytic) leukemia, monocytic leukemia, myelomonocytic leukemia, lymphocytic and lymphoblastic leukemia, hairy cell leukemia, and adult T-cell leukemia/lymphoma. The temporal relationship between the diagnoses of systemic leukemia and leukemia cutis, the course, and the prognosis are also discussed. Other sites of extramedullary involvement are correlated with leukemia cutis. The appearance of specific skin lesions in leukemia is usually associated with a very poor prognosis.
Cutis laxa is a rare skin disorder characterized by wrinkled, redundant, inelastic and sagging skin due to defective synthesis of elastic fibers and other proteins of the extracellular matrix. Wrinkled, inelastic skin occurs in many cases as an acquired condition. Syndromic forms of cutis laxa, however, are caused by diverse genetic defects, mostly coding for structural extracellular matrix proteins. Surprisingly a number of metabolic disorders have been also found to be associated with inherited cutis laxa. Menkes disease was the first metabolic disease reported with old-looking, wrinkled skin. Cutis laxa has recently been found in patients with abnormal glycosylation. The discovery of the COG7 defect in patients with wrinkled, inelastic skin was the first genetic link with the Congenital Disorders of Glycosylation (CDG). Since then several inborn errors of metabolism with cutis laxa have been described with variable severity. These include P5CS, ATP6V0A2-CDG and PYCR1 defects. In spite of the evolving number of cutis laxa-related diseases a large part of the cases remain genetically unsolved. In metabolic cutis laxa syndromes the clinical and laboratory features might partially overlap, however there are some distinct, discriminative features. In this review on metabolic diseases causing cutis laxa we offer a practical approach for the differential diagnosis of metabolic cutis laxa syndromes.
OBJECTIVE: To describe characteristics and treatment of patients with calcinosis cutis in the clinical setting of autoimmune connective tissue disease (ACTD). DESIGN: Retrospective study. SETTING: Tertiary referral center. PATIENTS: Seventy-eight patients with calcinosis cutis and ACTD between 1996 and 2009. MAIN OUTCOME MEASURES: Clinical features, treatments, and outcomes of patients with calcinosis cutis in the clinical setting of ACTD. RESULTS: Of 78 patients (mean age at onset of calcinosis cutis, 40.1 years), 64 (82%) were female. The following diseases were associated with calcinosis cutis: dermatomyositis (n = 30) with classic (n = 15), juvenile (n = 14), and amyopathic (n = 1) subtypes; systemic sclerosis with limited cutaneous scleroderma (n = 24); lupus panniculitis (n = 4); systemic lupus erythematosus (n = 2); mixed connective tissue disease (n = 4); overlap connective tissue disease (n = 6); undifferentiated connective tissue disease (n = 6); polymyositis (n = 1); and rheumatoid arthritis (n = 1). Therapy for calcinosis cutis consisted of medical treatment alone (n = 19), surgical therapy alone (n = 11), combined medical and surgical treatment (n = 17), no treatment (n = 30), and unknown (n = 1). Diltiazem hydrochloride was the most commonly used medical therapy, with 9 of 17 patients having a partial response. Twenty-eight patients had surgical excision of 1 or more lesions of calcinosis cutis: 22 had a complete response, 5 had a partial response, and 1 had no response. CONCLUSIONS: Dermatomyositis and systemic sclerosis were the most common ACTDs associated with calcinosis cutis. Although no treatment was uniformly effective, surgical excision of symptomatic lesions and medical treatment with diltiazem provided benefit for some patients.
Hereditary cutis laxa comprises a heterogeneous group of connective tissue disorders characterized by loose skin and variable systemic involvement. Autosomal dominant and recessive as well as X-linked forms have been described. Some dominant forms are caused by mutations in the elastine gene (ELN). The X-linked form is now classified in the group of copper transport diseases. The genetic defect underlying the autosomal recessive (AR) forms of cutis laxa is not known. The phenotypic abnormalities recently observed in a fibulin-5 knockout mouse model are reminiscent of human AR cutis laxa type I. Both share cutis laxa, lung emphysema and arterial involvement. Molecular study of the fibulin-5 (FBLN5) gene in a large consanguineous Turkish family with four patients affected by AR cutis laxa type I demonstrated the presence of a homozygous missense mutation (T998C) in the FBLN5 gene resulting in a serine-to-proline (S227P) substitution in the fourth calcium-binding epidermal growth factor-like domain of fibulin-5 protein. This amino acid substitution is predicted to have important structural and functional consequences for normal elastogenesis. As such, we provide evidence that a genetic defect in fibulin-5 (FBLN5, also known as EVEC or DANCE) is responsible for a recessive form of cutis laxa in humans.
BACKGROUND: Cutis laxa is an acquired or inherited condition characterized by redundant, pendulous and inelastic skin. Autosomal dominant cutis laxa has been described as a benign disease with minor systemic involvement. OBJECTIVE: To report a family with autosomal dominant cutis laxa and a young girl with sporadic cutis laxa, both with variable expression of an aortic aneurysmal phenotype ranging from mild dilatation to severe aneurysm or aortic rupture. METHODS AND RESULTS: Histological evaluation of aortic aneurysmal specimens indicated classical hallmarks of medial degeneration, paucity of elastic fibres, and an absence of inflammatory or atherosclerotic lesions. Electron microscopy showed extracellular elastin deposits lacking microfibrillar elements. Direct sequencing of genomic amplimers detected defects in exon 30 of the elastin gene in affected individuals, but did not in 121 normal controls. The expression of mutant elastin mRNA forms was demonstrated by reverse transcriptase polymerase chain reaction analysis of cutis laxa fibroblasts. These mRNAs coded for multiple mutant tropoelastins, including C-terminally truncated and extended forms as well as for molecules lacking the constitutive exon 30. CONCLUSIONS: ELN mutations may cause severe aortic disease in patients with cutis laxa. Thus regular cardiac monitoring is necessary in this disease to avert fatal aortic rupture.
BACKGROUND AND RATIONALE: Xerosis cutis (also referred to as xeroderma, dry skin, asteatosis) affects more than 10 million individuals in Germany. It is among the most common dermatological diagnoses and a cardinal symptom of many dermatological, internal and neurological diseases. Even though it has been established that basic skin care plays a significant role in the management of patients with xerosis cutis, there are as yet no evidence-based algorithms for diagnosis and treatment. OBJECTIVE: The present position paper provides physicians across all specialties with a practical, symptom-based approach to the prevention, diagnosis and treatment of xerosis cutis. METHODS: Within a structured decision-making process, a panel of experienced dermatologists first defined questions relevant to everyday clinical practice, which were then addressed by a systematic review of the literature. Based on the evidence available as well as expert consensus, diagnostic and treatment algorithms were subsequently developed and agreed upon. RESULTS: Xerosis cutis is generally diagnosed on clinical grounds. Possible trigger factors must be avoided, and comorbidities should be adequately and specifically treated. Suitable skin care products should be chosen with a view to improving skin hydration and restoring its barrier function. They should therefore contain both rehydrating and lipid-replenishing components. The "drier" the skin appears, the greater the lipid content should be (preferably using water-in-oil formulations). The choice of ingredients is based on a patient's individual symptoms, such as scaling (e.g., urea), fissures/rhagades (e.g., urea or dexpanthenol), erythema (e.g., licochalcone A) and pruritus (e.g., polidocanol). Other factors to be considered include the site affected and patient age. Ingredients or rather combinations thereof for which there is good clinical evidence should be preferentially used. The best evidence by far is available for urea, whose efficacy in the treatment of xerosis is further enhanced by combining it with other natural moisturizing components and ceramides. The "xerosimeter" is a tool developed in an effort to facilitate patient management and for training purposes. It not only includes practical tools for diagnosis and follow-up but also a classification of ingredients and a structured treatment algorithm. CONCLUSION: The structured symptom- and evidence-based approach proposed herein contains a road map for diagnosis and treatment of xerosis cutis. It aims to raise awareness in terms of prevention and early treatment of this condition and may thus improve quality of life and prevent potential sequelae.
BACKGROUND: The skin and the nervous system are both derived from ectoderm. Separation of neural ectoderm from epithelial ectoderm occurs concurrently with the closure of the neural tube. This chronologic association may explain the cutaneous abnormalities often found overlying neural tube defects. A ring of dark long hair encircling a congenital scalp lesion (the hair collar sign) is one such marker and is often associated with encephaloceles, meningoceles, and heterotopic brain tissue. OBSERVATIONS: We describe six children with aplasia cutis who displayed the hair collar sign. Aplasia cutis is a relatively heterogeneous disorder; however, these lesions had a unique and strikingly similar appearance. This subtype of aplasia cutis, which we have termed membranous aplasia cutis, shares several clinical and histologic features with cranial neural tube defects. CONCLUSIONS: We propose that membranous aplasia cutis is a form fruste of a neural tube defect and may be derived from a similar embryological defect. Recent advances in the understanding of cranial neural tube closure may provide support for this hypothesis.
<h3>Background:</h3> Congenital leukemia cutis is rarely reported in the dermatology literature despite various authors citing 50% of infants with congenital leukemia have skin involvement. These seemingly disparate facts prompted a review of the world literature that was performed by searching the MEDLINE database from 1966 up to and through December 1992, reviewing<i>Index Medicus</i>for the years prior to computerized MEDLINE search, and reviewing appropriate case report references. <h3>Observations:</h3> A 2-month premature male infant was born with 182×10<sup>9</sup>/L circulating monoblasts, prominent hepatosplenomegaly, and multiple firm blue and red cutaneous nodules. <h3>Conclusion:</h3> Approximately 175 cases of congenital leukemia have been reported with at least 41 and possibly 56 cases identified with leukemia cutis. Specific cutaneous leukemic infiltrates occur in 25% to 30% of infants with congenital leukemia and usually appear as firm blue, red, or purple nodules in a generalized distribution. In contrast to the 50% incidence of gingival and oral infiltrates reported in adult monocytic leukemia, only 1% of congenital leukemia patients have oral involvement. Congenital leukemia cutis may precede other manifestations of leukemia by as much as 4 months. Whereas leukemia cutis is associated with a poor prognosis in adult leukemics, the natural history of congenital leukemia is not altered by leukemia cutis. (<i>Arch Dermatol.</i>1993;129:1301-1306)
Video object segmentation (VOS) is a challenging task with wide applications such as video editing and autonomous driving. While Cutie provides strong query-based segmentation and SAM2 offers enriched representations via a pretrained ViT encoder, each has limitations in feature capacity and temporal modeling. In this report, we propose a framework that integrates their complementary strengths by replacing the encoder of Cutie with the ViT encoder of SAM2 and introducing a motion prediction module for temporal stability. We further adopt an ensemble strategy combining Cutie, SAM2, and our variant, achieving 3rd place in the MOSEv2 track of the 7th LSVOS Challenge. We refer to our final model as SCOPE (SAM2-CUTIE Object Prediction Ensemble). This demonstrates the effectiveness of enriched feature representation and motion prediction for robust video object segmentation. The code is available at https://github.com/2025-LSVOS-3rd-place/MOSEv2_3rd_place.
First-principle based molecular-dynamics simulations have been performed for binary Cu$_x$Ti$_{1-x}$ (x = 0.31, 0.50, and 0.76) alloys to investigate the relationship between local structure and dynamical properties in the liquid and undercooled melt. The undercooled melts show a pronounced short-range order, majorly a five-fold symmetry (FFS) around the Cu atoms, which competes with bcc ordering. This complex SRO is also reflected in the partial coordination numbers, where mainly a Z12 coordination is present around Cu, which corresponds to an icosahedral ordering. Higher coordination numbers were obtained for Ti compatible with Frank-Kasper polyhedra. The increasing Frank-Kasper polyhedra coordination scenario around Ti impacts on the interatomic distances of Ti atoms, which increase with increasing Ti content. The Cu$_{50}$Ti$_{50}$ composition exhibits the highest FFS ordering and amount of Frank-Kasper polyhedra, which explains the slowest melt dynamics, found experimentally and in simulations for this composition. Thus, our results suggest that the high undercooling degree and glass-forming ability of binary CuTi alloys, originates from the high complexity of the local struct
Video Object Segmentation (VOS) is a vital task in computer vision, focusing on distinguishing foreground objects from the background across video frames. Our work draws inspiration from the Cutie model, and we investigate the effects of object memory, the total number of memory frames, and input resolution on segmentation performance. This report validates the effectiveness of our inference method on the coMplex video Object SEgmentation (MOSE) dataset, which features complex occlusions. Our experimental results demonstrate that our approach achieves a J\&F score of 0.8139 on the test set, securing the third position in the final ranking. These findings highlight the robustness and accuracy of our method in handling challenging VOS scenarios.
Annolid is a deep learning-based software package designed for the segmentation, labeling, and tracking of research targets within video files, focusing primarily on animal behavior analysis. Based on state-of-the-art instance segmentation methods, Annolid now harnesses the Cutie video object segmentation model to achieve resilient, markerless tracking of multiple animals from single annotated frames, even in environments in which they may be partially or entirely concealed by environmental features or by one another. Our integration of Segment Anything and Grounding-DINO strategies additionally enables the automatic masking and segmentation of recognizable animals and objects by text command, removing the need for manual annotation. Annolid's comprehensive approach to object segmentation flexibly accommodates a broad spectrum of behavior analysis applications, enabling the classification of diverse behavioral states such as freezing, digging, pup huddling, and social interactions in addition to the tracking of animals and their body parts.
Video Object Segmentation (VOS) presents several challenges, including object occlusion and fragmentation, the dis-appearance and re-appearance of objects, and tracking specific objects within crowded scenes. In this work, we combine the strengths of the state-of-the-art (SOTA) models SAM2 and Cutie to address these challenges. Additionally, we explore the impact of various hyperparameters on video instance segmentation performance. Our approach achieves a J\&F score of 0.7952 in the testing phase of LSVOS challenge VOS track, ranking third overall.
Referring video object segmentation (RVOS) relies on natural language expressions to segment target objects in video. In this year, LSVOS Challenge RVOS Track replaced the origin YouTube-RVOS benchmark with MeViS. MeViS focuses on referring the target object in a video through its motion descriptions instead of static attributes, posing a greater challenge to RVOS task. In this work, we integrate strengths of that leading RVOS and VOS models to build up a simple and effective pipeline for RVOS. Firstly, We finetune the state-of-the-art RVOS model to obtain mask sequences that are correlated with language descriptions. Secondly, based on a reliable and high-quality key frames, we leverage VOS model to enhance the quality and temporal consistency of the mask results. Finally, we further improve the performance of the RVOS model using semi-supervised learning. Our solution achieved 62.57 J&F on the MeViS test set and ranked 1st place for 6th LSVOS Challenge RVOS Track.
We present Cutie, a video object segmentation (VOS) network with object-level memory reading, which puts the object representation from memory back into the video object segmentation result. Recent works on VOS employ bottom-up pixel-level memory reading which struggles due to matching noise, especially in the presence of distractors, resulting in lower performance in more challenging data. In contrast, Cutie performs top-down object-level memory reading by adapting a small set of object queries. Via those, it interacts with the bottom-up pixel features iteratively with a query-based object transformer (qt, hence Cutie). The object queries act as a high-level summary of the target object, while high-resolution feature maps are retained for accurate segmentation. Together with foreground-background masked attention, Cutie cleanly separates the semantics of the foreground object from the background. On the challenging MOSE dataset, Cutie improves by 8.7 J&F over XMem with a similar running time and improves by 4.2 J&F over DeAOT while being three times faster. Code is available at: https://hkchengrex.github.io/Cutie
As scientific and technological advancements result from human intellectual labor and computational costs, protecting model intellectual property (IP) has become increasingly important to encourage model creators and owners. Model IP protection involves preventing the use of well-trained models on unauthorized domains. To address this issue, we propose a novel approach called Compact Un-Transferable Isolation Domain (CUTI-domain), which acts as a barrier to block illegal transfers from authorized to unauthorized domains. Specifically, CUTI-domain blocks cross-domain transfers by highlighting the private style features of the authorized domain, leading to recognition failure on unauthorized domains with irrelevant private style features. Moreover, we provide two solutions for using CUTI-domain depending on whether the unauthorized domain is known or not: target-specified CUTI-domain and target-free CUTI-domain. Our comprehensive experimental results on four digit datasets, CIFAR10 & STL10, and VisDA-2017 dataset demonstrate that CUTI-domain can be easily implemented as a plug-and-play module with different backbones, providing an efficient solution for model IP protection.
Bayesian inference paradigms are regarded as powerful tools for solution of inverse problems. However, when applied to inverse problems in physical sciences, Bayesian formulations suffer from a number of inconsistencies that are often overlooked. A well known, but mostly neglected, difficulty is connected to the notion of conditional probability densities. Borel, and later Kolmogorov's (1933/1956), found that the traditional definition of conditional densities is incomplete: In different parameterizations it leads to different results. We will show an example where two apparently correct procedures applied to the same problem lead to two widely different results. Another type of inconsistency involves violation of causality. This problem is found in model selection strategies in Bayesian inversion, such as Hierarchical Bayes and Trans-Dimensional Inversion where so-called hyperparameters are included as variables to control either the number (or type) of unknowns, or the prior uncertainties on data or model parameters. For Hierarchical Bayes we demonstrate that the calculated 'prior' distributions of data or model parameters are not prior-, but posterior information. In fact, the c
Tiny Machine Learning (TinyML) applications impose uJ/Inference constraints, with a maximum power consumption of tens of mW. It is extremely challenging to meet these requirements at a reasonable accuracy level. This work addresses the challenge with a flexible, fully digital Ternary Neural Network (TNN) accelerator in a RISC-V-based System-on-Chip (SoC). Besides supporting Ternary Convolutional Neural Networks, we introduce extensions to the accelerator design that enable the processing of time-dilated Temporal Convolutional Neural Networks (TCNs). The design achieves 5.5 uJ/Inference, 12.2 mW, 8000 Inferences/sec at 0.5 V for a Dynamic Vision Sensor (DVS) based TCN, and an accuracy of 94.5 % and 2.72 uJ/Inference, 12.2 mW, 3200 Inferences/sec at 0.5 V for a non-trivial 9-layer, 96 channels-per-layer convolutional network with CIFAR-10 accuracy of 86 %. The peak energy efficiency is 1036 TOp/s/W, outperforming the state-of-the-art silicon-proven TinyML quantized accelerators by 1.67x while achieving competitive accuracy.
The multidomain protein gelsolin (GSN) is composed of six homologous modules, sequentially named G1 to G6. Single point substitutions in this protein are responsible for AGel amyloidosis, a hereditary disease characterized by progressive corneal lattice dystrophy, cutis laxa, and polyneuropathy. Several different amyloidogenic variants of GSN have been identified over the years, but only the most common D187N/Y mutants, in G2, have been thoroughly characterized, and the underlying functional mechanistic link between mutation, altered protein structure, susceptibility to aberrant furin cleavage and aggregative potential resolved. Little is known about the recently identified mutations A551P, E553K and M517R hosted at the interface between G4 and G5, whose aggregation process likely follows an alternative pathway. We demonstrate that these three substitutions impair temperature and pressure stability of GSN but do not increase its susceptibility to furin cleavage, the first event of the canonical aggregation pathway. The variants are also characterized by a higher tendency to aggregate in the unproteolysed forms and show a higher proteotoxicity in a C. elegans-based assay. Structural
A clever nanoscale redesign may have solved one of superconductivity’s biggest problems。 Researchers in Sweden discovered that by subtly sculpting the surface beneath an ultrathin superconducting material, they could make it stay superconducting at higher temperatures and under much stronger magnetic fields