- [Show abstract] [Hide abstract] ABSTRACT: Superficial minor wounds represent frequent events in daily practice. Their evolution is often favourable and without any mishap. However, they possibly evolve to some serious episodes. Normal healing develops in three successive phases. They correspond to the inflammatory, proliferative and remodeling phases, respectively. Their treatments are distinct. The major active agents are cleaning products, antiseptics, corticosteroids and silicone gels. By contrast products claiming some healing properties are disappointing in most cases, and in some instances, they are of no use.
- [Show abstract] [Hide abstract] ABSTRACT: Basal cell carcinomas (BCCs) are the most frequent human cancer. Over 90% of all BCCs have a mutation in PTCH1 or smoothened, two conducting proteins of the Hedgehog pathway. They rarely progress deeply and metastasize; however, if they do, these advanced basal cell carcinoma become amenable to treatment by inhibiting the Hedgehog and the P13K-mTOR pathways. Such innovative drugs include vismodegib, cyclopamine, itraconazole, everolimus and a few other agents that are in early clinical development.
Article: Secrezione sudorale e sebacea
- [Show abstract] [Hide abstract] ABSTRACT: Wound healing following partial thickness thermal burns is commonly hampered by the risk of hypertrophic scarring. Skin myofibroblast (MF) density is commonly increased in postburn healing. The transition between fibroblast-like cells and α-smooth muscle actin (SMA)+ MF possibly begins with CD14+ monocytes, evolving to CD14+ CD34+ fibrocytes, followed by β-SMA+ protomyofibroblast (PMF) maturation. Skin biopsies from 25 burn patients were collected about 1 and 4 weeks after injury. Immunohistochemistry was performed using monoclonal antibodies to α-SMA, β-SMA, factor XIIIa, lysozyme, Mac 387, CD14, CD117 and Ulex europaeus agglutinin-1 (UEA-1). The set of Mac 387+ and CD14+ monocytes was accompanied by both CD34+ fibrocytes and factor XIIIa+ dendrocytes. By contrast, β-SMA+ PMF were rare. Of note, α-SMA+ MF were more abundant at week 4 than at week 1 (p < 0.01). The UEA-1+ endothelial cells showed marked variations in their dermal distribution, irrespective of the densities in the other scrutinized cells. In conclusion, healing of partial thickness thermal burns involves a diversity of cell types including PMF. In the present samples, the PMF density remained low. © 2015 S. Karger AG, Basel.
- [Show abstract] [Hide abstract] ABSTRACT: Ehlers-Danlos syndrome (EDS) represents a heterogeneous group of disorders of the connective tissue structure. Currently, several types are distinguished following a limited set of clinical signs and genetic mutations. However, there is a lack of specificity of most recognized genetic alterations with the current clinical typing. In addition, the criteria from dermatopathology, ultrastructure and biomechanics are not considered. In addition, the established EDS frontiers are hazardous because a series of anatomo-clinical signs are not considered in the classical EDS concept. The hypermobile type EDS represents an example of the diagnostic uncertainties. It results that guidelines based on evidence-based medicine cannot be established. Only an individual management can be offered to the concerned patients.
- [Show abstract] [Hide abstract] ABSTRACT: The melanotic facial pigmentation of each individual is frequently heterogeneous, even when this condition remains imperceptible under natural ambient light. However, with aging, this aspect may appear to everybody. The melanin heterochromia has various origins including ethnicity, the hormonal impact, the influence of various inflammatory, toxic and drug-induced disorders, as well as the impact of photoaging. The cheetah-look aspect is thus established and well identified under ultraviolet light or using an ingenious trick selecting some wavelengths of visible light.
- [Show abstract] [Hide abstract] ABSTRACT: Bi-allelic variants in CHST14, encoding dermatan-4-O-sulfotransferase-1 (D4ST1), cause musculocontractural EDS (MC-EDS), a recessive disorder characterized by connective tissue fragility, craniofacial abnormalities, congenital contractures and developmental anomalies. Recently, the identification of bi-allelic variants in DSE, encoding dermatan sulfate epimerase-1 (DS-epi1), in a child with MC-EDS features, suggested locus heterogeneity for this condition. DS-epi1 and D4ST1 are crucial for biosynthesis of dermatan sulfate (DS) moieties in the hybrid chondroitin sulfate (CS)/DS glycosaminoglycans (GAGs). Here we report four novel families with severe MC-EDS caused by unique homozygous CHST14 variants and the second family with a homozygous DSE missense variant, presenting a somewhat milder MC-EDS phenotype. The glycanation of the dermal DS proteoglycan decorin is impaired in fibroblasts from D4ST1- as well as DS-epi1-deficient patients. However, in D4ST1-deficieny the decorin GAG is completely replaced by CS, whereas in DS-epi1-deficiency still some DS moieties are present. The multisystemic abnormalities observed in our patients support a tight spatiotemporal control of the balance between CS and DS, which is crucial for multiple processes including cell differentiation, organ development, cell migration, coagulation and connective tissue integrity. This article is protected by copyright. All rights reserved. This article is protected by copyright. All rights reserved.
- [Show abstract] [Hide abstract] ABSTRACT: Whereas the vast majority of osteogenesis imperfecta (OI) is caused by autosomal dominant defects in the genes encoding type I procollagen, mutations in a myriad of genes affecting type I procollagen biosynthesis or bone formation and homeostasis have now been associated with rare autosomal recessive OI forms. Recently, homozygous or compound heterozygous mutations in BMP1, encoding the metalloproteases bone morphogenetic protein-1 (BMP1) and its longer isoform mammalian Tolloid (mTLD), were identified in five children with a severe autosomal recessive form of OI and in four individuals with mild-to-moderate bone fragility. BMP1/mTLD functions as the procollagen carboxy-(C)-proteinase for types I-III procollagen, but was also suggested to participate in amino-(N)-propeptide cleavage of types V and XI procollagens, and in proteolytic trimming of other extracellular matrix (ECM) substrates. We report the phenotypic characteristics and natural history of four adults with severe, progressive OI characterized by numerous fractures, short stature with rhizomelic shortening and deformity of the limbs and variable kyphoscoliosis, in whom we identified novel bi-allelic missense and frameshift mutations in BMP1. We show that BMP1/mTLD-deficiency in humans not only results in delayed cleavage of the type I procollagen C-propeptide, but also hampers the processing of the small leucine-rich proteoglycan prodecorin, a regulator of collagen fibrillogenesis. Immunofluorescent staining of types I and V collagen and transmission electron microscopy of the dermis show impaired assembly of heterotypic type I/V collagen fibrils in the ECM. Our study thus highlights the severe and progressive nature of BMP1-associated OI in adults, and broadens insights into the functional consequences of BMP1/mTLD-deficiency on ECM organization. This article is protected by copyright. All rights reserved. This article is protected by copyright. All rights reserved.
- [Show abstract] [Hide abstract] ABSTRACT: Healthy aging in women is a physiological process modulated by special features particularly occurring during the perimenopausal period. The dermal extracellular matrix is involved in this process linked to hypoestrogenemia. In these instances, the skin presentation is occasionally indicative of some distinct internal failures. Measuring the in vivo mechanical functions of skin represents a convenient means for quantifying the perimenopausal skin changes. Such assessments have shown a beneficial effect of hormone replacement therapy on the skin status.
- [Show abstract] [Hide abstract] ABSTRACT: In recent years, the population and producers of consumer products became aware of deleterious effects of some substances on human health and environment. Cosmetic products are part of such concern. What are the risks currently involved? The so-called «natural», «bio» or «green» products, do they represent an ideal panacea ? This topic has a complex issue because documents available for the general public are of unequal quality, and objective scientifc publications remain rare and prone to controversies.
- [Show abstract] [Hide abstract] ABSTRACT: During perimenopause, the skin and a series of other organs become altered as prominent sex hormone declines. The natural climacteric is associated with a decline in functional properties of various tissues including viscoelasticity of the dermal connective tissue. There are many ways for assessing in vivo skin viscoelasticity, and the various procedures frequently measure distinct physical characteristics. The suction method has been selected by a majority of investigators looking at dermal functional changes during climacteric. Some women benefit from oral hormone replacement therapy (HRT) or transdermal estrogen therapy (TET) for controlling some unpleasant climacteric changes (osteoporosis, vasomotor instability, skin withering, dermal depletion in versican-hyaluronic acid, etc.). In particular, the hormonal repair exhibits some preventive and corrective effects in the functional decline of the dermal physical properties.
- [Show abstract] [Hide abstract] ABSTRACT: Cutaneous melanoma is the deadliest skin cancer showing an increasingly growing incidence in white populations of Europe and United States. Intensive research in recent years has begun to unlock its molecular pathogenesis. Screening the neoplasm at an early stage remains primordial. Hence, targeting populations at risk is likely efficient. In such an attempt, the regular clinical examination benefits from a series of non invasive procedures such as skin surface biopsies, in vivo confocal microscopy, dermoscopy and specular fluorescent light reflectance.
Article: Secreciones sudorípara y sebácea
- [Show abstract] [Hide abstract] ABSTRACT: The stratum corneum materializes the interface between the body and its environment. Such a structure is influenced by the climate and seasons. A series of physicochemical parameters are involved in this relationship. Among them, the relative humidity, the dew point, the insensible loss of water and the concepts of water-as-ice and the higgledy-piggledy-water.
- [Show abstract] [Hide abstract] ABSTRACT: The recognition of a complex disorder associating skin psoriasis and its comorbidities has considerably progressed over recent years. Beyond the skin lesions, psoriatic arthritis, the metabolic syndrome, type II diabetes, cardiovascular disease, inflammatory bowel diseases, and some cancers represent a group of chronic systemic disorders. The process is initiated and sustained by an immunological pathway involving Th1, Th17 and Th22 lymphocytes. The recognition of this complex disorder indicates that the clinical impact of psoriasis is not exclusively limited to the skin only.
- [Show abstract] [Hide abstract] ABSTRACT: The presentations of primary and metastatic cutaneous malignant melanoma (CMM) are very diverse. Evidence increasingly indicates that single CMM cells spread to distant sites quite early during cancer progression and are soon eliminated before they become clinically detectable. However bulky metastases which appear at a later stage might derive from some of these early neoplastic cells. It seems that local CMM single cell micro-metastases commonly predict sentinel lymph node involvement without overtly reflecting CMM progression to bulky visceral metastases. This study is intended to review the current understanding of the mechanisms underlying two CMM presentations. The first is the long interval, apparently disease-free, with persistent CMM dormancy, which may precede overt metastatic growth. Immunosurveillance may induce dormancy in single CMM cells disseminated in the body by blocking their proliferation cycle. The second is the so-called CMM smoldering phenomenon, which is marked by an alternate progression and regression of CMM locally with metastases that wax and wane for long periods of time over restricted skin areas. These very diverse patterns of CMM progression are likely to be ascribable to a number of biological factors, including the activation of CMM stem cells, and the combined phenotypic heterogeneity and variability in proliferative amplification in CMM cell clusters. Furthermore an adequate stimulation of CMM immune-surveillance and the induction of a specific stromal structure and vascular response are required. In this context, most early CMM tumors are in part controlled by lymphocyte-mediated responses before they become clinically detectable. However both the role of immune-surveillance and the mechanisms underlying both persistent and smoldering CMM dormancy remain unclear.