Extent Of Skin Research Articles

Identification of a Distinct Monocyte-Driven Signature in Systemic Sclerosis Using Biophysical Phenotyping of Circulating Immune Cells.

Pathologically activated circulating immune cells, including monocytes, play major roles in systemic sclerosis (SSc). Their functional characterization can provide crucial information with direct clinical relevance. However, tools for the evaluation of pathologic immune cell activation and, in general, of clinical outcomes in SSc are scarce. Biophysical phenotyping (including characterization of cell mechanics and morphology) provides access to a novel, mostly unexplored layer of information regarding pathophysiologic immune cell activation. We hypothesized that the biophysical phenotyping of circulating immune cells, reflecting their pathologic activation, can be used as a clinical tool for the evaluation and risk stratification of patients with SSc. We performed biophysical phenotyping of circulating immune cells by real-time fluorescence and deformability cytometry (RT-FDC) in 63 SSc patients, 59 rheumatoid arthritis (RA) patients, 28 antineutrophil cytoplasmic antibody-associated vasculitis (AAV) patients, and 22 age- and sex-matched healthy donors. We identified a specific signature of biophysical properties of circulating immune cells in SSc patients that was mainly driven by monocytes. Since it is absent in RA and AAV, this signature reflects an SSc-specific monocyte activation rather than general inflammation. The biophysical properties of monocytes indicate current disease activity, the extent of skin or lung fibrosis, and the severity of manifestations of microvascular damage, as well as the risk of disease progression in SSc patients. Changes in the biophysical properties of circulating immune cells reflect their pathologic activation in SSc patients and are associated with clinical outcomes. As a high-throughput approach that requires minimal preparations, RT-FDC-based biophysical phenotyping of monocytes can serve as a tool for the evaluation and risk stratification of patients with SSc.

Assessment of Immune Cell Activation in Pemphigus.

(1) Background: Pemphigus is a blistering autoimmune disease of the skin and/or mucous membranes, characterised by the presence of specific autoantibodies directed against structural proteins of the human skin. Recent reports indicate that new haematological parameters, termed Extended Inflammation Parameters (EIP), can be used to assess the activation of immune cells during active inflammation. These include parameters assessing both neutrophil activation (NEUT-RI, NEUT-GI) and the number of activated lymphocytes (RE-LYMP). The aim of this study was to investigate the relationship between changes in NEUT-RI, NEUT-GI and RE-LYMP and the disease activity in patients with pemphigus. (2) Results: The study involved 32 patients with diagnosed different types of pemphigus. Neutrophil activation parameters (NEUT-RI and NEUT-GI) and lymphocytes (RE-LYMP) were significantly higher in these patients compared to the parameters in healthy participants (respectively p = 0.0127, p = 0.0011 and p = 0.0033). The increased quantity of activated lymphocytes (RE-LYMP) also correlated significantly with the extent of skin and/or mucosal lesions in patients assessed by the PDAI scale (p < 0.02). (3) Conclusions: The NEUT-RI, NEUT-GI and RE-LYMP parameters proved to be appropriate markers of inflammation severity in pemphigus, also in relation to local lesions, which was not possible with the inflammation markers (CRP, ESR) used so far on a routine basis.

Genetic and clinical characterization of 73 Pigmentary Mosaicism patients: revealing the genetic basis of clinical manifestations

BackgroundPigmentary mosaicism constitutes a heterogeneous group of skin pigmentation alterations associated with multisystem involvement. The aim of this study was to establish a complete cytogenetic and molecular characterization of PM patients, emphasizing on searching for possible low chromosomal mosaicism and on establishing an accurate genotype-phenotype correlation.ResultsA total of 73 patients were included (3 months to 18 years of age), 52% male and 48% female. Observed in 69 (95%) patients, the most frequent pattern of pigmentation was fine and whorled BL, which was associated with disseminated skin extent in 41 (59%) patients. Central nervous system (84%) alterations were the most frequent observed in the group of patients, followed by the musculoskeletal (53%) and ophthalmologic (27%) alterations. Considering the pattern of pigmentation, no significant differences in association with skin extent or extracutaneous manifestations were detected. Following a strict cytogenetic analysis strategy, screening metaphases from three different tissues (peripheral blood, hyperpigmented and hypopigmented skin) we found that 23/73 patients had chromosomal abnormalities classified as follows: 1) Mosaic with 2 or more different cell lines with structural alterations n = 19; 2) Polyploidy (mosaic) n = 1 and 3) Alterations in all cells in three different tissues n = 3. SNP array, array CGH and FISH were useful for the complete characterization of the chromosomal aberrations, for the detection of microdeletions in patients with normal karyotype but with strong clinical suspicious of chromosomal alteration, and for a better establishment of genotype-phenotype correlation. In 2 patients we found genes associated with some of the extracutaneous manifestations (SHH, MNX1, PPP2R2C).ConclusionsThis group of 73 patients finely described is the largest series of patients with pigmentary mosaicism reported worldwide. As we showed in this study, the followed analysis strategy allowed the detection of cytogenetic and molecular abnormalities, and made possible the establishment of genotype-phenotype associations in some patients. An important limitation of our study was the analysis of fibroblasts cultures instead of melanocytes and keratinocytes. In some cases the direct molecular DNA analysis of skin biopsy could be another choice.

Volumetric Cone Beam Computed Tomography for the Assessment of Oral Manifestations in Systemic Sclerosis: Data from an EUSTAR Cohort.

Background: Oral health issues are commonly reported in systemic sclerosis (SSc), comprising a broad spectrum of manifestations, e.g., reduced mouth opening, periodontal disease, increased periodontal ligament (PDL) space width, and mandibular resorption. We aimed to assess oral radiographic abnormalities, particularly PDL space widening and erosions, and to identify potential relations with disease measures. Methods: cross-sectional study in 43 SSc and matching controls receiving systematic oral assessments (full mouth dental/periodontal) and imaging (radiographs and cone beam computed tomography (CBCT)). Associations between disease variables and radiologic findings were investigated by univariate and multivariate analysis (SPSS-v.20, p < 0.05). Results: CBCT demonstrated generalized PDL space widening in up to half SSc, with at least one tooth involved, essentially in the posterior region (p < 0.05). Significant correlations between number of teeth with PDL space widening and disease severity, skin score, disease subset, topoisomerase I specificity, age, and disease duration were reported (p < 0.05). Additionally, mandibular erosions were described in one out of four patients, commonly condylar erosions. Conclusions: Tridimensional CBCT approach confirmed widening of PDL and mandibular erosions as common dental findings in scleroderma. Furthermore, widened PDL spaces correlated with several disease characteristics including severity, skin extent, and antibody profile.

Quality of Life in Children With Sturge-Weber Syndrome

AimWe assessed the utilization of the National Institutes of Health Quality of Life in Neurological Disorders (Neuro-QoL) in pediatric patients with Sturge-Weber syndrome, a rare neurovascular disorder which frequently results in seizures, brain atrophy, calcification, and a range of neurological impairments. MethodsSubjects were seen clinically and consented for research. All 22 patients filled out the Pediatric Neuro-QoL. The Neuro-QoL subscores were converted to T-scores to compare with the referenced control population. Twenty-one participants also filled out the Brain Vascular Malformation Consortium Database Questionnaire containing data pertaining to Sturge-Weber syndrome–related medical history, medications, comorbidities, and family history. All data were analyzed with a significance threshold of P < 0.05. ResultsCognitive function quality of life was significantly lower (P < 0.001) in pediatric patients with Sturge-Weber syndrome compared with referenced control subjects. Male gender (P = 0.02) was associated with lower cognitive function Neuro-QoL. The extent of skin (R = −0.46, P = 0.04), total eyelid port-wine birthmark (R = −0.56, P = 0.007), eye (R = −0.58, P = 0.005), and total Sturge-Weber syndrome involvement (R = −0.63, P = 0.002) were negatively correlated with cognitive function Neuro-QoL. A younger age at seizure onset was associated with lower cognitive function Neuro-QoL (hazard ratio = 0.90, P = 0.004) even after controlling for extent of brain, skin, or eye involvement. Antidepressant use was associated with lower cognitive function Neuro-QoL (P = 0.005), and cognitive function Neuro-QoL was negatively correlated with depression Neuro-QoL; however, after adjusting for depression this relationship was no longer significant. ConclusionsThe results suggest targeting cognitive function Neuro-QoL in treatment trials and reiterate the prognostic value of early seizure onset. In addition, sex-related differences were noted, which should be further studied.

Physical and Family History Variables Associated With Neurological and Cognitive Development in Sturge-Weber Syndrome

BackgroundSturge-Weber syndrome (SWS) is caused by a somatic mutation in GNAQ leading to capillary venous malformations in the brain presenting with various neurological, ophthalmic, and cognitive symptoms of variable severity. This clinical variability makes accurate prognosis difficult. We hypothesized that the greater extent of physical factors (extent of skin, eye, and brain involvement), presence of possible genetic factors (gender and family history), and age of seizure onset may be associated with greater symptom severity and need for surgery in patients with SWS. MethodsThe questionnaire was collected from 277 participants (age: two months to 66 years) with SWS brain involvement at seven US sites. ResultsBilateral brain involvement was associated with both learning disorder and intellectual disability, whereas port-wine birthmark extent was associated with epilepsy and an increased likelihood of glaucoma surgery. Subjects with family history of vascular birthmarks were also more likely to report symptomatic strokes, and family history of seizures was associated with earlier seizure onset. Learning disorder, intellectual disability, strokelike episodes, symptomatic stroke, hemiparesis, visual field deficit, and brain surgery were all significantly associated with earlier onset of seizures. ConclusionThe extent of brain and skin involvement in SWS, as well as the age of seizure onset, affect prognosis. Other genetic factors, particularly variants involved in vascular development and epilepsy, may also contribute to neurological prognosis, and further study is needed.

Nail manifestations in Hong Kong Chinese patients with psoriatic arthropathy (PsA)

Abstract Background Nail psoriasis is accepted as a common feature in psoriatic arthropathy (PsA) and is one of the diagnostic criteria for PsA. Several nail characteristics are allegedly associated with joint damage, however, information concerning their prevalence and features is extremely lacking, particularly in Hong Kong. Objective The primary objective of the study was to investigate the frequency of psoriatic nail disease and to understand their patterns. The secondary objective was to explore the associated factor for nail dystrophy among them. Methods This study was a cross-sectional observation study. The eligible PsA patients were recruited from Rheumatology Clinic of Tseung Kwan O Hospital. Their demographics and clinical characteristics were collected, their respective nail psoriasis were scored and the nail features were recorded. By comparing the clinical variables between PsA patients, with and without nail involvement, the associated factor was explored. Results A total of 106 PsA patients were recruited and 61.3% (65/106) of them had nail psoriasis. Among all the patients with nail involvement, 72.3% of them had pitting, 50.8% had onycholysis, 15.7 had crumbling, 6.9% had leukonychia and 3.9% suffered from nail-bed hyperkeratosis, with mean modified Nail Psoriasis Severity Index of 9.5 +/-15.3. Nail involvement was more common in those with severe skin extent, but neither related to psoriatic disease duration, arthritis subclasses, nor inflammatory markers. Conclusion A significant proportion of PsA patients had nail involvement and there were various nail features noted. The occurrence of psoriatic nail disease was found to be associated with severe skin problem. This common manifestation of PsA should not be overlooked in the rheumatologists’ daily practice, in view of its common prevalence.

Intestinal dysbiosis is common in systemic sclerosis and associated with gastrointestinal and extraintestinal features of disease.

BackgroundRecent evidence suggests a link between autoimmunity and the intestinal microbial composition in several rheumatic diseases including systemic sclerosis (SSc). The objective of this study was to investigate the prevalence of intestinal dysbiosis in SSc and to characterise patients suffering from this potentially immunomodulatory deviation.MethodsThis study consisted of 98 consecutive patients subject to in-hospital care. Stool samples were analysed for intestinal microbiota composition using a validated genome-based microbiota test (GA-map™ Dysbiosis Test, Genetic Analysis, Oslo, Norway). Gut microbiota dysbiosis was found present as per this standardised test. Patients were examined regarding gastrointestinal and extraintestinal manifestations of SSc by clinical, laboratory, and radiological measures including esophageal cineradiography, the Malnutrition Universal Screening Tool (MUST), levels of plasma transthyretin (a marker of malnutrition) and faecal (F-) calprotectin (a marker of intestinal inflammation).ResultsA majority (75.5%) of the patients exhibited dysbiosis. Dysbiosis was more severe (rs = 0.31, p = 0.001) and more common (p = 0.013) in patients with esophageal dysmotility. Dysbiosis was also more pronounced in patients with abnormal plasma levels of transthyretin (p = 0.045) or micronutrient deficiency (p = 0.009). In 19 patients at risk for malnutrition according to the MUST, 18 exhibited dysbiosis. Conversely, of the 24 patients with a negative dysbiosis test, only one was at risk for malnutrition. The mean ± SEM levels of F-calprotectin were 112 ± 14 and 45 ± 8 μg/g in patients with a positive and negative dysbiosis test, respectively. Dysbiosis was more severe in patients with skin telangiectasias (p = 0.020), pitting scars (p = 0.023), pulmonary fibrosis (p = 0.009), and elevated serum markers of inflammation (p < 0.001). However, dysbiosis did not correlate with age, disease duration, disease subtype, or extent of skin fibrosis.ConclusionsIn this cross-sectional study, intestinal dysbiosis was common in patients with SSc and was associated with gastrointestinal dysfunction, malnutrition and with some inflammatory, fibrotic and vascular extraintestinal features of SSc. Further studies are needed to elucidate the potential causal relationship of intestinal microbe-host interaction in this autoimmune disease.

Nucleosome in patients with systemic sclerosis: possible association with immunological abnormalities via abnormal activation of T and B cells.

To determine the serum levels of nucleosome in patients with systemic sclerosis (SSc) and relate the results to the clinical features of SSc. Serum nucleosome levels in 91 patients with SSc were examined by ELISA. The expression of Toll-like receptor (TLR) 9 in T and B cells was quantified by flow cytometric intracellular protein analysis. The effects of nucleosomes on lymphocytes were also analysed. Moreover, we assessed the effects of nucleosomes on fibrosis, using wild type and CD19-deficient bleomycin-treated mice, an experimental model for human SSc. Serum nucleosome levels were elevated in SSc compared with healthy controls and correlated positively with the extent of skin and pulmonary fibrosis and immunological abnormalities. The retrospective longitudinal analysis showed the serum nucleosome levels to be attenuated during the follow-up period. TLR9, which can be stimulated by nucleosome expression was upregulated in the affected T and B cells of patients with SSc. Moreover, nucleosome stimulation strongly increased interleukin (IL)-4 and IL-17 expression of T cells, B-cell IgG production and proliferation of lymphocytes in SSc compared with those in healthy controls. In bleomycin-induced SSc model mice, serum nucleosome levels were elevated compared with control mice. Furthermore, nucleosomes increased IgG production and proliferation of mouse B cells. Although TLR9 expression was similar between wild type and CD19-deficient splenic B cells, CD19 deficiency reduced these nucleosome effects. These results suggest that nucleosomes and its signalling in B and T cells contribute to disease development in SSc via TLR9.

Diagnostic précoce du syndrome APECED : un défi pour le dermatologue

Polyglandular auto-immune syndrome type 1 (PAS-1) or auto-immune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) is a rare autosomal recessive disorder linked to auto-immune regulator (AIRE) gene mutations. Herein, we report the case of a 3-year-old boy with APECED emphasizing the wide phenotypic variability and the extent of skin lesions. A 3-year-old boy with a history of auto-immune hepatitis was referred for a generalized pruriginous urticaria-like eruption present for one month. He was born to non-consanguineous parents. Cutaneous examination revealed twenty-nail dystrophy, which had been present since the age of 2 years. Both direct microscopy and culture of nail samples were negative for Candida albicans. Esophagogastroduodenoscopy revealed esophageal candidiasis. A diagnosis of APECED was suspected and subsequently confirmed by molecular analysis of the AIRE gene, which showed two mutations. No other auto-immune endocrinopathies were found. Our case report illustrates the phenotypic variability of APECED with the absence of typical manifestations such as Addison's disease and hypoparathyroidism. APECED should thus be systematically suspected in young children presenting with cutaneous lesions associated with mucocutaneous candidiasis or auto-immune disease, even in the absence of known endocrinopathies. Dermatologists should be aware of this association since early diagnosis of APECED is critical in preventing life-threatening endocrinological crises.

Multicentre consensus recommendations for skin care in inherited epidermolysis bullosa.

BackgroundInherited epidermolysis bullosa (EB) comprises a highly heterogeneous group of rare diseases characterized by fragility and blistering of skin and mucous membranes. Clinical features combined with immunofluorescence antigen mapping and/or electron microscopy examination of a skin biopsy allow to define the EB type and subtype. Molecular diagnosis is nowadays feasible in all EB subtypes and required for prenatal diagnosis. The extent of skin and mucosal lesions varies greatly depending on EB subtype and patient age. In the more severe EB subtypes lifelong generalized blistering, chronic ulcerations and scarring sequelae lead to multiorgan involvement, major morbidity and life-threatening complications. In the absence of a cure, patient management remains based on preventive measures, together with symptomatic treatment of cutaneous and extracutaneous manifestations and complications. The rarity and complexity of EB challenge its appropriate care. Thus, the aim of the present study has been to generate multicentre, multidisciplinary recommendations on global skin care addressed to physicians, nurses and other health professionals dealing with EB, both in centres of expertise and primary care setting.MethodsAlmost no controlled trials for EB treatment have been performed to date. For this reason, recommendations were prepared by a multidisciplinary team of experts from different European EB centres based on available literature and expert opinion. They have been subsequently revised by a panel of external experts, using an online-modified Delphi method to generate consensus.ResultsRecommendations are reported according to the age of the patients. The major topics treated comprise the multidisciplinary approach to EB patients, global skin care including wound care, management of itching and pain, and early diagnosis of squamous cell carcinoma. Aspects of therapeutic patient education, care of disease burden and continuity of care are also developed.ConclusionThe recommendations are expected to be useful for daily global care of EB patients, in particular in the community setting. An optimal management of patients is also a prerequisite to allow them to benefit from the specific molecular and cell-based treatments currently under development.

SAT0292 Facilitating a modular approach to the assessment of psoriatic arthritis

BackgroundPsoriatic Arthritis (PsA) is a complex condition involving both axial and peripheral joints, together with enthesitis, dactylitis and skin. A complete assessment of PsA is challenging within the short time...

Nailfold capillaroscopy by digital microscope in an Indian population with systemic sclerosis

Nailfold capillaroscopy (NFC) is a simple, non-invasive method with exceptional predictive value for the analysis of microvascular abnormalities, especially in systemic sclerosis (SSc) but remains underutilized due to cost factors of the nailfold videocapillaroscope, lack of expertise and availability issues. The aim of this study was to establish the utility of an inexpensive digital microscope to study NFC changes in SSc in correlation with disease subsets and extent of skin involvement. Twenty-two diffuse cutaneous SSc (DSS), 20 limited cutaneous SSc (LSS) patients and 42 controls were evaluated with NFC using a digital microscope at 30× and 100× magnification. Digital micrographs were used to study qualitative and quantitative changes in microvasculature. The capillary density was significantly less in all cases of SSc as compared to controls (5.3 ± 1.4 vs. 8.7 ± 1.2; P < 0.00001). Disorganized architecture was much more prevalent in DSS versus LSS (86.4%vs. 25%). The vascular deletion score (VDS) was significantly higher in DSS as compared to LSS (P < 0.0001). Scleroderma pattern (SP) was seen in 18 (81.9%) and 15 (75%) of patients with DSS and LSS, respectively. Only 4% of normal subjects showed non-specific pattern and none showed SP. The mean modified Rodnan skin score (MRSS) was positively correlated with vascular deletion score (r = 0.572; P < 0.001) and negatively with capillary density (r = -0.8; P < 0.001). Nailfold capillaroscopy changes in SSc are related to disease subset and MRSS. NFC with digital microscope is a simplified, inexpensive, outpatient procedure with results comparable to previous studies.

Assessment of osteoporosis in psoriasis with and without arthritis: correlation with disease severity

The most frequent extracutaneous association with psoriasis is arthritis. Because proinflammatory cytokines are increased in psoriasis, patients with this disease may be more prone to osteoporosis than the healthy individuals. We evaluated 50 patients with psoriasis, with or without psoriatic arthritis (PsA), for the presence and degree of osteoporosis by performing dual energy x-ray absorptiometry (DEXA) and obtaining serum osteoprotegrin (OPG) levels. In addition, we correlated these results with the extent of skin and joint disease. Psoriasis area and severity index (PASI) was determined in all 50 patients with psoriasis, and total joint score (TJS) was recorded in the 16 patients who also had PsA. Results of DEXA and serum OPG were also obtained for 20 healthy individuals who served as controls. Osteoprotegrin level was significantly increased in psoriasis patients (with or without PsA) vs. controls. However, DEXA revealed that PsA patients had a higher degree of osteoporosis in the femur neck and wrist. In PsA patients, TJS correlated positively with both disease duration and PASI but correlated negatively with Z score of the femur. Psoriasis patients with or without arthritis may suffer from osteoporosis as evidenced by significantly increased serum OPG. Prolonged and extensive cutaneous disease is an important risk factor for the development and severity of PsA. Patients with a greater number of affected joints are at higher risk of osteoporosis.

Elevated serum interleukin-27 levels in patients with systemic sclerosis: association with T cell, B cell and fibroblast activation

ObjectiveTo determine serum levels of interleukin 27 (IL-27) in patients with systemic sclerosis (SSc) and relate the results to the clinical features of SSc.MethodsSerum levels of IL-27 in 91 patients...

Estudio epidemiológico de 20 casos de pénfigo en el Hospital Clínico Universitario Virgen de la Victoria de Málaga

Introduction Pemphigus comprises a group of autoimmune blistering diseases that affect the skin and mucous membranes. Its clinical and epidemiologic features vary according to geographic location and ethnic background. Objective An exhaustive search of the literature reveals very few reports of the epidemiology of pemphigus in setting. Our aim, thus, was to conduct a retrospective study of the clinical and epidemiologic features of pemphigus at a secondary care hospital in Málaga, Spain. Material and methods We studied 20 patients diagnosed with pemphigus in our department over a period of 13 years (January 1995 to January 2008). Results We analyzed a large variety of clinical and epidemiologic parameters including sex; age; type of pemphigus; time since onset; associated symptoms; type, morphology, and location of lesions at the time of diagnosis; extent of skin and mucosal involvement; treatment received; treatment-related adverse effects and complications; number of hospital admissions; and patient outcome. Conclusions Except for minor differences, our results are in agreement with published data on pemphigus regarding sex, age, and clinical presentation. According to our results, male sex is a predictor of poor prognosis as it is associated with poorer response to treatment and a higher rate of adverse effects and hospital admission.

Impaired exercise performance in systemic sclerosis and its clinical correlations

Objectives: To investigate the prevalence of impaired exercise performance as assessed by a standardized cardiopulmonary exercise test (CPET) in systemic sclerosis (SSc) and to identify the associated disease features.Methods: Forty-six SSc patients were enrolled and evaluated for clinical and serological SSc subset, extent of skin and internal organ involvement, and disease activity and severity. Exercise performance was subsequently evaluated in these patients and in 23 healthy individuals matched for sex and age, using a standardized CPET.Results: Exercise performance, measured by maximum oxygen uptake ( max < 80% of predicted value), was found to be impaired in 43/46 patients. Stepwise regression analysis showed that max adjusted for body weight ( max/kg) was independently correlated with the severity of heart (p = 0.001) and lung (p = 0.013) involvement, left ventricular diastolic dysfunction (p = 0.009), and the Health Assessment Questionnaire Disability Index (HAQ-DI) score (p = 0.016).Conclusions: This study demonstrates that physical disability contributes significantly to the development of impaired exercise performance in SSc patients. Cardiopulmonary exercise testing may be included among the battery of tests used to determine the severity of SSc.

Epidemiologic Study of 20 Cases of Pemphigus at Hospital Clínico Universitario Virgen de la Victoria de Málaga, Spain

IntroductionPemphigus comprises a group of autoimmune blistering diseases that affect the skin and mucous membranes. Its clinical and epidemiologic features vary according to geographic location and ethnic background. ObjectiveAn exhaustive search of the literature reveals very few reports of the epidemiology of pemphigus in our setting. Our aim, thus, was to conduct a retrospective study of the clinical and epidemiologic features of pemphigus at a secondary care hospital in Malaga, Spain. Material and methodsWe studied 20 patients diagnosed with pemphigus in our department over a period of 13 years (January 1995 to January 2008). ResultsWe analyzed a large variety of clinical and epidemiologic parameters including sex; age; type of pemphigus; time since onset; associated symptoms; type, morphology, and location of lesions at the time of diagnosis; extent of skin and mucosal involvement; treatment received; treatment-related adverse effects and complications; number of hospital admissions; and patient outcome. ConclusionsExcept for minor differences, our results are in agreement with published data on pemphigus regarding sex, age, and clinical presentation. According to our results, male sex is a predictor of poor prognosis as it is associated with poorer response to treatment and a higher rate of adverse effects and hospital admission.

Influence of Transendothelial Mechanisms on Microcirculation: Consequences for Reperfusion Injury after Free Flap Transfer. Previous, Current, and Future Aspects

The success of a free microvascular tissue transfer is based on a sufficient microanastomosis which meets the following requirements: a pedicle placed without kinking or twisting, good drainage, a well-defined recipient vessel, integrity of the endothelium, and duration of ischemia. The extent of skin and muscle necrosis increases significantly with increases in ischemia time. Reperfusion of ischemic tissue results in local and systemic damage associated with the release of oxygen free radicals, polymorphonuclear leucocytes, and such endothelial hormones as endothelin-1, EDRF (endothelial-derived relaxing factor), thromboxane, complement, and cytokines. Ischemia-reperfusion disrupts the delicate balance that maintains homeostasis in the microcirculation. This review discusses the clinical and therapeutic aspects of such injury, concentrating on perioperative management in free flap transfer. It points out the possible influence of endothelin-1 on vasospasm at the site of anastomosis, and emphasizes the importance of the endothelium as a highly dynamic network. Finally, future diagnostic and therapeutical aspects are discussed.

An assessment of the quality of life in older patients with skin disease

There are few data regarding quality of life in older people with skin disease, particularly concerning those with skin cancer. Access to U.K. secondary care dermatological services is increasingly focusing on skin cancer, which may have a negative effect on waiting times for patients with rashes. To assess the quality of life in patients aged 65 years and over and to determine how this relates to type and extent of skin disease. One hundred consecutive new dermatology patient referrals aged 65 years and above had quality of life assessed by the Dermatology Life Quality Index (DLQI), the Hospital Anxiety and Depression Scale (HADS) and the Illness Perception Questionnaire. Patients were examined for type and extent of skin disease. There were 49 men (mean age 75.1 years) and 51 women (mean age 75.0 years). Fifty-five per cent of patients had been referred with one or more lesions (76% premalignant or malignant) and 45% had rashes. There was no significant difference between the quality of life scores of men vs. women regarding all three of the questionnaires. However, patients with rashes scored significantly higher (i.e. poorer quality of life) on all but one of the questionnaire parts compared with patients with lesions. Patients with more extensive disease had significantly higher DLQI scores. Older people suffering from rashes had significantly poorer quality of life than patients with lesions, even those with skin cancer. The quality of life is negatively associated with extent of skin disease. Access to specialist dermatological care should take into account the suffering caused by extensive skin diseases. DLQI and a general quality of life questionnaire such as the HADS are quick to deliver and to score, particularly in the setting of a busy outpatient department. They provide an excellent screening tool for psychological problems in older people with skin disease.