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1.
Int J Dermatol ; 2024 Jan 28.
Artigo em Inglês | MEDLINE | ID: mdl-38282244

RESUMO

BACKGROUND: While ultraviolet radiation (UVR) present in sunlight is recognized as the main etiological agent of skin cancer, the most frequent form of which is basal cell carcinoma (BCC), other exposome factors like pollution, diet, and lifestyle may also contribute. This study aimed to investigate the association of BCC and exposome-related factors in the Spanish population. METHODS: BCC cases (n = 119) and controls (n = 127) with no history of skin cancer were recruited between April 2020 and August 2022 by 13 dermatologists throughout Spain in this prospective multicenter case-control study. RESULTS: The BCC group had a higher proportion of outdoor workers, more years of UVR exposure, and a greater consumption of drugs (statins, ASA, hydrochlorothiazide, ACE inhibitors and omeprazole), P < 0.05. Avoidance of sun exposure was the most used photoprotection measure in both groups. The use of hats or caps was higher in the BCC group (P = 0.01). The solar protection factor (SPF) used 15 years previously was higher in the control group (P = 0.04). The control group had a higher daily screen time (P < 0.001), and practiced more relaxation activities (P = 0.03). Higher linolenic acid intake and lower coffee consumption were the only dietary variables associated with BCC (P < 0.05). Statistical significance for all the aforementioned variables was maintained in the multivariate analysis (P < 0.05). CONCLUSIONS: The study found a significant association between BCC and multiple exposome-related factors in addition to chronic sun exposure in the Spanish population. Primary prevention strategies should target specific populations, such as outdoor workers, promoting sun-safe behaviors and stress-reducing activities, and also adequate skin photoprotection in patients on certain medications associated with increased BCC risk.

2.
Skin Res Technol ; 29(12): e13493, 2023 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-38017667

RESUMO

BACKGROUND: Photodynamic therapy (PDT) is an effective treatment for actinic keratosis (AKs), but there is little information on how PDT affects skin barrier function. The objectives of this study are: To compare skin barrier function between skin with AKs and healthy skin and to evaluate the impact of PDT on skin homeostasis in patients with AKs. METHODS: A prospective observational study was conducted in patients with AKs to evaluate epidermal barrier function and skin homeostasis before and 1 ek after receiving PDT. RESULTS: A total of 21 subjects were included in the study, male/female ratio was 17:4, mean age was 75.86 years. The number of AKS observed before starting treatment was reduced with respect to those diagnosed 1 month after starting PDT (14.83 vs. 1.91, p < 0.0001). Application of PDT for treating AKs modifies epidermal barrier function. Immediately after the first session temperature, transepidermal water loss (TEWL), stratum corneum hydration (SCH) and total antioxidant capacity (TAC) increased while pH decreased on lesional skin. After 1-month follow-up, the only remained change was the increased in SCH. Higher increases in temperature were observed when using occlusive PDT compared to mixed modality. 5-ALA and M-ALA seem to have a similar impact on skin barrier. CONCLUSIONS: PDT can improve skin barrier function in patients with AKs. Skin homeostasis parameters can be used to assess efficacy and optimize dosing.


Assuntos
Ceratose Actínica , Fotoquimioterapia , Dermatoses do Couro Cabeludo , Idoso , Feminino , Humanos , Masculino , Ácido Aminolevulínico/uso terapêutico , Ácido Aminolevulínico/efeitos adversos , Ceratose Actínica/tratamento farmacológico , Fotoquimioterapia/efeitos adversos , Fármacos Fotossensibilizantes , Resultado do Tratamento , Estudos Prospectivos
3.
Cancers (Basel) ; 15(22)2023 Nov 12.
Artigo em Inglês | MEDLINE | ID: mdl-38001636

RESUMO

INTRODUCTION: The concept of exposome refers to the total of harmful and beneficial environmental exposures that can help predict the organism's biological responses over time. Ultraviolet radiation (UVR) from sun exposure has been recognized as the main etiological agent of skin cancer, and squamous cell carcinoma (SCC) is one most commonly associated with chronic exposure. However, in recent years, evidence suggests that lifestyle, environmental pollution, and contaminants in water and food can have an influence. OBJECTIVES: To study the relationship between SCC and sun exposure, pollution, stress, and lifestyle in a Spanish cohort. MATERIALS AND METHOD: A multicenter case-control study was carried out in which 13 dermatologists from different regions of Spain recruited cases and controls between April 2020 and August 2022. The group of cases were patients diagnosed with SCC and, as a control group, people who attended Dermatology consultations as companions with no history of skin cancer. RESULTS: A total of 62 patients with SCC and 126 controls were included (62.9% males, median age 76.46 (10.1) and 33.3%, median age 55.7 (15), respectively). The SCC group had experienced more outside work than the controls (75% vs. 22.4%, p < 0.001), less recreational exposure (sunbathing, p = 0.05, and outdoor sports, p = 0.01), and a lower annual income (p = 0.01), with an increase in tobacco exposure (p < 0.001), without differences in other carcinogens, such as ionizing radiation or chemical exposure. The control group had a higher daily screentime use (p < 0.001) and practiced more relaxation activities (p = 0.03). A higher linolenic acid intake and lower coffee consumption were the only dietary variables associated with SCC (p < 0.05). Some chronic medications (anxiolytics, antidepressants, beta-blockers, statins, hydrochlorothiazide, ACE inhibitors, metformin, and omeprazole) were also statistically associated with SCC. Statistical significance for all aforementioned variables was maintained in the multivariate analysis (p < 0.05). CONCLUSIONS: The study found a significant association between SCC and multiple exposome-related factors in addition to chronic sun exposure in the Spanish population. Primary prevention strategies should target specific populations, such as outdoor workers promoting sun-safe behaviors and stress-reducing activities, in addition to adequate skin photoprotection in patients under certain medications associated with SCC.

4.
Photodermatol Photoimmunol Photomed ; 39(5): 457-465, 2023 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-37130164

RESUMO

BACKGROUND: While skin cancer awareness programs have significantly furthered public understanding about the harmful effects of the sun, there is a disparity between photoprotection knowledge and protection practices. OBJECTIVE: To compare sun exposure habits and photoprotection measures in patients diagnosed with basal cell carcinoma (BCC), squamous cell carcinoma (SCC), and melanoma versus controls. METHODS: Multicentre case-control observational study carried out by 13 Spanish dermatologists between April 2020 and August 2022. Patients diagnosed with BCC, SCC, or melanoma were considered cases. The control group consisted of individuals with no history of skin cancer. RESULTS: Of the 254 cases (56.2% female; mean age, 62.67 ± 15.65), 119 (31.2%) had BCC, 62 (16.27%) SCC, and 73 (19.1%) melanoma. The control group consisted of 127 (33.33%) individuals. Avoiding sun exposure between 12:00 and 16:00 was the most commonly used photoprotection measure (habitually/always: 63.1%), followed by the use of sunscreen (habitually/always: 58.9%). Patients with melanoma were less likely to use clothing and shade to avoid sun exposure (p < .05), whereas those with BCC and SCC reported greater use of head coverings (p = .01). BCC and SCC groups reported greater sun exposure 15 years prior, whereas controls reported greater use of sunscreen. However, at the time of this study all groups reported using SPF ≥ 21, and the majority SPF > 50. No differences were observed in photoprotection measures between people with and without a previous history of skin cancer. CONCLUSIONS: We describe differences in photoprotection measures and sun exposure patterns among patients diagnosed with different skin tumor types. Whether these differences may influence the type of tumor each developed will require further investigation.


Assuntos
Carcinoma Basocelular , Carcinoma de Células Escamosas , Melanoma , Neoplasias Cutâneas , Humanos , Feminino , Pessoa de Meia-Idade , Idoso , Masculino , Luz Solar/efeitos adversos , Protetores Solares/uso terapêutico , Estudos de Casos e Controles , Neoplasias Cutâneas/epidemiologia , Neoplasias Cutâneas/prevenção & controle , Carcinoma Basocelular/epidemiologia , Carcinoma Basocelular/prevenção & controle , Carcinoma de Células Escamosas/epidemiologia , Carcinoma de Células Escamosas/prevenção & controle , Melanoma/epidemiologia , Melanoma/prevenção & controle
5.
Artigo em Inglês | MEDLINE | ID: mdl-36950898

RESUMO

INTRODUCTION: There is still a need to develop a simple algorithm to identify patients likely to need complex Mohs micrographic surgery (MMS) and optimize MMS schedule. The main objectives of this study are to identify factors associated with a complex MMS and develop a predictor model of the number of stages needed in surgery and the need for a complex closure. MATERIALS AND METHODS: A nationwide prospective cohort study (REGESMOHS, the Spanish Mohs surgery registry) was conducted including all patients with a histological diagnosis of basal cell carcinoma (BCC). Factors related to three or more stages and a complex closure (that needing a flap and/or a graft) were explored and predictive models were constructed and validated to construct the REGESMOSH scale. RESULTS: A total of 5226 patients that underwent MMS were included in the REGESMOHS registry, with 4402 (84%) having a histological diagnosis of BCC. A total of 3689 (88.9%) surgeries only needed one or two stages and 460 (11.1%) required three or more stages. A model to predict the need for three or more stages included tumour dimension, immunosuppression, recurrence, location in risk areas, histological aggressiveness and previous surgery. Regarding the closure type, 1616 (38.8%) surgeries were closed using a non-complex closure technique and 2552 (61.2%) needed a complex closure. A model to predict the need for a complex closure included histological aggressiveness, evolution time, patient age, maximum tumour dimension and location. CONCLUSION: We present a model to predict MMS needing ≥3 stages and a complex closure based on epidemiological and clinical data validated in a large population (with real practice variability) including different centres that could be easily implemented in clinical practice. This model could be used to optimize surgery schedule and properly inform patients about the surgery duration.

6.
Acta Derm Venereol ; 103: adv00846, 2023 Jan 10.
Artigo em Inglês | MEDLINE | ID: mdl-36625209

RESUMO

Type D personality is characterized by social inhibition and negative affectivity. Poorer outcomes and worse quality of life have been linked to type D personality in patients with a variety of non-dermatological diseases. Despite increasing evidence of the importance of type D personality in skin diseases, there are no reviews on this subject. The aim of this review is to summarize the current evidence regarding type D personality and skin diseases. A systematic search was performed using Medline and Web of Science databases from inception to 11 October 2022. Studies addressing the presence of type D personality, its associated factors, its impact on the outcomes of the disease or the quality of life of the patients were included in the systematic review. A total of 20 studies, including 3,124 participants, met the eligibility criteria and were included in the review. Acne, hidradenitis suppurativa, psoriasis, melanoma, atopic dermatitis, chronic spontaneous urticaria and pruritic disorders were the main diseases assessed. Type D personality was more frequent among patients with skin diseases than among controls. Type D personality was found to be associated with poorer quality of life and higher rates of psychological comorbidities in patients with skin diseases. In conclusion, type D personality appears to be a marker of patients with increased risk of poorer quality of life and higher rates of psychological comorbidities. Screening for type D personality in specialized dermatology units might be beneficial to identify patients who are more psychologically vulnerable to the consequences of chronic skin diseases.


Assuntos
Hidradenite Supurativa , Psoríase , Personalidade Tipo D , Humanos , Qualidade de Vida/psicologia , Psoríase/psicologia , Transtornos do Humor/diagnóstico , Transtornos do Humor/epidemiologia
7.
Dermatology ; 239(2): 255-261, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-36470224

RESUMO

BACKGROUND: Surgery is an essential part of hidradenitis suppurativa (HS) treatment. Understanding and reducing surgical recurrence are crucial to obtaining the best results in patients' treatment. OBJECTIVE: The aim of the study was to characterize surgical recurrences in a cohort of patients with HS treated with wide excision and second-intention healing. METHODS: A prospective nested case-control study was conducted. A cohort of patients with HS treated with wide excision and second-intention healing was monitored for 68 weeks. The surgical procedure was classified as case (recurrence) or control (no recurrence). The type of recurrence was classified according to the elementary lesion in tunnel or abscess and inflammatory nodule (AN) recurrence. Sociodemographic and clinical data likely related to recurrence and the type of recurrence were evaluated. RESULTS: Sixty-three patients were included, receiving a total of 82 surgical procedures. The mean age of the patients was 36.18 years, and the surgical site presented a Hurley stage II severity in 79.26% (65/82) of the interventions. Tunnel recurrence was observed in 8.5% (7/82) and AN recurrence in 15.85% (13/82) of the interventions. Obesity was associated with a higher risk of recurrence, for both tunnel and AN recurrence. Hurley III at the surgical site, a history of pilonidal sinus, and higher International Hidradenitis Suppurativa Severity Score System (IHS4) after surgery and at week 68 increased the risk of tunnel recurrence. CONCLUSION: We propose classifying surgical recurrence based on the elemental type of lesion. Tunnel recurrence could originate in the depth of the surgical scar and could be associated with both surgical site factors and inflammatory load. AN recurrence could originate in the borders of the surgical scar and may particularly benefit from preoperative ultrasound.


Assuntos
Hidradenite Supurativa , Humanos , Adulto , Hidradenite Supurativa/complicações , Cicatriz , Estudos de Casos e Controles , Estudos Prospectivos , Índice de Gravidade de Doença
8.
Pediatr Dermatol ; 40(3): 537-539, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-36394113

RESUMO

We report the case of a neonate presenting with the clinical features of blueberry muffin syndrome caused by ganglioneuroblastoma, a rare variant of neuroblastoma. This syndrome may be the only visible manifestation of a neonatal tumor and highlights the importance of early recognition and initiation of therapy to reduce mortality.


Assuntos
Ganglioneuroblastoma , Recém-Nascido , Lactente , Humanos , Síndrome
9.
Life (Basel) ; 12(12)2022 Dec 12.
Artigo em Inglês | MEDLINE | ID: mdl-36556448

RESUMO

Transepidermal water-loss (TEWL), stratum-corneum hydration (SCH), erythema, elasticity, pH and melanin, are parameters of the epidermal barrier function and skin homeostasis that objectively indicate the integrity of the skin barrier. Sunscreens are necessary to protect people from skin cancer, but could modify the skin barrier function. Nevertheless, there are not many studies on their impact on skin homeostasis. The aim of this study is to evaluate the impact of sunscreens on the epidermal-barrier function and skin homeostasis of healthy individuals. A prospective observational study was designed. TEWL, SCH, erythema, elasticity, pH and melanin were measured on the cheek and volar region of the forearm, using non-invasive methods before and after applying sunscreen. Four different sunscreens were tested, one full-body sunscreen and three facial sunscreens. The study included 51 healthy volunteers, 72.5% (37/51) women, with a mean age of 41.63 years. After full-body sunscreen application, temperature increased by 0.68 °C (p < 0.001), pH by 0.16 units (p < 0.001), and elasticity by 0.22% (p = 0.039), while melanin decreased by 10.95 AU (p < 0.001), erythema by 28.79 AU (p < 0.001) and TEWL by 0.66 g·m−2·h−1 (p = 0.019). On the cheek, facial sunscreen 1 increased temperature by 0.51 °C, TEWL 0.7 g·m−2·h−1 (p < 0.05), pH by 0.12 units (p < 0.001) and elasticity by 0.059% (p < 0.001), but decreased erythema by 19.87 AU (p < 0.05) and SCH by 5.63 AU (p < 0.001). Facial sunscreen 2 increased temperature by 0.67 °C, TEWL by 1.93 g·m−2·h−1 (p < 0.001), pH by 0.42 units (p < 0.001) and elasticity by 0.12% (p < 0.01), but decreased melanin by 15.2 AU (p = 0.000), erythema by 38.61 AU (p < 0.05) and SCH by 10.80 AU (p < 0.01). Facial sunscreen 3 increased temperature by 1.15 °C, TEWL by 2.29 g·m−2·h−1 (p < 0.001), pH by 0.46 units (p < 0.001) and elasticity by 0.15% (p < 0.01), but decreased erythema by 35.7 (p < 0.05) and SCH by 10.80 AU (p < 0.01). In conclusion, sunscreen could slightly modify the skin-barrier function. All of them decreased erythema, likely in relation to anti-inflammatory power.

10.
Artigo em Inglês | MEDLINE | ID: mdl-36011808

RESUMO

People who practice outdoor sports have an increased risk of skin cancer as they are exposed to high doses of ultraviolet (UV) radiation. Recent studies have shown that in many athletes, sun protection behaviours are inadequate, with the risk that this entails. The aim of this review is to collect the information published to date about the knowledge, attitudes and habits of athletes in relation to sun exposure and its risks. A systematic review was conducted using PubMed and Embase with the search algorithm "(skin cancer OR melanoma) AND (exercise OR sport OR athletes)". All studies analysing the knowledge, attitudes and habits of photoprotection in athletes were included. A total of 2,365 publications were found, of which 23 were selected, including a total of 10,445 sportspeople. The majority of participants declared their voluntary intention to tan and stated that the sun made them feel better, although they also showed concern about possible damage associated with UV radiation. In most studies, less than half of the participants made adequate use of photoprotective measures. In general, most athletes had a high level of knowledge regarding the risk of skin cancer associated with sun exposure. In conclusion, most athletes are aware of the risks associated with UV radiation but do not make adequate use of photoprotective cream. New training programs on photoprotection could help improve athletes' photoprotective behaviour, reducing the incidence of skin cancer and precancerous lesions in this population.


Assuntos
Melanoma , Neoplasias Cutâneas , Queimadura Solar , Conhecimentos, Atitudes e Prática em Saúde , Humanos , Melanoma/epidemiologia , Melanoma/etiologia , Melanoma/prevenção & controle , Neoplasias Cutâneas/epidemiologia , Neoplasias Cutâneas/etiologia , Neoplasias Cutâneas/prevenção & controle , Queimadura Solar/prevenção & controle , Luz Solar/efeitos adversos , Protetores Solares/uso terapêutico , Raios Ultravioleta/efeitos adversos
11.
J Clin Med ; 11(14)2022 Jul 19.
Artigo em Inglês | MEDLINE | ID: mdl-35887944

RESUMO

Background: Early detection of melanoma is one of the main diagnostic goals of dermatologists worldwide, due to the increasing incidence of the disease in our environment. However, the irruption of the SARS-CoV-2 pandemic has posed a challenge to global healthcare, forcing systems to focus their resources on the fight against COVID-19. Methods: Retrospective cohort study. The exposed cohort were patients diagnosed with melanoma in the year after the general confinement in Spain (15 March 2020) and the unexposed cohort were patients with melanoma diagnosed in the previous year. Results: 130 patients were included. No differences were observed between demographic characteristics in both cohorts. The mean Breslow of melanoma before the onset of the pandemic was 1.08, increasing to 2.65 in the year after the onset of the pandemic (p < 0.001). On the other hand, the percentage of melanomas in situ decreased from 38.96% to 16.98% in the year after the declaration of the state of alarm in Spain. Conclusions: The SARS-CoV-2 outbreak has led to a reduction in the early diagnosis of melanoma, with an increase in invasive melanomas with poor prognosis histological factors. This could lead to an increase in melanoma-related mortality in the coming years in our environment.

13.
Acta Derm Venereol ; 102: adv00757, 2022 Aug 11.
Artigo em Inglês | MEDLINE | ID: mdl-35604235

RESUMO

Patients with frontal fibrosing alopecia report higher rates of sunscreen use than control subjects. However, it is not known whether the higher use of sunscreens is a cause or a consequence of the alopecia. A greater use of sunscreens should be associated with a lower incidence of signs of actinic damage. The aim of this study is to assess the presence of actinic damage in patients with frontal fibrosing alopecia. A cross-sectional study was carried out on 101 patients with frontal fibrosing alopecia and 40 control subjects. The presence of actinic damage, in the form of solar lentigines, actinic keratoses, and basal and squamous cell carcinomas, was recorded in both groups, together with sunscreen use. Trichoscopy and skin biopsy were performed on patients. Actinic damage was present more frequently in patients with frontal fibrosing alopecia (69.3%) than in control subjects (50%) (p = 0.031). Patients used sunscreens more frequently than did control subjects (83.2% vs 62.5%, p = 0.008). However, the prevalence of trichoscopic inflammatory signs, peripheral alopecia, and inflammatory infiltrate and sebaceous gland involvement in skin biopsy, were similar in patients who used sunscreens and those who did not use them. In conclusion, patients with frontal fibrosing alopecia had greater actinic damage than did control subjects, and this is hypothesized as a reason for the higher use of sunscreens among patients. Thus, use of sunscreens may not be the trigger for frontal fibrosing alopecia that dermatologists have proposed.


Assuntos
Líquen Plano , Protetores Solares , Alopecia/induzido quimicamente , Alopecia/diagnóstico , Alopecia/epidemiologia , Estudos Transversais , Humanos , Líquen Plano/induzido quimicamente , Pele/patologia , Protetores Solares/efeitos adversos
14.
Nutrients ; 14(3)2022 Jan 27.
Artigo em Inglês | MEDLINE | ID: mdl-35276909

RESUMO

There is scarce scientific information regarding the potential benefits of healthy lifestyles in patients with hidradenitis suppurativa (HS). The objective of this study is to explore the potential association between the adherence to a Mediterranean diet (MD), physical activity and HS severity. A cross-sectional study that included patients with HS was conducted. Disease severity was evaluated by the International Hidradenitis Suppurativa Severity Score System (IHS4) and self-reported disease activity using a Numeric Rating Scale (NRS, 0−10). The adherence to a MD was assessed by the PREvención con DIeta MEDiterránea (PREDIMED) questionnaire and the level of physical activity by the International Physical Activity questionnaire. A total of 221 patients with HS were included in our study. The adherence to a MD was average for a Spanish population. A higher adherence to a MD was associated with lower disease activity, lower self-reported Hurley and lower IHS4. The use of extra virgin olive oil as the main culinary lipid was the dietary habit that implied a lower degree of disease activity (p < 0.05). Regarding physical activity, both the self-reported severity and IHS4 presented an inverse association with the intensity of physical activity. The adherence to a MD and the intensity of physical activity were positively associated. The Mediterranean dietary pattern may have an impact on HS. Greater adherence to a MD is related to lower HS severity and more physical activity also correlates to lower disease severity. The MD could be an appropriate dietary pattern for patients with HS due to its anti-inflammatory properties, and combining this with increased levels of physical activity could have additional benefits.


Assuntos
Dieta Mediterrânea , Hidradenite Supurativa , Estudos Transversais , Exercício Físico , Comportamento Alimentar , Humanos
16.
J Clin Med ; 10(22)2021 Nov 09.
Artigo em Inglês | MEDLINE | ID: mdl-34830503

RESUMO

Hidradenitis suppurativa (HS) is a chronic inflammatory skin disease that has been associated with a greater risk of metabolic and cardiovascular comorbidities. The aim of this study is to assess cardiovascular risk by means of intima-media thickness (IMT), metabolic syndrome, and other potential biomarkers in patients with severe hidradenitis suppurativa who are candidates for biologic therapy and to explore potentially associated factors. A cross-sectional study was performed. Body mass index (BMI), carotid intima-media thickness (IMT), and blood tests, including glycemic and lipid profile, insulin, vitamin D, and inflammation markers were performed. Fifty patients were included in the study; the male/female ratio was 3:2. The mean age was 38 years, and the mean disease duration was 21.8 years. The mean carotid IMT was 651.39 µm. A positive association of IMT with disease duration, tobacco consumption, and HbA1c levels was observed. HbA1c correlated with the age of onset, hypertension, metabolic syndrome, and glucose levels. Vitamin D levels inversely correlated with the number of areas affected. In conclusion, patients with severe HS present a higher cardiovascular risk, but it is not distributed equally within the patients: Tobacco consumption, inadequate glycemic control, and disease duration could be useful clinical and biochemical markers to identify patients at higher risk.

17.
J Clin Med ; 10(19)2021 Oct 08.
Artigo em Inglês | MEDLINE | ID: mdl-34640625

RESUMO

Genetic variants that result in truncation in desmoplakin (DSP) are a known cause of arrhythmogenic cardiomyopathy (AC). In homozygous carriers, the combined involvement of skin and heart muscle is well defined, however, this is not the case in heterozygous carriers. The aim of this work is to describe cutaneous findings and analyze the molecular and ultrastructural cutaneous changes in this group of patients. Four women and eight men with a mean age of 48 ± 14 years were included. Eight met definitive criteria for AC, one was borderline and three were silent carriers. No relevant macroscopic changes in skin and hair were detected. However, significantly lower skin temperature (29.56 vs. 30.97 °C, p = 0.036) and higher transepidermal water loss (TEWL) (37.62 vs. 23.95 g m 2 h 1, p = 0.028) were observed compared to sex- and age-matched controls. Histopathology of the skin biopsy showed widening of intercellular spaces and acantholysis of keratinocytes in the spinous layer. Immunohistochemistry showed a strongly reduced expression of DSP in all samples. Trichogram showed regular nodules (thickening) compatible with pseudomonilethrix. Therefore, regardless of cardiac involvement, heterozygous patients with truncation-type variants in DSP have lower skin temperature and higher TEWL, constant microscopic skin involvement with specific patterns and pseudomonilethrix in the trichogram.

18.
Dermatol Ther ; 34(3): e14942, 2021 05.
Artigo em Inglês | MEDLINE | ID: mdl-33719170

RESUMO

The term clinically amyopathic dermatomyositis (CADM) is used to represent a subgroup of patients with the typical cutaneous features of dermatomyositis (DM) in the absence of muscle involvement. Similar to classic DM, CADM can be associated with other connective tissue disorders and systemic manifestations such as interstitial lung disease and malignancy. Owing to the frequent discordance between muscle response and skin disease, the therapeutic approach to CADM represents a challenge. The current literature suggests that CADM treatment should follow a specific protocol, influenced by visceral involvement and the expression of certain myositis-specific antibodies, and different from the recommendation in the presence of myositis. Here, we present five new cases of CADM. We describe the available therapeutic options for skin manifestations in this type of DM, and we propose a step-by-step therapeutic scheme, using the cutaneous dermatomyositis disease area and severity index to assess response. Our literature review establishes mycophenolate mofetil and intravenous immunoglobulin as the most frequently successful therapies in refractory skin disease.


Assuntos
Dermatomiosite , Doenças Pulmonares Intersticiais , Neoplasias , Autoanticorpos , Dermatomiosite/diagnóstico , Dermatomiosite/tratamento farmacológico , Humanos , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/tratamento farmacológico , Pele
20.
Acta Derm Venereol ; 100(10): adv00150, 2020 May 28.
Artigo em Inglês | MEDLINE | ID: mdl-32374410

RESUMO

The aim of this study was to determine the prevalence and type of cutaneous manifestations which occur in patients with granulomatosis with polyangiitis and to explore the potential association between cutaneous and systemic involvement in these patients. A retrospective case series study was designed, including all granulomatosis with polyangiitis cases diagnosed between 2010 and 2018 at the Hospital Universitario Virgen de las Nieves. Thirty-nine patients with granulomatosis with polyangiitis were identified, of which 53.85% presented cutaneous manifestations. In decreasing order of frequency, the types of cutaneous problems observed included: palpable purpura, mucocutaneous ulcers, subcutaneous nodules, pyoderma gangrenosum-like ulcers, digital necrosis, papulonecrotic lesions and livedo reticularis. Patients with palpable purpura presented a higher frequency of renal involvement (p = 0.008). Cutaneous manifestations of granulomatosis with polyangiitis may facilitate early disease diagnosis. Likewise, a manifestation such as palpable purpura may be a predictor of kidney damage.


Assuntos
Granulomatose com Poliangiite/epidemiologia , Granulomatose com Poliangiite/patologia , Dermatopatias/epidemiologia , Dermatopatias/patologia , Pele/patologia , Adolescente , Adulto , Idoso , Anti-Infecciosos/uso terapêutico , Biópsia , Diagnóstico Precoce , Feminino , Granulomatose com Poliangiite/tratamento farmacológico , Humanos , Imunossupressores/uso terapêutico , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Prevalência , Prognóstico , Estudos Retrospectivos , Pele/efeitos dos fármacos , Dermatopatias/tratamento farmacológico , Espanha/epidemiologia , Adulto Jovem
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