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1.
Pediatr Dermatol ; 41(3): 523-525, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-38273779

RESUMEN

We present the case of a 20-month-old girl with Schimmelpenning-Feuerstein-Mims (SFM) syndrome with extensive head, neck, and torso skin involvement successfully managed with topical trametinib. Trametinib interferes downstream of KRAS and HRAS in the MAPK signaling pathway, of which KRAS was implicated in our child's pathogenic variant. Although other dermatologic conditions have shown benefit from oral trametinib, its topical use has not been well reported. Our patient showed benefit from the use of twice-daily topical trametinib, applied to the epidermal and sebaceous nevi over a 16-month period, leading to decreased pruritus and thinning of the plaques.


Asunto(s)
Piridonas , Pirimidinonas , Neoplasias Cutáneas , Humanos , Piridonas/uso terapéutico , Piridonas/administración & dosificación , Femenino , Pirimidinonas/uso terapéutico , Pirimidinonas/administración & dosificación , Lactante , Neoplasias Cutáneas/tratamiento farmacológico , Neoplasias Cutáneas/patología , Nevo/tratamiento farmacológico , Insuficiencia de Crecimiento/tratamiento farmacológico , Administración Tópica , Anomalías Múltiples/tratamiento farmacológico , Nevo Sebáceo de Jadassohn/tratamiento farmacológico , Síndromes Neurocutáneos/tratamiento farmacológico , Síndromes Neurocutáneos/diagnóstico , Anomalías Cutáneas/tratamiento farmacológico , Antineoplásicos/uso terapéutico , Anomalías del Ojo/tratamiento farmacológico , Enfermedades de Inmunodeficiencia Primaria/tratamiento farmacológico
2.
São Paulo; s.n; 2023. 41 p.
Tesis en Portugués | Coleciona SUS, Sec. Munic. Saúde SP, HSPM-Producao, Sec. Munic. Saúde SP | ID: biblio-1531266

RESUMEN

O termo amiloidose nomeia um grupo de doenças cuja principal característica é o depósito extracelular de uma proteína modificada e insolúvel: a proteína amiloide. O depósito exclusivo na pele caracteriza a amiloidose cutânea localizada primária, que em geral, cursa com hiperpigmentação, alteração da textura da pele e prurido moderado a grave. Os tratamentos disponíveis atualmente têm como objetivo a redução do prurido, sintoma que mais incomoda e prejudica o cotidiano dos pacientes. A hialuronidase é uma enzima que degrada glicosaminoglicanas, alterando a permeabilidade da matriz extracelular e facilitando a difusão de líquidos. O presente estudo teve como objetivo avaliar a melhora do prurido, da dimensão e da pigmentação de cinco casos de amiloidose cutânea primária submetidos a múltiplas aplicações intradérmicas de hialuronidase. Através da análise retrospectiva dos prontuários e de registros fotográficos, foi possível observar a evolução dessas variáveis ao longo do tratamento. Todos apresentaram melhora de pelo menos uma variável e não houve relato de efeitos adversos. A hialuronidase pode ser considerada uma opção terapêutica efetiva para o tratamento da amiloidose cutânea devido à sua ação na difusão de líquidos e à resposta positiva obtida no tratamento de outras doenças de depósito. Palavras-chave: Amiloide. Amiloidose. Hiperpigmentação. Proteínas. Prurido.


Asunto(s)
Humanos , Masculino , Femenino , Persona de Mediana Edad , Anciano , Anciano de 80 o más Años , Piel/enzimología , Anomalías Cutáneas/tratamiento farmacológico
3.
Sci Rep ; 12(1): 11041, 2022 06 30.
Artículo en Inglés | MEDLINE | ID: mdl-35773320

RESUMEN

Skin wound healing requires accurate therapeutic topical managements to accelerate tissue regeneration. Here, for the first time, we found that the association mesoglycan/VEGF has a strong pro-healing activity. In detail, this combination induces angiogenesis in human endothelial cells promoting in turn fibroblasts recruitment. These ones acquire a notable ability to invade the matrigel coating and to secrete an active form of metalloproteinase 2 in presence of endothelial cells treated with mesoglycan/VEGF. Next, by creating intrascapular lesions on the back of C57Bl6 mice, we observed that the topical treatments with the mesoglycan/VEGF promotes the closure of wounds more than the single substances beside the control represented by a saline solution. As revealed by eosin/hematoxylin staining of mice skin biopsies, treatment with the combination mesoglycan/VEGF allows the formation of a well-structured matrix with a significant number of new vessels. Immunofluorescence analyses have revealed the presence of endothelial cells at the closed region of wounds, as evaluated by CD31, VE-cadherin and fibronectin staining and of activated fibroblasts assessed by vimentin, col1A and FAP1α. These results encourage defining the association mesoglycan/VEGF to activate endothelial and fibroblast cell components in skin wound healing promoting the creation of new vessels and the deposition of granulation tissue.


Asunto(s)
Células Endoteliales , Glicosaminoglicanos , Anomalías Cutáneas , Traumatismos de los Tejidos Blandos , Factor A de Crecimiento Endotelial Vascular , Animales , Movimiento Celular/efectos de los fármacos , Movimiento Celular/fisiología , Células Endoteliales/efectos de los fármacos , Células Endoteliales/fisiología , Fibroblastos/efectos de los fármacos , Fibroblastos/fisiología , Glicosaminoglicanos/farmacología , Metaloproteinasa 2 de la Matriz , Ratones , Ratones Endogámicos C57BL , Piel/efectos de los fármacos , Anomalías Cutáneas/tratamiento farmacológico , Traumatismos de los Tejidos Blandos/tratamiento farmacológico , Factor A de Crecimiento Endotelial Vascular/farmacología
4.
Br J Dermatol ; 186(5): 875-886, 2022 05.
Artículo en Inglés | MEDLINE | ID: mdl-34921679

RESUMEN

BACKGROUND: The skin of patients with atopic dermatitis is characterized by abnormal stratum corneum lipid levels. Consequently, the lamellar matrices are disrupted and skin barrier function is diminished, increasing skin sensitivity to irritants and allergens. OBJECTIVES: To determine whether a cream containing ceramides, triglycerides and cholesterol in a multivesicular emulsion can reinforce the skin barrier and protect against skin irritation. METHODS: A randomized observer-blind intrapatient-controlled study in 34 adults with dry, eczema-prone skin was conducted. Each participant underwent 4 weeks of treatment with the test cream on one forearm and lower leg and a reference emollient cream on the other. Skin properties were determined before and after treatment. Lipid structure was assessed by Fourier-transform infrared spectroscopy using a novel interface. RESULTS: Skin barrier integrity was greater at sites treated with the test cream [effect size for area under the transepidermal water loss curve -162, 95% confidence interval (CI) -206 to -118]. Skin sensitivity to sodium lauryl sulfate was reduced (-0·5 points visual redness, 97·57% CI -1·00 to -0·25), as was transepidermal water loss (-15·3 g m-2 h-1 , 95% CI -20·3 to -10·4) compared with the reference. Sites treated with the test cream displayed enhanced lipid chain ordering, which was significantly associated with skin barrier integrity (r = 0·61). Compared with the reference, treatment with the test cream increased hydration (8·61 capacitance units, 95% CI 6·61-10·6) and decreased signs of dryness. CONCLUSIONS: The test cream facilitates skin barrier restoration and protects the skin from dryness and irritation. Compared with a commonly prescribed emollient in the UK, the test cream is highly suited to the management of dry, sensitive skin.


Asunto(s)
Eccema , Anomalías Cutáneas , Adulto , Eccema/tratamiento farmacológico , Eccema/prevención & control , Emolientes/uso terapéutico , Humanos , Piel , Anomalías Cutáneas/tratamiento farmacológico , Dodecil Sulfato de Sodio/farmacología , Agua , Pérdida Insensible de Agua
5.
Biochim Biophys Acta Biomembr ; 1862(9): 183335, 2020 09 01.
Artículo en Inglés | MEDLINE | ID: mdl-32376224

RESUMEN

Terbinafine (Tbf) is a well-established anti-fungal agent used for management of a variety of dermal conditions including ringworm and athlete's foot. Both the biochemical mechanism of Tbf fungicidal action (based on squalene epoxidase inhibition) and the target region for Tbf in vivo (the stratum corneum (SC)) are well determined. However, the biochemical and pharmacokinetic approaches used to evaluate Tbf biochemistry provide no biophysical information about molecular level physical changes in the SC upon Tbf binding. Such information is necessary for improved drug and formulation design. IR spectroscopic methods were used to evaluate the effects of Tbf on keratin structure in environments commonly used in pharmaceutics to mimic those in vivo. The Amide I and II spectral regions (1500-1700 cm-1) provided an effective means to monitor keratin secondary structure changes, while a Tbf spectral feature near 775 cm-1 provides a measure of relative Tbf levels in skin. Interaction of Tbf with the SC induced substantial ß-sheet formation in the keratin, an effect which was partially reversed both by ethanol washing and by exposure to high relative humidity. The irreversibility suggests the presence of a Tbf reservoir (consistent with kinetic studies), permitting the drug to be released in a controlled manner into the surrounding tissue.


Asunto(s)
Queratinas/química , Anomalías Cutáneas/tratamiento farmacológico , Terbinafina/química , Terbinafina/farmacología , Proteínas Filagrina , Humanos , Proteínas de Filamentos Intermediarios/química , Queratinas/antagonistas & inhibidores , Queratinas/ultraestructura , Conformación Proteica en Lámina beta , Piel/efectos de los fármacos , Piel/microbiología , Anomalías Cutáneas/microbiología , Anomalías Cutáneas/patología , Escualeno-Monooxigenasa/antagonistas & inhibidores , Escualeno-Monooxigenasa/química , Terbinafina/farmacocinética , Tiña/tratamiento farmacológico , Tiña/microbiología , Tiña/patología , Tiña del Pie/tratamiento farmacológico , Tiña del Pie/microbiología , Tiña del Pie/patología
6.
Skin Res Technol ; 25(2): 158-164, 2019 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-30368923

RESUMEN

BACKGROUND/PURPOSE: Skin care via moisturization compensates for the lack of skin barrier function. However, moisturizer application methods are not clearly decided. Here, we focused on and examined the retention of externally applied ceramide in the stratum corneum (SC) using fluorescent imaging method. This study aimed to compare ceramide retention in the SC between normal skin and dry skin using an animal model. METHODS: Nine-week-old Sprague-Dawley rats were divided into two groups: normal skin and dry skin model. The dry skin model group was treated with acetone-diethyl ether solution. A fluorescently labeled ceramide solution was prepared and applied to rats' back skin. Skin samples were taken at 0 minute and 12 hours after ceramide application. Fluorescently labeled ceramide was evaluated and observed under a microscope. RESULTS: The intensity of externally applied ceramide in the normal skin group showed no significant change from 0 minute to 12 hours after application. In contrast, in the dry skin model group, the intensity of externally applied ceramide increased significantly from 0 minute to 12 hours after application. CONCLUSION: Our findings demonstrate that the externally applied ceramide penetrated the SC of dry skin more than that of normal skin.


Asunto(s)
Ceramidas/administración & dosificación , Epidermis/metabolismo , Piel/diagnóstico por imagen , Piel/metabolismo , Animales , Agua Corporal/efectos de los fármacos , Agua Corporal/fisiología , Ceramidas/farmacología , Epidermis/anatomía & histología , Epidermis/efectos de los fármacos , Epidermis/ultraestructura , Masculino , Microscopía Fluorescente/instrumentación , Modelos Animales , Ratas , Ratas Sprague-Dawley , Piel/efectos de los fármacos , Piel/ultraestructura , Anomalías Cutáneas/tratamiento farmacológico , Absorción Cutánea/efectos de los fármacos , Absorción Cutánea/fisiología , Fenómenos Fisiológicos de la Piel/efectos de los fármacos
7.
Biosci Rep ; 38(6)2018 12 21.
Artículo en Inglés | MEDLINE | ID: mdl-30341238

RESUMEN

It was previously reported that the expression of CD274 was down-regulated in psoriatic epidermis, leading to immune disorders of psoriasis. However, the regulatory mechanisms of CD274 were rarely elucidated. We aimed to explore the regulatory mechanisms of CD274. Skin samples were collected from 18 patients with psoriasis vulgaris and 9 healthy participants for RNA sequencing. Candidate genes were chosen based on degree and k-core difference of genes in the co-expression network. The relations between candidate genes and CD274 were validated by flow cytometry and real-time PCR in primary human epidermal keratinocytes. The therapeutic effect of indirubin was assessed in an imiquimod-treated mouse model. Interferon-γ (IFN-γ), cyclin-dependent kinase (CDK) 1, Toll-like receptor 3 (TLR3), TLR4 and interleukin (IL)-17A were considered as candidate genes. In primary human epidermal keratinocytes, the level of CD274 was obviously increased under the stimulation of IFN-γ and CDK1 inhibitor (indirubin), independent of TLR4, TLR3 or IL-17A. Indirubin alleviated the severity of psoriatic mice in a CD274-dependent manner. Co-expression network analysis served as an effective method for the exploration of molecular mechanisms. We demonstrated for the first time that CD274 was the regulator of indirubin-mediated effect on mouse psoriasis-like skin lesion based on co-expression network analysis, contributing to the alleviation of mouse psoriasis-like skin lesion.


Asunto(s)
Antígeno B7-H1/genética , Proteína Quinasa CDC2/genética , Psoriasis/tratamiento farmacológico , Anomalías Cutáneas/tratamiento farmacológico , Adolescente , Adulto , Anciano , Animales , Proteína Quinasa CDC2/antagonistas & inhibidores , Modelos Animales de Enfermedad , Epidermis/efectos de los fármacos , Epidermis/patología , Femenino , Citometría de Flujo , Regulación de la Expresión Génica/efectos de los fármacos , Humanos , Indoles/farmacología , Interferón gamma/genética , Interleucina-17/genética , Queratinocitos/efectos de los fármacos , Queratinocitos/patología , Masculino , Ratones , Persona de Mediana Edad , Cultivo Primario de Células , Psoriasis/genética , Psoriasis/patología , Análisis de Secuencia de ARN/métodos , Anomalías Cutáneas/genética , Anomalías Cutáneas/patología , Receptor Toll-Like 3/genética , Receptor Toll-Like 4/genética , Adulto Joven
8.
J Med Case Rep ; 12(1): 83, 2018 Mar 24.
Artículo en Inglés | MEDLINE | ID: mdl-29571300

RESUMEN

BACKGROUND: Dermatomyositis is a humoral-mediated inflammatory myopathy with symmetrical proximal muscle weakness and dermatological manifestations such as Gottron's papules, heliotrope rash, periungual abnormalities, and flagellate erythema. Erythroderma is a severe and potentially life-threatening dermatological condition with diffuse erythema and scaling involving more than 90% of the skin surface area. Poikiloderma vasculare atrophicans refers to mottled hyperpigmentation and hypopigmentation of the skin with in-between telangiectases and areas of atrophy and is considered a variant of mycosis fungoides. Poikilodermatomyositis is the term given to the condition with poikiloderma and inflammatory myopathy. Only a few cases are reported on erythroderma in dermatomyositis and poikilodermatomyositis. Erythrodermal pattern of dermatomyositis transforming into poikilodermatomyositis is a recognized rare manifestation of dermatomyositis and we could find only one case report in the literature. CASE PRESENTATION: A 53-year-old Sri Lankan woman presented with intermittent fever of 5 months' duration with erythroderma. Later she developed progressive, symmetrical proximal muscle weakness. Following a short course of small dose steroids, erythroderma settled but changed to extensive poikiloderma involving more than 90% of her skin with her face being relatively spared. She had an early heliotrope rash, shawl sign, and Gottron papules. Electromyography and muscle biopsy were supportive of inflammatory myositis and skin biopsy showed evidence of dermatomyositis. Inflammatory markers and muscle enzymes were also elevated. Autoimmune antibodies and myositis-specific autoantibodies were negative. She was started on orally administered prednisolone 1 mg/kg per day with methotrexate 10 mg once a week and had a good response to treatment with resolution of the skin condition and improvement of muscle power. Imaging studies, endoscopies, and tumor markers did not reveal any malignancy. CONCLUSIONS: This case illustrates a rare presentation of dermatomyositis initially presenting as fever, erythroderma, and proximal muscle weakness and later developing poikiloderma involving more than 90% of the skin. It is important to be aware of this rare presentation to avoid misdiagnosis. With the currently available literature it is not possible to conclude that erythroderma is a bad prognostic factor in dermatomyositis or a predictive factor for a malignancy. Patients have a good response to steroids with a combination of immunosuppressants.


Asunto(s)
Dermatomiositis/complicaciones , Dermatomiositis/diagnóstico , Neutropenia/complicaciones , Neutropenia/diagnóstico , Anomalías Cutáneas/complicaciones , Anomalías Cutáneas/diagnóstico , Fármacos Dermatológicos/uso terapéutico , Dermatomiositis/tratamiento farmacológico , Dermatomiositis/patología , Electromiografía , Femenino , Fiebre , Humanos , Metotrexato/uso terapéutico , Persona de Mediana Edad , Debilidad Muscular , Neutropenia/tratamiento farmacológico , Neutropenia/patología , Prednisolona/uso terapéutico , Anomalías Cutáneas/tratamiento farmacológico , Anomalías Cutáneas/patología , Resultado del Tratamiento
10.
J Med Case Rep ; 11(1): 135, 2017 May 14.
Artículo en Inglés | MEDLINE | ID: mdl-28501028

RESUMEN

BACKGROUND: Castleman disease is a rare lymphoproliferative disorder presenting with localized or disseminated lymphadenopathy and systemic manifestations. It can be categorized in numerous ways, such as unicentric versus multicentric, histopathological variants (hyaline-vascular, plasma cell, and mixed), or subtypes based on causative viral infections (human immunodeficiency virus, human herpesvirus-8, or Kaposi sarcoma herpesvirus). Presentation ranges from asymptomatic to symptoms involving multiple organs. Even though the exact mechanism of pathogenesis is unknown, treatment is directed toward possible etiologies such as interleukin-6, cluster of differentiation 20, and viral agents. CASE PRESENTATION: A 36-year-old Sri Lankan woman presented with generalized body swelling and foamy urine of 2 weeks' duration. Examination revealed pallor; generalized edema; axillary, cervical, and inguinal lymphadenopathy; hypertension; and hepatomegaly. Investigations showed bicytopenia, nephrotic range proteinuria with hypoalbuminemia, hypogammaglobulinemia, and features of hyaline-vascular type Castleman disease in a lymph node biopsy. She was managed with rituximab and had good clinical improvement. CONCLUSIONS: Castleman disease has a broad spectrum of clinical manifestations, disease pathogeneses, and associations and/or complications. Medical professionals need to be familiar with this spectrum because timely diagnosis and aggressive targeted therapy are the cornerstones of managing these patients.


Asunto(s)
Enfermedad de Castleman/diagnóstico , Diarrea/diagnóstico , Enfermedades Hereditarias del Ojo/diagnóstico , Hepatomegalia/patología , Enfermedades Intestinales/diagnóstico , Linfadenopatía/patología , Proteinuria/patología , Anomalías Cutáneas/diagnóstico , Enfermedades Vasculares/diagnóstico , Adulto , Antihipertensivos/uso terapéutico , Enfermedad de Castleman/complicaciones , Enfermedad de Castleman/tratamiento farmacológico , Diarrea/complicaciones , Diarrea/tratamiento farmacológico , Diuréticos/uso terapéutico , Enfermedades Hereditarias del Ojo/complicaciones , Enfermedades Hereditarias del Ojo/tratamiento farmacológico , Femenino , Hepatomegalia/etiología , Humanos , Factores Inmunológicos/uso terapéutico , Enfermedades Intestinales/complicaciones , Enfermedades Intestinales/tratamiento farmacológico , Linfadenopatía/etiología , Proteinuria/etiología , Rituximab/uso terapéutico , Biopsia del Ganglio Linfático Centinela , Anomalías Cutáneas/complicaciones , Anomalías Cutáneas/tratamiento farmacológico , Resultado del Tratamiento , Enfermedades Vasculares/complicaciones , Enfermedades Vasculares/tratamiento farmacológico
12.
Skin Res Technol ; 23(3): 295-302, 2017 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-27796064

RESUMEN

BACKGROUND: An objective measurement of scar is important for evaluating treatment outcomes. However, to date, there is no 'gold standard' for quantitative measurement of properties of hypertrophic scar. Existing objective modalities are neither portable nor easy to use. OBJECTIVE: This study aims to validate the correlation between objective measurements with SkinFibrometer® , SkinGlossMeter® , and Mexameter® and subjective assessment with Vancouver Scar Scale (VSS) of keloid and hypertrophic scar. METHODS: A total of 25 patients with keloids and hypertrophic scars were enrolled in this study. Patients were treated with intralesional triamcinolone acetonide at 2-6 week intervals. Scar assessments using VSS, Skinfibrometer, Skinglossmeter, and Mexameter were performed in both scars and contralateral normal skin at each treatment session. Correlations between the measurements by these tools and VSS parameters were examined. RESULTS: We found statistically significant differences between scar and contralateral normal skin using Skinfibrometer, Skinglossmeter, and Mexameter. A strong correlation was found between the VSS pliability scores and the stiffness of skin of Skinfibrometer (r = 0.628, P < 0.001). VSS vascularity scores showed weak correlation with erythema index of Mexameter (r = 0.372, P < 0.001). However, no correlation appeared to exist between any parameters of VSS and Skinglossmeter and between VSS pigmentation scores and the melanin index of Mexameter. CONCLUSION: In our study, Skinfibrometer can be an objective noninvasive evaluation tool for pliability of the scar.


Asunto(s)
Cicatriz Hipertrófica/diagnóstico por imagen , Cicatriz Hipertrófica/patología , Queloide/diagnóstico por imagen , Queloide/patología , Piel/diagnóstico por imagen , Piel/patología , Adolescente , Adulto , Anciano , Niño , Cicatriz Hipertrófica/tratamiento farmacológico , Femenino , Glucocorticoides/uso terapéutico , Humanos , Inyecciones Intralesiones/métodos , Queloide/tratamiento farmacológico , Masculino , Persona de Mediana Edad , Piel/efectos de los fármacos , Anomalías Cutáneas/diagnóstico por imagen , Anomalías Cutáneas/tratamiento farmacológico , Anomalías Cutáneas/patología , Resultado del Tratamiento , Triamcinolona Acetonida/administración & dosificación , Triamcinolona Acetonida/uso terapéutico , Adulto Joven
13.
Mol Med Rep ; 13(2): 1661-6, 2016 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-26707180

RESUMEN

The present study aimed to investigate the effect of nano-microcapsule-basic fibroblast growth factor (bFGF) combined with hypoxia-inducible factor-1 (HIF-1) on the random skin flap survival of rats. Male Sprague-Dawley rats were used to establish the McFarlane flap model and subsequently, all model rats were randomly divided into four groups: Control, bFGF, HIF-1 and bFGF combined with HIF-1. The model rats were treated with 2.5 µg/day bFGF and 1.0 µg/day HIF-1 for 5 days by intraperitoneal injection. On day 5 following treatment, the boundaries between necrotic and surviving regions were significantly inhibited by bFGF combined with HIF-1. bFGF combined with HIF-1 inhibited oxidative stresses and inflammatory factors in random skin flap survival of rats. bFGF combined with HIF-1 also activated the protein expression levels of cyclooxygenase (COX)-2 and vascular endothelial growth factor (VEGF) in the random skin flap survival of rats. In conclusion, nano-microcapsule bFGF combined with HIF-1 prevented random skin flap survival in rats through antioxidative, anti-inflammatory and activation of the protein expression levels of COX-2 and VEGF.


Asunto(s)
Cápsulas/administración & dosificación , Factor 2 de Crecimiento de Fibroblastos/administración & dosificación , Subunidad alfa del Factor 1 Inducible por Hipoxia/administración & dosificación , Anomalías Cutáneas/tratamiento farmacológico , Animales , Ciclooxigenasa 2/biosíntesis , Factor 2 de Crecimiento de Fibroblastos/química , Regulación de la Expresión Génica , Supervivencia de Injerto , Subunidad alfa del Factor 1 Inducible por Hipoxia/química , Masculino , Nanopartículas/administración & dosificación , Ratas , Ratas Sprague-Dawley , Anomalías Cutáneas/patología , Colgajos Quirúrgicos/patología , Factor A de Crecimiento Endotelial Vascular/biosíntesis
14.
Zhonghua Shao Shang Za Zhi ; 32(12): 714-720, 2016 Dec 20.
Artículo en Chino | MEDLINE | ID: mdl-28043294

RESUMEN

Objective: To observe the effects of Huanglian ointment on wound healing of mice with full-thickness skin defect, and to explore the related mechanism. Methods: Thirty male C57BL/6J mice were divided into Huanglian ointment group and vehicle group according to the random number table after round wounds of full-thickness skin defect with diameter of 7.5 mm were inflicted on the back of each mouse, with 15 mice in each group. Wounds of mice in Huanglian ointment group and vehicle group were treated with Huanglian ointment and vehicle respectively from post injury day (PID) 1 on, 2 times each day. Five mice from each group were selected to observe wound changes on PID 0, 3, 7, 10, and 14, and wound healing rates were calculated. Five mice out of the 10 mice that hadn't been used for general observation in each group were sacrificed on PID 3 and 7 respectively, and 5 mice after being used for general observation in each group were sacrificed on PID 14. Wound and skin tissue within 2 mm from the edge of wound was collected. Histologic scoring was conducted based on the histomorphological observation with HE staining. The expression of double positive cells of alpha smooth muscle actin (α-SMA) and Ki-67 (myofibroblast) in tissue of wounds of mice was observed by immunofluorescence staining. Protein expressions of transforming growth factor beta (TGF-ß) and collagen in tissue of wounds of mice were determined by enzyme-linked immunosorbent assay. Data were processed with analysis of variance for repeated measurement, analysis of variance of factorial design, t test of two independent samples, one-way analysis of variance, and Bonferronni test or correction. Results: (1) Wounds of mice in two groups were red and swollen on PID 0, while they were neither red nor swollen with scabs on PID 3 and 7. On PID 10, woundsof mice in Huanglian ointment group contracted obviously, while the contracted wounds of mice in vehicle group were smaller than those in Huanglian ointment group. On PID 14, wounds of most mice in Huanglian ointment group were healed, while wounds of some mice in vehicle group failed to heal. Wound healing rates of mice in two groups were close on PID 3 and 7 (with t values respectively 0.64 and 1.90, P values above 0.05). Wound healing rates of mice in Huanglian ointment group on PID 10 and 14 were (76±7)% and (93±5)% respectively, significantly higher than those of vehicle group [(48±9)% and (68±11)%, with t values respectively 7.44 and 3.89, P values below 0.01]. Wound healing rates of mice in two groups on PID 7, 10, and 14 were significantly higher than those on the previous time points of the same group (with P values below 0.01). (2) Histologic scores of wounds of mice in two groups were close on PID 3 (t=-0.76, P>0.05). Histologic scores of wounds of mice in Huanglian ointment group on PID 7 and 14 were (7.0±1.6) and (11.6±2.1) points respectively, significantly higher than those of vehicle group [(4.2±1.3) and (7.2±1.3) points, with t values respectively 1.96 and 2.50, P<0.05 or P<0.01]. Histologic scores of wounds of mice in two groups on PID 7 and 14 were significantly higher than those on the previous time points of the same group (with P values below 0.01). (3) Percentages of double positive cells of α-SMA and Ki-67 in tissue of wounds of mice in Huanglian ointment group on PID 3 and 7 were (35±12)% and (62±10)% respectively, significantly higher than those of vehicle group [(17±12)% and (34±6)%, with t values respectively -2.48 and -5.25, P<0.05 or P<0.01]. The percentage of double positive cells of α-SMA and Ki-67 in tissue of wounds of mice in Huanglian ointment group on PID 14 was (25±5)%, significantly lower than that of vehicle group [(44±17)%, t=2.50, P<0.05]. The percentage of double positive cells of α-SMA and Ki-67 in tissue of wounds of mice on PID 7 was significantly higher than that on PID 3 or 14 in Huanglian ointment group (with P values below 0.01). Percentages of double positive cells of α-SMA and Ki-67 in tissue of wounds of mice on PID 7 and 14 were significantly higher than those on the previous time points in vehicle group (with P values below 0.05). (4) Protein expressions of TGF-ß in tissue of wounds of mice in Huanglian ointment group on PID 3 and 7 were (396±45) and (722±96) pg/mL respectively, significantly higher than those of vehicle group [(290±42) and (382±62) pg/mL, with t values respectively -8.17 and -6.65, P values below 0.01]. Protein expressions of TGF-ß in tissue of wounds of mice in two groups were close on PID 14 (t=1.60, P>0.05). The protein expression of TGF-ß in tissue of wounds of mice in Huanglian ointment group on PID 7 was significantly higher than that on PID 3 or 14 (with P values below 0.01). Protein expressions of TGF-ß in tissue of wounds of mice in vehicle group on PID 7 and 14 were significantly higher than those on the previous time points (with P values below 0.05). Protein expressions of collagen in tissue of wounds of mice in two groups were close on PID 3 (t=1.99, P>0.05). Protein expressions of collagen in tissue of wounds of mice in Huanglian ointment on PID 7 and 14 were (47±10) and (70±14) ng/mL respectively, significantly higher than those of vehicle group [(34±10) and (42±12) ng/mL, with t values respectively 3.15 and 3.52, P<0.05 or P<0.01]. Protein expressions of collagen in tissue of wounds of mice in two groups on PID 7 and 14 were significantly higher than those on the previous time points of the same group (P<0.05 or P<0.01). Conclusions: Huanglian ointment can promote wound healing of full-thickness skin defect of mice through increasing production of myofibroblasts and protein expressions of TGF-ß and collagen.


Asunto(s)
Medicamentos Herbarios Chinos/uso terapéutico , Pomadas/uso terapéutico , Anomalías Cutáneas/tratamiento farmacológico , Cicatrización de Heridas/efectos de los fármacos , Actinas/metabolismo , Animales , Colágeno/metabolismo , Ensayo de Inmunoadsorción Enzimática , Masculino , Ratones , Ratones Endogámicos C57BL
15.
J Endocrinol Invest ; 39(2): 227-33, 2016 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-26100530

RESUMEN

OBJECTIVE: Blepharophimosis syndrome (BPES) is an autosomal dominant genetic condition resulting from heterozygous mutations in the FOXL2 gene and clinically characterized by an eyelid malformation associated (type I) or not (type II) with premature ovarian failure. The distinction between the two forms is critical for female patients, as it may allow to predict fertility and to plan an appropriate therapy. Identifying an underlying causative mutation is not always predictive of the clinical type of BPES since genotype-phenotype correlations are not yet fully delineated. Here, we describe the clinical and hormonal phenotypes of three female patients with BPES type 1 from two novel families, correlate their phenotypes with identified mutations, and investigate the effects of hormone replacement therapy (HRT). METHODS: Clinical, biochemical, and genetic evaluation were undertaken in all the patients and genotype-phenotype correlation was analyzed. The effects of substitutive hormonal therapy on secondary sexual characteristics development and induction of menarche were evaluated. RESULTS: All patients presented with primary amenorrhea or other signs of ovarian dysfunction. Two distinct mutations, a missense p.H104R change and an in-frame p.A222_A231dup10 duplication in the FOXL2 gene were identified. Observed phenotypes were not in accordance with the prediction based on the current genotype-phenotype correlations. HRT significantly improved secondary sexual characteristics development, as well as the induction of menarche. CONCLUSIONS: This study highlights the importance of early recognition of BPES and emphasizes the need of personalized therapy and follow-up in female patients carrying distinct FOXL2 mutations.


Asunto(s)
Amenorrea/etiología , Blefarofimosis/genética , Factores de Transcripción Forkhead/genética , Duplicación de Gen , Mutación Missense , Ovario/fisiopatología , Insuficiencia Ovárica Primaria/etiología , Anomalías Cutáneas/genética , Anomalías Urogenitales/genética , Adulto , Amenorrea/prevención & control , Sustitución de Aminoácidos , Blefarofimosis/tratamiento farmacológico , Blefarofimosis/fisiopatología , Blefarofimosis/cirugía , Terapia Combinada , Análisis Mutacional de ADN , Párpados/anomalías , Femenino , Proteína Forkhead Box L2 , Estudios de Asociación Genética , Terapia de Reemplazo de Hormonas , Humanos , Italia , Menarquia/efectos de los fármacos , Ovario/efectos de los fármacos , Linaje , Insuficiencia Ovárica Primaria/prevención & control , Anomalías Cutáneas/tratamiento farmacológico , Anomalías Cutáneas/fisiopatología , Anomalías Cutáneas/cirugía , Anomalías Urogenitales/tratamiento farmacológico , Anomalías Urogenitales/fisiopatología , Anomalías Urogenitales/cirugía , Adulto Joven
16.
J Wound Care ; 24(10): 446-50, 2015 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-26488735

RESUMEN

OBJECTIVE: Epidermolysis bullosa (EB) describes a number of genetically inherited conditions which cause skin fragility and minor trauma leading to skin damage, skin loss and wounding. Owing to the fragility of the skin and requirement for frequent dressing changes, at present, the optimal dressing(s) is not clear. Our objective was to assess the use of a keratin gel in the management of wounds in patients with different forms of EB. METHOD: We treated patients with different types of EB and a range of wounds with a novel keratin gel. In a convenience sample of consecutive patients, we introduced the keratin gel into their treatment regimen maintaining other aspects of their care. RESULTS: Patients reported faster healing and more resilient healed skin. Of the ten patients treated in this pilot study, six found the gel effective; two found it ineffective; and in two patients, it caused itching leading to discontinuation of the treatment. CONCLUSION: The results of this case study series suggest that keratin gel can be useful in the management of EB and are consistent with previous published experiences.


Asunto(s)
Vendajes , Epidermólisis Ampollosa/tratamiento farmacológico , Queratinas/uso terapéutico , Anomalías Cutáneas/tratamiento farmacológico , Cicatrización de Heridas/efectos de los fármacos , Adolescente , Niño , Preescolar , Manejo de la Enfermedad , Epidermólisis Ampollosa/complicaciones , Femenino , Geles/uso terapéutico , Humanos , Lactante , Masculino , Proyectos Piloto , Anomalías Cutáneas/etiología , Resultado del Tratamiento
17.
Cornea ; 34(10): 1326-8, 2015 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-26266434

RESUMEN

PURPOSE: To describe corneal cross-linking (CXL) as a treatment option for brittle cornea syndrome (BCS). METHODS: Case report. RESULTS: Ethical decision making enabled bilateral sequential transepithelial CXL in an 11-year-old girl with BCS. Postoperative courses were uneventful with a bilateral stromal demarcation line, unchanged corneal transparency, constant endothelial cell density, and stable topography 2 years after intervention. CONCLUSIONS: Modified CXL can safely be performed in patients with BCS. Ethical review may be helpful for interventions deviating from standard practice.


Asunto(s)
Colágeno/metabolismo , Sustancia Propia/metabolismo , Reactivos de Enlaces Cruzados , Anomalías del Ojo/tratamiento farmacológico , Inestabilidad de la Articulación/congénito , Fármacos Fotosensibilizantes/uso terapéutico , Riboflavina/uso terapéutico , Anomalías Cutáneas/tratamiento farmacológico , Niño , Proteínas de Unión al ADN/genética , Anomalías del Ojo/genética , Anomalías del Ojo/metabolismo , Femenino , Humanos , Inestabilidad de la Articulación/tratamiento farmacológico , Inestabilidad de la Articulación/genética , Inestabilidad de la Articulación/metabolismo , Mutación , Anomalías Cutáneas/genética , Anomalías Cutáneas/metabolismo , Factores de Transcripción/genética , Rayos Ultravioleta , Agudeza Visual
18.
Pediatr Int ; 57(2): e34-6, 2015 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-25868957

RESUMEN

Diffuse neonatal hemangiomatosis (DNH) is a rare condition characterized by the concomitant development of multiple cutaneous infantile hemangiomas (IH) and visceral hemangiomas. Recently, an association between erythropoietin treatment and an increased incidence of infantile hemangioma was noted. A Japanese male infant was born via cesarean section at 27 weeks of gestation. Following the commencement of erythropoietin treatment for anemia of prematurity, he developed multiple cutaneous hemangiomas, high cardiac output heart failure and hepatomegaly. Abdominal imaging indicated comorbidity of diffuse infantile hepatic hemannigomas, resulting in the final diagnosis of DNH. The discontinuation of erythropoietin treatment and long-term therapy with propranolol improved the hepatic lesions and cutaneous hemangiomas. The possibility of multiple organ involvement and the exacerbating effects of erythropoietin treatment should be considered in cases in which multiple cutaneous hemangiomas develop in preterm infants receiving erythropoietin treatment.


Asunto(s)
Eritropoyetina/efectos adversos , Hemangioma/inducido químicamente , Recién Nacido de muy Bajo Peso , Inestabilidad de la Articulación/inducido químicamente , Fimosis/inducido químicamente , Anomalías Cutáneas/inducido químicamente , Antagonistas Adrenérgicos beta/uso terapéutico , Anemia Neonatal/tratamiento farmacológico , Edad Gestacional , Hemangioma/diagnóstico , Hemangioma/tratamiento farmacológico , Humanos , Recién Nacido , Recien Nacido Prematuro , Inestabilidad de la Articulación/diagnóstico , Inestabilidad de la Articulación/tratamiento farmacológico , Imagen por Resonancia Magnética , Masculino , Fimosis/diagnóstico , Fimosis/tratamiento farmacológico , Propranolol/uso terapéutico , Anomalías Cutáneas/diagnóstico , Anomalías Cutáneas/tratamiento farmacológico
20.
Rev. pediatr. electrón ; 11(1)abr. 2014.
Artículo en Español | LILACS | ID: lil-719016

RESUMEN

Es conocido que la radiación ha sido asociada a una cicatrización disminuida de las heridas, necrosis grasa y decoloración. La quimioterapia, por otra parte, se ha descrito con efectos adversos como infección, necrosis, contractura o deformación de los colgajos. Los antecedentes previos son los causantes de cierta confusión al enfrentar pacientes oncológicos que durante su evolución requieren solucionar defectos de piel y tejidos blandos: es seguro realizar injertos o colgajos en un paciente que está siendo tratado con quimio o radioterapia? Por medio de la revisión de la literatura publicada buscamos dar respuesta a esta interrogante.


It is known that the radiation has been associated with decreased wound healing, fat necrosis and bleaching. Chemotherapy, on the other hand, has been described with side effects as infection, necrosis, contraction or deformation of the flaps. The previous history are causing some confusion that may arise when facing cancer patients that during their evolution require fix skin and soft tissue defects: is it safe do grafts or flaps in a patient being treated with chemotherapy or radiation therapy? Through the review of the published literature we seek to answer this question.


Asunto(s)
Humanos , Masculino , Femenino , Niño , Adulto , Anomalías Cutáneas/tratamiento farmacológico , Colgajos Quirúrgicos , Neoplasias/cirugía , Quimioterapia Adyuvante/efectos adversos , Radioterapia Adyuvante/efectos adversos , Trasplantes/anomalías , Anomalías Cutáneas/radioterapia , Neoplasias/tratamiento farmacológico , Neoplasias/radioterapia , Piel , Terapia Combinada/efectos adversos
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