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1.
An. bras. dermatol ; 99(1): 43-52, Jan.-Feb. 2024. tab, graf
Article in English | LILACS-Express | LILACS | ID: biblio-1527699

ABSTRACT

Abstract Background: Pemphigus constitutes a group of autoimmune bullous diseases. A reduction in the incidence of endemic pemphigus foliaceus and an increase in pemphigus vulgaris has been described, but there are no studies in Minas Gerais that address the subject. Objective: To describe the epidemiological and clinical profile of patients with pemphigus treated at the Dermatology Service of a public University Hospital in the state of Minas Gerais, Brazil. Methods: An observational, descriptive, and cross-sectional study was carried out of cases of endemic pemphigus foliaceus and pemphigus vulgaris, for a period of six months. A questionnaire was filled out with epidemiological and clinical data on the disease. Results: A total of 122 patients were included in the study, 64 with endemic pemphigus foliaceus and 58 with pemphigus vulgaris. When comparing patients with endemic pemphigus foliaceus and those with pemphigus vulgaris, a statistical difference was observed between the median age of initial disease manifestation (p = 0.001), patient occupation (p = 0.010), area of residence (p = 0.000), forests (p = 0.000) and rivers/streams close to the dwelling (p = 0.001) and the number of systemic medications required to control the disease (p = 0.002). When comparing patients with endemic pemphigus foliaceus to those evaluated in a study carried out at the same service in 2008, there was a statistical difference in the area of residence (p = 0.030). Study limitations: The assessed population comes from a tertiary care service that is not a reference for the entire state. Conclusions: Patients with endemic pemphigus foliaceus and pemphigus vulgaris maintain statistically significant differences regarding their main variables in the literature, such as age and area of residence. Historically, there has been a reduction in cases of endemic pemphigus foliaceus and an increase in cases of pemphigus vulgaris in this population.

2.
Hepatología ; 5(1): 13-24, ene 2, 2024. fig, tab
Article in Spanish | LILACS, COLNAL | ID: biblio-1525303

ABSTRACT

La piel y sus anexos tienen amplia relación con todos los órganos y sistemas. Los cambios presentes en estos pueden ser el primer hallazgo en un paciente con enfermedad hepática, encontrándose hasta en el 20 % de los casos, por lo que las manifestaciones extrahepáticas adquieren importancia, y aunque muchas de estas no son específicas, algunos marcadores dermatológicos pueden ayudar al diagnóstico de la enfermedad y se pueden correlacionar con su severidad. El desarrollo de las lesiones cutáneas en los pacientes con cirrosis hepática se genera principalmente por hipertensión portal y exceso de estrógenos circulantes, por lo tanto, su tratamiento se basa en el manejo de la patología hepática subyacente. En el presente artículo se hace una revisión de la literatura y se describe un amplio espectro de manifestaciones dermatológicas asociadas a cirrosis hepática, con sus características y etiopatogénesis, siendo las más frecuentes la ictericia, el prurito, los nevus en araña, el eritema palmar, las venas en cabeza de Medusa, y los cambios de uñas y del vello, entre otras.


The skin and its annexes have a broad relationship with all organs and systems. Changes present in these can be the initial finding in a patient with liver disease, occurring in up to 20% of cases, therefore the extrahepatic manifestations become important, and although many of these are not specific, some dermatological markers can help in the diagnosis of the disease and may correlate with its severity. The development of cutaneous lesions in patients with liver cirrhosis is mainly generated by portal hypertension and excess of circulating estrogens, therefore their treatment is based on managing the underlying liver pathology. This article reviews the literature and describes a wide range of dermatological manifestations associated with liver cirrhosis, with their characteristics and etiopathogenesis, being the most frequent jaundice, pruritus, spider nevus, palmar erythema, caput Medusae veins, nail and hair changes, among others.


Subject(s)
Humans
3.
São Paulo med. j ; 142(4): 2023148, 2024. tab, graf
Article in English | LILACS-Express | LILACS | ID: biblio-1536908

ABSTRACT

ABSTRACT BACKGROUND: The prevalence of chronic kidney disease (CKD) has increased in the recent decades, along with the number of patients in the terminal stages of this disease, requiring transplantation. Some skin disorders are more frequent in patients with CKD and in renal transplant recipients (RTR). OBJECTIVES: To evaluate the frequency of skin diseases in RTR and patients with CKD receiving conservative treatment. DESIGN AND SETTING: This observational cross-sectional study recruited consecutive patients with CKD and RTR from a nephrology clinic at a teaching hospital in Brazil between 2015 and 2020. METHODS: Quantitative, descriptive, and analytical approaches were used. The sample was selected based on convenience sampling. Data were collected from dermatological visits and participants' medical records. RESULTS: Overall, 308 participants were included: 206 RTR (66.9%, median age: 48 years, interquartile range [IQR] 38.0-56.0, 63.6% men) and 102 patients with CKD (33.1%, median age: 61.0 years, IQR 50.0-71.2, 48% men). The frequency of infectious skin diseases (39.3% vs. 21.6% P = 0.002) were higher in RTR than in patients with CKD. Neoplastic skin lesions were present in nine (4.4%) RTR and in only one (1.0%) patient with CKD. Among the RTR, the ratio of basal cell carcinoma to squamous cell carcinoma was 2:1. CONCLUSIONS: This study revealed that an increased frequency of infectious skin diseases may be expected in patients who have undergone kidney transplantation. Among skin cancers, BCC is more frequently observed in RTR, especially in those using azathioprine.

4.
Article in English | LILACS-Express | LILACS | ID: biblio-1521598

ABSTRACT

ABSTRACT Objective: The objective of this study was to describe a case of cutaneous lichen planus (LP) that appeared following COVID-19 infection. Case description: We report a case of extensive cutaneous classic familial LP in a 4-year-old male child after an asymptomatic serologically confirmed COVID-19 infection. The patient developed intensely itchy, purple, flat-topped papules and plaques, mainly on the dorsal surface of the hands, feet, forearms, and shins. Histopathological examination of the skin biopsy showed vacuolar and apoptotic degeneration of the basal cell layer with a band-like lymphocyte infiltrate at the dermo-epidermal junction and confirmed the diagnosis of LP. Comments: LP could be considered among the differential diagnoses of pediatric post-COVID inflammatory skin lesions, either in the patients recovering from COVID-19 infection or in the suspicious asymptomatic cases in close contact with COVID-19-infected patients.


RESUMO Objetivo: Descrever um caso de líquen plano cutâneo (LP) após infecção por COVID-19. Descrição do caso: Relatamos um caso de LP familiar clássico extenso cutâneo em uma criança de quatro anos de idade após uma infecção por COVID-19 assintomática e sorologicamente confirmada. O paciente desenvolveu pápulas e placas intensamente pruriginosas, roxas e achatadas, principalmente na superfície dorsal das mãos, pés, antebraços e canelas. O exame histopatológico da biópsia de pele mostrou degeneração vacuolar e apoptótica da camada basal com infiltrado de linfócitos em faixa na junção dermoepidérmica e confirmou o diagnóstico de líquen plano. Comentários: O líquen plano pode ser considerado entre os diagnósticos diferenciais de lesões cutâneas inflamatórias pós-COVID pediátricas, tanto em pacientes em recuperação de infecção por COVID-19 quanto em casos assintomáticos suspeitos em contato próximo com pacientes infectados por COVID-19.

5.
An. Fac. Cienc. Méd. (Asunción) ; 56(3): 89-94, 20231201.
Article in Spanish | LILACS | ID: biblio-1519402

ABSTRACT

Las manifestaciones típicas de la Chikungunya son la fiebre y las artralgias en la mayoría de los casos, pero debemos recordar que presentan manifestaciones mucocutáneas, donde la más frecuente es el exantema, además pueden presentar manifestaciones mucocutáneas atípicas con un gran polimorfismo simulando varias dermatosis como los casos expuestos anteriormente. Este reporte cuenta con 5 casos clínicos del mes de enero y febrero del año 2023 en el departamento Central de Paraguay, durante la mayor epidemia de Chikungunya registrada en el país hasta el momento. Es importante conocerlas y correlacionar con los demás signos y síntomas de la enfermedad para un diagnóstico y tratamiento adecuados.


The typical manifestations of Chikungunya are fever and arthralgia in most cases, but we must remember that they present mucocutaneous manifestations, where the most frequent is rash, they can also present atypical mucocutaneous manifestations with great polymorphism simulating various dermatoses such as cases outlined above. This report has 5 clinical cases from the month of January and February 2023 in the Central area of Paraguay, during the largest recorded Chikungunya epidemic in the country so far. It is important to know them and correlate them with the other signs and symptoms of the disease for proper diagnosis and treatment.


Subject(s)
Skin Diseases
6.
Article in Spanish | LILACS-Express | LILACS | ID: biblio-1522884

ABSTRACT

El síndrome de Sweet es un tipo de dermatosis neutrofílica infrecuente, caracterizado por un cuadro febril agudo con aparición de lesiones en piel tipo pápulas y placas eritematosas y dolorosas, con neutrofilia periférica acompañante, que mejora con el uso de corticoides. Se presenta el caso de una paciente de 22 años, con vitíligo como enfermedad de base, que acude por un cuadro de 1 semana de evolución de sensación febril no graduada y aparición insidiosa de lesiones en piel foto expuesta. Se realizó estudios laboratoriales e histopatológicos llegando al diagnóstico de síndrome de Sweet. Con los resultados de los estudios paraclínicos se inició tratamiento con corticoides sistémicos y tópicos con excelente respuesta.


Sweet syndrome is a rare type of neutrophilic dermatosis, characterized by an acute febrile picture with the appearance of painful erythematous papules and plaques on the skin, with accompanying peripheral neutrophilia, which improves with the use of corticosteroids. The case of a 22-year-old patient, with vitiligo as the underlying disease, is presented. She attended for a 1-week history of ungraded feverish sensation and insidious appearance of lesions on photo-exposed skin. Laboratory and histopathological studies were carried out, leading to the diagnosis of Sweet syndrome. With the results of the paraclinical studies, treatment with systemic and topical corticosteroids was started with an excellent response.

7.
Article | IMSEAR | ID: sea-223142

ABSTRACT

Background: The prevalence of skin diseases has increased over the last few decades, and they contribute to a significant burden on health-care systems across the world. Aims/Objective: This report looks at the burden of skin and subcutaneous diseases in terms of years lived with disability and age-standardised years lived with disability in India using the Global Burden of Disease Study results from 2017. Methods: Data were obtained from the Global Burden of Disease online interactive tool. Updated estimates of the world’s health for 359 diseases and injuries and 84 risk factors from 1990 to 2017 are available in this interactive tool. Results: Years lived with disability due to skin and subcutaneous diseases accounted for 4.02% of the total years lived with disability in India in 2017. There was an increase of 53.7% in all age standardised years lived with disability for all the skin and subcutaneous diseases from 1990 to 2017. Among skin and subcutaneous diseases, dermatitis contributed maximum years lived with disability (1.40 million; 95% uncertainty interval, 0.82–2.21) in 2017, followed by urticaria (1.02 million; 95% uncertainty interval, 0.06–1.44) with percentage increases of 48.9% and 45.7% respectively. Conclusion: The burden due to infectious skin diseases (e.g., scabies, fungal skin disease and bacterial skin disease) and non-infectious diseases (e.g., dermatitis, urticaria and psoriasis) has increased over the past three decades, however the age-standardised years lived with disability for leprosy, scabies, fungal infections, sexually transmitted infections and non-melanoma skin cancer (basal cell carcinoma) has decreased. The high burden of skin and subcutaneous diseases demand that they be given due importance in the national programmes and health policy of India.

8.
Article | IMSEAR | ID: sea-222023

ABSTRACT

Background: Skin diseases occur in all age groups in developed and developing countries. Various types of skin diseases are found worldwide and depend on factors like environment, surrounding ecology, dietary habits, socioeconomic status, mental health, and literacy. Health-related quality of life (HRQOL) improves after treatment or cure of the diseases. Objectives: To estimate the pattern of skin disease. To find out the quality of life and association of skin disease with sociodemographic factors among patients. Methods: This observational cross-sectional study was done in skin OPD at the tertiary care center, Banda district of Uttar Pradesh, India from May 2022 to July 2022. All patients who attended to skin OPD during study period were included in the study after taking informed verbal consent. Results: Skin diseases had further divided in subcategories which were infectious conditions, pigmentary disorders, inflammatory dermatoses, miscellaneous skin conditions, benign tumor and other lesions with their percentage 44.8, 10.5, 28.6, 7.1, 2.9 and 6.2%, respectively in this study Classes of DQLI where major portion of patients (49.52%) were in ‘Small effect on patient’s life’ category. The relation of age, religion, education and member of household with history of skin disease were found to be highly statistically significant. Conclusion: Infectious skin diseases were found more common in study area. Male populations were more commonly affected. Skin diseases were more common in age group of 18–60 years. Study participants showed no predominant effect on quality of life. But small effect class had major effect on participant’s quality of life.

9.
J. oral res. (Impresa) ; 12(1): 119-126, abr. 4, 2023. ilus
Article in English | LILACS | ID: biblio-1451421

ABSTRACT

Introduction: The present report describes the case of a 12-year-old patient with 17-year follow-up who was previously diagnosed with Papillon-Lefèvre Syndrome (PLS), which is a rare autosomal recessive irregularity in the cathepsin C gene (CTSC) characterized by palmoplantar hyperkeratosis and premature loss of primary and permanent teeth. Case Report: A specific mutation in the c.203 T > G gene inducing loss of function leading to PLS was detected, as was a mutation in the HLA-DRB1*11 allele, which is associated with this syndrome. There is no consanguinity of the parents, and the siblings are entirely healthy. Early identification of the main characteristics of this syndrome is imperative. Accurate diagnosis by genetic analysis allows differential diagnoses and timely comprehensive dental treatment. Conclusions: Additionally, it allows consultation with a dermatologist to maintain or improve the quality of life of patients with this condition due to progressive worsening and severity of the main physical manifestations. Keywords: Papillon-Lefevre Disease; Keratoderma, Palmo-plantar; Cathepsin C; Periodontitis; Skin Diseases, Genetic; Case reports


Introducción: El presente reporte describe el caso de un paciente de 12 años de edad con 17 años de seguimiento a quien previamente se le diagnosticó Síndrome de Papillon-Lefèvre (PLS), el cual es una rara irregularidad autosómica recesiva en el gen de la catepsina C (CTSC) caracterizada por hiperqueratosis palmoplantar y pérdida prematura de dientes primarios y permanentes. Reporte de Caso: Se detectó una mutación específica en el gen c.203 T > G que induce pérdida de función que conduce a PLS, así como una mutación en el alelo HLA-DRB1*11, que se asocia a este síndrome. No presenta consanguinidad de los padres, padres y hermanos totalmente sanos. La identificación temprana de las principales características de este síndrome es imperativa. El diagnóstico certero por análisis genético permite diagnósticos diferenciales y tratamientos odontológicos integrales oportunos. Conclusiones: Adicionalmente, permite la consulta con un dermatólogo para mantener o mejorar la calidad de vida de los pacientes con esta condición debido al progresivo empeoramiento y severidad de las principales manifestaciones físicas.


Subject(s)
Humans , Male , Child , Papillon-Lefevre Disease/diagnostic imaging , Keratoderma, Palmoplantar , Cathepsin C/genetics , Papillon-Lefevre Disease/therapy
10.
Medisur ; 21(2)abr. 2023.
Article in Spanish | LILACS-Express | LILACS | ID: biblio-1440654

ABSTRACT

La sarcoidosis es una enfermedad granulomatosa sistémica de causa desconocida, con mayor prevalencia en el sexo femenino, entre los 20 a 40 años de edad; caracterizada por la formación de granulomas no caseificantes en distintos órganos. La afectación cutánea de forma exclusiva es rara, pues se describe solo en el 4-5 % de los pacientes, ya que las lesiones en piel pueden aparecer antes o después del compromiso sistémico, o bien coincidir con este. Se presenta el caso de una paciente de 35 años con lesiones en piel de cuatro años de evolución. Considerando el cuadro clínico y la histopatología, se concluyó con el diagnóstico de sarcoidosis cutánea, crónica y asintomática. Se trata de una enfermedad muy polimorfa en sus manifestaciones cutáneas, lo cual la convierte en una gran simuladora, y su diagnóstico constituye un reto para el dermatólogo.


Sarcoidosis is a systemic granulomatous disease of unknown cause, with a higher prevalence in females, between 20 and 40 years of age; characterized by the formation of non-caseating granulomas in different organs. Exclusive skin involvement is rare, as it is described in only 4-5% of patients, since skin lesions may appear before or after systemic involvement, or coincide with it. The case of a 35-years-old patient with skin lesions of four years of evolution is presented. Considering the clinical case and histopathology, the diagnosis of chronic and asymptomatic cutaneous sarcoidosis was concluded. It is a very polymorphic disease in its skin manifestations, which makes it a great simulator, and its diagnosis constitutes a challenge for the dermatologist.

11.
Rev. méd. hered ; 34(1): 5-13, ene. - mar. 2023. tab, graf
Article in Spanish | LILACS, LIPECS | ID: biblio-1442070

ABSTRACT

Objetivo: Determinar las características clínicas y demográficas de los pacientes con lesiones de piel atendidos en un hospital general de Trujillo, Perú. Material y métodos: Estudio transversal, descriptivo, observacional y retrospectivo. Se hizo la revisión microscópica de 385 biopsias de piel de los archivos de Patología y de las historias clínicas de pacientes atendidos en el Hospital del Belén de Trujillo, durante el periodo comprendido entre enero del 2018 y diciembre del 2019. Resultados: La lesión más frecuente fue el carcinoma basocelular, localizado en alguna parte del rostro y de presentación mayor a edades de 60 años o más. En segundo lugar, carcinoma epidermoide. Las lesiones malignas afectaron al 66,7% de pacientes entre 55 y 79 años, con edad promedio de 67 años. En su mayoría (65,8%) fueron varones desocupados. De las lesiones benignas, la más frecuente fue el quiste de inclusión epidérmica y se ubicó en el tronco de varones entre 20 y 24 años. En segundo lugar, el pilomatrixoma se ubicó en los miembros superiores de menores de 10 años. Afectaron al 59,6%, entre los 30 y 54 años, con edad promedio de 44,1 años. En su mayoría (63,7%), se reportaron como ama de casa. Procedieron en su mayor parte de los distritos de Trujillo. No fue posible establecer el agente injuriante. Conclusiones: En personas de piel trigueña o cobriza, las lesiones tanto benignas como malignas tuvieron un patrón de presentación similar a la de otros estudios, tanto en localización, grupo etario y sexo. La procedencia y la ocupación no fueron contributorias.


SUMMARY Objective : To determine the clinical and socio demographic features of patients attended at a public hospital in Trujillo, Peru. Methods : A cross-sectional study that included 385 skin biopsies obtained from the archives of the Pathology Department and from the clinical charts of these patients at Hospital Belen in Trujillo from January 2018 to December 2019 was carried-out. Results : The most common entity was basocellular carcinoma located on the face in patients above 60 years of age. The second most common entity was epidermoid carcinoma. Malignant lesions affected 66.7% of patients between 55 and 79 years of age with a mean of 67 years. Most of the patients were unemployed males (65.8%). The most common benign lesion was the epidemic inclusion cyst mostly in the trunk among males from 20 to 24 years of age. The second most common benign lesion was the pilomatrixoma mostly located in the upper limbs of patients below 10 years of age. Benign lesions affected the 59.6% of the sample between 30 to 54 years of age with a mean of 44 years, mostly among housewives. Most of the patients came from districts of Trujillo. The causal agent was not identified. Conclusions : In mestizo patients both benign and malignant lesions had a clinical presentation similar as reported in the literature regarding location, age and gender distribution. Occupation and precedence were not contributory..


Subject(s)
Humans , Skin Diseases , Skin Manifestations , Dermatology , Cross-Sectional Studies
12.
Rev. argent. dermatol ; 104: 31-40, ene. 2023. graf
Article in Spanish | LILACS-Express | LILACS | ID: biblio-1529611

ABSTRACT

Resumen La pitiriasis liquenoide varioliforme aguda (PLEVA) pertenece a un grupo de enfermedades categorizadas como pitiriasis liquenoides, junto con la enfermedad febril úlcero-necrótica de Mucha-Habermann y la pitiriasis liquenoide crónica (PLC). Se caracteriza por la aparición aguda de múltiples pápulas eritemato-violáceas con posterior necrosis, discromía residual y cicatrices varioliformes. Dentro de las teorías patogénicas propuestas se encuentra el posible papel de agentes infecciosos, trastornos linfoproliferativos, complejos inmunes e incluso, asociación a medicamentos. Se presenta un casode una mujer adulta con un cuadro típico de PLEVA con confirmación histopatológica, cuyas lesiones aparecieron posteriormente al inicio de eritropoyetina.


Abstract Pityriasis lichenoides et varioliformes acuta (PLEVA) is part of a group of diseases clustered as pityriasis lichenoides, next to febrile ulceronecrotic Mucha-Habermann disease and pityriasis lichenoides chronica. It's characterized by a sudden onset of multiple erythematous and violaceous papules which develop necrosis, leaving residual dyschromia and varioliform scars. It's been hypothesized the possible role of infectious agents, lymphoproliferative diseases, immune complexes and drugs. We present the case of a woman with a typical PLEVA with histopathological confirmation, whose lesions appeared after therapy with erythropoietin.

13.
Rev. Eugenio Espejo ; 17(1): 5-7, 20230101.
Article in Spanish | LILACS | ID: biblio-1411815

ABSTRACT

La leishmaniasis resulta una enfermedad infectocontagiosa causada por un protozoo del género Leishmania. Esta constituye un problema de salud importante para diversas regiones de América Latina. El aislamiento social impuesta a raíz de la pandemia de COVID-19 favoreció que disminuyera la incidencia de esta entidad, pero las actividades sociales de la normalidad se retomaron paulatinamente, imponiéndose que se retomen las medidas de seguimiento y control correspondientes.


Leishmaniasis is an infectious disease caused by a protozoan of the genus Leishmania. Leishma-niasis constitutes a significant health problem for various regions of Latin America. The social isolation imposed because of the COVID-19 pandemic favored a decrease in the incidence of this entity. However, regular social activities were gradually resumed, imposing the correspon-ding monitoring and control measures.


Subject(s)
Humans , Male , Female , Infant , Child, Preschool , Child , Adolescent , Adult , Middle Aged , Aged , Aged, 80 and over , Epidemiology , Communicable Diseases , Leishmaniasis, Cutaneous , Leishmaniasis , Incidence , Leishmania
14.
Chinese Journal of General Practitioners ; (6): 987-991, 2023.
Article in Chinese | WPRIM | ID: wpr-994793

ABSTRACT

Neutrophil/lymphocyte ratio (NLR) is a inflammatory indicator, which can be easily calculated from the results of complete blood cell count. More and more studies have shown that NLR has its unique value in the diagnosis, and evaluation of severity, complications, treatment efficacy and prognosis of skin diseases. This article reviews the recent progress on the application of NLR in clinical management of skin diseases, such as psoriasis, systemic lupus erythematosus and Behcet disease.

15.
JOURNAL OF RARE DISEASES ; (4): 294-302, 2023.
Article in English | WPRIM | ID: wpr-1005084

ABSTRACT

Monogenic inherited skin diseases are a group of clinically rare diseases that include nearly 1000 phenotypically distinct disorders. Through the concerted efforts of researchers in dermatological sciences and related disciplines worldwide, many advances have been made in the etiology and pathogenesis of these diseases in the last 30 years. However, it is important to note that the treatment of the majority of monogenic inherited skin diseases remains a challenge for clinicians. Dupilumab is a fully human monoclonal IgG4 antibody that specifically binds to the α subunit of the IL-4 receptor, thereby inhibiting the IL-4 and IL-13 signaling pathway. It was first approved for the treatment of moderate-to-severe atopic dermatitis (AD) and has been used worldwide. In recent years, the drug has been successfully used to treat some monogenic inherited skin diseases with AD-like clinical manifestations, such as hyper-IgE syndrome and Netherton syndrome, with good efficacy. The drug was later tried for the treatment of other monogenic inherited skin diseases, such as Hailey-Hailey syndrome and epidermolysis bullosa pruriginosa, where it was also proven to be effective. In this paper, we review literature reports related to dupilumab for the treatment of monogenic inherited skin diseases in recent years, focusing on its efficacy, safety and possible therapeutic mechanisms. We aim to provide a possible scientific basis for the future application of this drug in the field of rare monogenic inherited skin diseases.

16.
JOURNAL OF RARE DISEASES ; (4): 261-272, 2023.
Article in English | WPRIM | ID: wpr-1005079

ABSTRACT

Rare diseases are characterized by extremely low incidence rate and small number of patients in total. The drugs used to treat rare diseases are called orphan drugs. Currently, 450 kinds of rare skin diseases have been identified, most of which lack in effective treatments. Supported by the policy-making from the country and pushed by all sectors of the society, drugs for rare skin diseases have been emerging continuously recently in China. This paper reviews the current accessibility of the orphan drugs for skin rare diseases that have been approved in China and in other countries and regions for a better understanding of rare dermatosis and orphan drugs for the diseases.

17.
JOURNAL OF RARE DISEASES ; (4): 157-163, 2023.
Article in English | WPRIM | ID: wpr-1005070

ABSTRACT

In clinical practice, early diagnosis, accurate assessment and effective management of rare skin diseases are difficult. The Big Data gives rise to the exponential growth of biomedical data, including medical images, multi-omics information and electronic health records. Artificial intelligence (AI), particularly machine learning, has its advantage in processing complex and abundant information. Researches have applied AI in the field of rare skin diseases. In this paper, we briefly describe, discuss, and foresee the research on AI based image data, multi-omics data & text data and AI in assisting rare skin disease drug development, in order to improve the awareness of dermatologist understanding of this field and actively promote the development of AI usage on rare skin diseases.

18.
JOURNAL OF RARE DISEASES ; (4): 153-156, 2023.
Article in English | WPRIM | ID: wpr-1005069

ABSTRACT

There are many kinds of rare skin diseases, but the research into the diagnosis and treatment of rare skin diseases is relatively scarce. In recent years, the rare skin diseases team has made a series of accomplishments, including establishing the professional committee of Rare Skin Disease Committee of China Alliance for Rare Diseases, establishing China's first Medical Care Alliance for Rare Skin Diseases, launching two national collaborative projects, promoting the project of improving the diagnosis and treatment of rare diseases supported by the Central Special Lottery public welfare Fund, exploring the multidisciplinary diagnosis and treatment model of skin rare diseases, holding academic conferences, and compiling professional books on rare skin diseases. In the future, we will further improve the remote consultation model of rare skin diseases, develop artificial intelligence assisted diagnosis system of rare skin diseases, carry out high-quality clinical research, and improve the overall diagnosis and treatment level of rare skin diseases in China, for the sake of benefiting more patients.

19.
Chinese Journal of Blood Transfusion ; (12): 436-439, 2023.
Article in Chinese | WPRIM | ID: wpr-1004842

ABSTRACT

【Objective】 To investigate the feasibility of allogeneic platelet-rich plasma (PRP) for the treatment of herpes zoster wounds secondary to systemic lupus erythematosus (SLE), especially large ulcer wounds. 【Methods】 The treatment process of a patient with massive herpes zoster wounds in perineum and hip accompanied by extensive soft tissue necrosis secondary to SLE was retrospectively analyzed. The clinical efficacy of allogeneic PRP was explored combined with treatment key points and literature review. 【Results】 The patient′s wound bed was prepared until the wound was fresh, then treated externally with allogeneic PRP 3 times a week. The wound was healed completely after 42 days. 【Conclusion】 In the case of autologous PRP unavailable or unsuitable, allogeneic PRP is a safe alternative, which can effectively promote tissue regeneration, and this patient achieved curative effect in a short period of time.

20.
Chinese Journal of Biochemistry and Molecular Biology ; (12): 814-820, 2023.
Article in Chinese | WPRIM | ID: wpr-1015606

ABSTRACT

microRNA-21(miR-21) is an endogenous non-coding RNA and plays a key regulatory role in the process of cell proliferation and differentiation. In recent years, miR-21, as a widely studied miRNA, has attracted much attention for its role in skin- related diseases and wound healing. The study shows that miR-21, as a " broad factor", affects the proliferation, apoptosis, migration and invasion of different cells (keratinocytes, T cells, fibroblasts, etc.) by inhibiting the transcription and translation of different target genes (PTEN, TIMP, PDCD4, etc.) . At the same time, it plays a crucial role in skin tumors, skin immune diseases, skin inflammatory diseases, skin wounds and scar tissue formation by promoting inflammation through different signaling pathways. In this study, we reviewed the regulatory roles of miR-21 in different skin diseases (melanoma, cutaneous squamous cell carcinoma, T-cell lymphoma, psoriasis, scleroderma, etc.) and wound healing, aiming at deepening the understanding of miR-21 molecule in skin-related diseases and wound healing. The potential of miR-21 as a biomarker for skin disease diagnosis and its ability to evaluate the efficacy of drugs were also discussed. miR-21 is expected to become a new target of skin disease and wound healing, which may provide a new direction for clinical research.

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