RESUMO
Linear morphea, also known as linear scleroderma, is a localized form of scleroderma characterized by the presence of lesions that follow a linear distribution pattern. Apart from the typical inflammation and fibrosis of the skin, the linear subtype of morphea often affects underlying structures such as muscles and bones, which can lead to functional limitations. Lichen striatus, a linear inflammatory skin condition, primarily affects children aged 5 to 15 years. Interestingly, both diseases can exhibit lesions that follow the lines of Blaschko. Here we report a case with linear morphea following the lines of Blaschko mimicking lichen striatus in a 4-year-old child. This unique case represents the first documented instance of linear morphea exhibiting a precise Blaschko pattern and being successfully treated with baricitinib. The patient received oral baricitinib at a daily dosage of 2 mg for a duration of 1 year, resulting in remarkable improvement. The majority of the lesions softened, and there was no significant disease progression or occurrence of adverse events throughout the treatment period. Recognizing linear morphea at an early stage is of utmost importance in ensuring effective treatment and preventing disfiguring sequelae. Patients suspected of lichen striatus should also be closely followed and linear morphea should be excluded during the follow-up. The recent breakthrough in the application and the safety of baricitinib in scleroderma is also reviewed.
Assuntos
Eczema , Exantema , Ceratose , Esclerodermia Localizada , Dermatopatias , Humanos , Pré-Escolar , Esclerodermia Localizada/diagnóstico , Esclerodermia Localizada/tratamento farmacológico , Esclerodermia Localizada/patologia , Dermatopatias/patologia , Pele/patologia , Eczema/patologiaAssuntos
Antibacterianos/administração & dosagem , Claritromicina/administração & dosagem , Dermatite Perioral/tratamento farmacológico , Pele/patologia , Administração Oral , Pré-Escolar , Dermatite Perioral/diagnóstico , Dermatite Perioral/patologia , Humanos , Masculino , Pescoço , Índice de Gravidade de Doença , Resultado do TratamentoAssuntos
Proteínas de Ciclo Celular/genética , Predisposição Genética para Doença , Síndrome de Rothmund-Thomson/genética , Síndrome de Rothmund-Thomson/patologia , Esclerose/genética , Esclerose/patologia , Anormalidades da Pele/genética , Anormalidades da Pele/patologia , Dermatopatias Genéticas/genética , Dermatopatias Genéticas/patologia , Biópsia por Agulha , China , Contratura/genética , Contratura/patologia , Humanos , Imuno-Histoquímica , Lactente , Masculino , Doenças Musculares/genética , Doenças Musculares/patologia , Mutação de Sentido Incorreto , Fibrose Pulmonar/genética , Fibrose Pulmonar/patologia , Doenças Raras , Medição de Risco , Tendinopatia/genética , Tendinopatia/patologiaRESUMO
Aberrant differentiation of keratinocytes has been demonstrated to be associated with a number of skin diseases. A growing number of studies have showed that long noncoding RNAs (lncRNAs) have an important part in gene regulation, however, the role of lncRNAs in keratinocyte differentiation remains to be largely unknown. In the present study, we demonstrated that lncRNA-H19 act as an endogenous 'sponge', which binds directly to miR-130b-3p and therefore inhibits its activity on Dsg1. MiR-130b-3p was illustrated to inhibit keratinocyte differentiation by targeting Dsg1. H19 regulates Dsg1 expression and the consequent keratinocyte differentiation through miR-130b-3p. Our study casts light on a novel regulatory model of keratinocyte differentiation, which may provide new therapeutic targets of skin diseases.
Assuntos
Queratinócitos/fisiologia , MicroRNAs/genética , MicroRNAs/metabolismo , RNA Longo não Codificante/genética , RNA Longo não Codificante/metabolismo , Diferenciação Celular/fisiologia , Criança , Desmogleína 1/genética , Desmogleína 1/metabolismo , Regulação da Expressão Gênica , Genes Supressores de Tumor , Humanos , Queratinócitos/citologia , Queratinócitos/metabolismo , MicroRNAs/antagonistas & inibidores , TransfecçãoRESUMO
BACKGROUND: Atopic dermatitis (AD) is one of the most common inflammatory cutaneous diseases. Thymic stromal lymphopoietin (TSLP) has been demonstrated to be an important immunologic factor in the pathogenesis of AD. The production of TSLP can be induced by a high level of intracellular calcium concentration and activation of the receptor-interacting protein 2/caspase-1/NF-κB pathway. Andrographolide (ANDRO), a natural bicyclic diterpenoid lactone, has been found to exert anti-inflammatory effects in gastrointestinal inflammatory disorders through suppressing the NF-κB pathway. OBJECTIVE: To explore the effect of ANDRO on the production of TSLP in human mast cells and AD mice model. METHODS: We utilized enzyme-linked immunosorbent assay, real-time reverse transcription polymerase chain reaction analysis, Western blot analysis, and immunofluorescence staining assay to investigate the effects of ANDRO on AD. RESULTS: ANDRO ameliorated the increase in the intracellular calcium, protein, and messenger RNA levels of TSLP induced by phorbol myristate acetate/calcium ionophore A23187, through the blocking of the receptor-interacting protein 2/caspase-1/NF-κB pathway in human mast cell line 1 cells. ANDRO, via oral or local administration, also attenuated clinical symptoms in 2,4-dinitrofluorobenzene-induced AD mice model and suppressed the levels of TSLP in lesional skin. CONCLUSION: Taken together, ANDRO may be a potential therapeutic agent for AD through suppressing the expression of TSLP.