Background Langerhans cell histiocytosis(LCH)is a group of diseases characterized by the proliferation and accumulation of Langerhans cells.Clinical presentations of LCH vary widely.Data sources A PubMed search was co...Background Langerhans cell histiocytosis(LCH)is a group of diseases characterized by the proliferation and accumulation of Langerhans cells.Clinical presentations of LCH vary widely.Data sources A PubMed search was conducted using Clinical Queries with the key term "Langerhans cell histiocytosis".The search strategy included meta-analyses,randomized controlled trials,clinical trials,observational studies,and reviews.This paper is based on,but not limited to,the search results.Results Generally,patients with LCH can be divided into two groups based on the extent of involvement at diagnosis,namely,single-system LCH and multisystem LCH.The involvement may be unifocal or multifocal.Patients with isolated bone lesions typically present between 5 and 15 years of age,whereas those with multisystem LCH tend to present before 5 years of age.The clinical spectrum is broad,ranging from an asymptomatic isolated skin or bone lesion to a life-threatening multisystem condition.Clinical manifestations include,among others,"punched out" lytic bone lesion,seborrheic dermatitis-like erup-tion,erythematous/reddish-brown crusted/scaly papules/maculopapules/plaques/patches,and eczematous lesions,diabetes insipidus,hepatosplenomegaly,cytopenias,lymphadenopathy,and an acute fulminant disseminated multisystem condition presenting with fever,skin rash,anemia,thrombocytopenia,lymphadenopathy,and hepatosplenomegaly.The diagnosis is clinicopathologic,based on typical clinical findings and histologic/immunohistochemical examination of a biopsy of lesional tissue.Positive CD1a,S100,and/or CD207(Langerin)immunohistochemical staining of lesional cells is required for a definitive diagnosis.Watchful waiting is recommended for patients with skin-only LCH.Patients with symptomatic or refractory skin-only LCH may be treated with topical tacrolimus/corticosteroids,topical nitrogen mustard,oral methotrexate,or oral hydroxyurea.The current recommended first-line therapy for patients with multisystem LCH is 12 months therapy with prednisone and vinblast展开更多
Background:Amarogentin(AMA)is a secoiridoid glycoside extracted from Swertia and Gentiana roots and exhibits many biological effects such as antioxidative,antiinflammatory,and antitumor activities.Atopic dermatitis(AD...Background:Amarogentin(AMA)is a secoiridoid glycoside extracted from Swertia and Gentiana roots and exhibits many biological effects such as antioxidative,antiinflammatory,and antitumor activities.Atopic dermatitis(AD)is a chronic inflammatory skin disease caused by disorders in the regulation of multiple inflammatory cytokines.No effective cure has been found for AD now.Methods:We constructed the HaCat and splenocyte model and tested the inhibitory effect of AMA on IL-4,IL-6,and IL-13 secretions using enzyme-linked immunosorbent assay(ELISA).The AD mouse model was constructed and treated with AMA,the severity of skin lesions was observed,epidermal tissue was collected,and epidermal thickness and mast cell infiltration were observed using hematoxylin and eosin and toluidine blue staining,respectively.The expression of kallikrein-related peptidase 7(KLK7)and filaggrin(FLG)was detected using immunostaining and Western blot analysis.The mRNA expression of KLK7 and FLG was detected using quantitative polymerase chain reaction(qPCR).Blood immunoglobulin E(IgE)secretion was detected.Results:AMA inhibited IL-6 secreted by tumor necrosis factor(TNF)-α-induced HaCaT cells and reduced IL-4 and IL-13 secreted by phytohemagglutinin(PHA)-induced primary cells in the mice spleen.It was found that the treatment of AMA with 2,4-din itrochlorobenzene-induced AD-like mice could promote the recovery of dermatitis,reduce the score of dermatitis severity and the scratching frequency,treat the skin lesions,reduce the epidermal thickness,decrease the infiltration of mast cells,reduce the IgE level in serum,decrease the expression levels of AD-related cytokines,increase protein and mRNA expression of FLG,and reduce the protein and mRNA expression of KLK7 in the skin tissues of AD-like mice.Conclusion:In conclusion,AMA inhibits inflammatory response at the cellular level,and AMA reduces the validation response of specific dermatitis mice,relieves pruritus,and repairs the damaged skin barrier.展开更多
文摘Background Langerhans cell histiocytosis(LCH)is a group of diseases characterized by the proliferation and accumulation of Langerhans cells.Clinical presentations of LCH vary widely.Data sources A PubMed search was conducted using Clinical Queries with the key term "Langerhans cell histiocytosis".The search strategy included meta-analyses,randomized controlled trials,clinical trials,observational studies,and reviews.This paper is based on,but not limited to,the search results.Results Generally,patients with LCH can be divided into two groups based on the extent of involvement at diagnosis,namely,single-system LCH and multisystem LCH.The involvement may be unifocal or multifocal.Patients with isolated bone lesions typically present between 5 and 15 years of age,whereas those with multisystem LCH tend to present before 5 years of age.The clinical spectrum is broad,ranging from an asymptomatic isolated skin or bone lesion to a life-threatening multisystem condition.Clinical manifestations include,among others,"punched out" lytic bone lesion,seborrheic dermatitis-like erup-tion,erythematous/reddish-brown crusted/scaly papules/maculopapules/plaques/patches,and eczematous lesions,diabetes insipidus,hepatosplenomegaly,cytopenias,lymphadenopathy,and an acute fulminant disseminated multisystem condition presenting with fever,skin rash,anemia,thrombocytopenia,lymphadenopathy,and hepatosplenomegaly.The diagnosis is clinicopathologic,based on typical clinical findings and histologic/immunohistochemical examination of a biopsy of lesional tissue.Positive CD1a,S100,and/or CD207(Langerin)immunohistochemical staining of lesional cells is required for a definitive diagnosis.Watchful waiting is recommended for patients with skin-only LCH.Patients with symptomatic or refractory skin-only LCH may be treated with topical tacrolimus/corticosteroids,topical nitrogen mustard,oral methotrexate,or oral hydroxyurea.The current recommended first-line therapy for patients with multisystem LCH is 12 months therapy with prednisone and vinblast
基金The present study was supported by the National Natural Science Foundation of China(grant numbers:81902067 and 82078189)the Medical Scientific Research Foundation of Guangdong Province(grant number:A2019502)+2 种基金the Shenzhen Science and Technology Innovation Committee(grant numbers:JCY20180305124849781 and 20200812211704001)the SZU Top Ranking Project(grant number:86000000210)the Hospital Project of Huazhong University of Science and Technology Union Shenzhen Hospital(grant number:2021042).
文摘Background:Amarogentin(AMA)is a secoiridoid glycoside extracted from Swertia and Gentiana roots and exhibits many biological effects such as antioxidative,antiinflammatory,and antitumor activities.Atopic dermatitis(AD)is a chronic inflammatory skin disease caused by disorders in the regulation of multiple inflammatory cytokines.No effective cure has been found for AD now.Methods:We constructed the HaCat and splenocyte model and tested the inhibitory effect of AMA on IL-4,IL-6,and IL-13 secretions using enzyme-linked immunosorbent assay(ELISA).The AD mouse model was constructed and treated with AMA,the severity of skin lesions was observed,epidermal tissue was collected,and epidermal thickness and mast cell infiltration were observed using hematoxylin and eosin and toluidine blue staining,respectively.The expression of kallikrein-related peptidase 7(KLK7)and filaggrin(FLG)was detected using immunostaining and Western blot analysis.The mRNA expression of KLK7 and FLG was detected using quantitative polymerase chain reaction(qPCR).Blood immunoglobulin E(IgE)secretion was detected.Results:AMA inhibited IL-6 secreted by tumor necrosis factor(TNF)-α-induced HaCaT cells and reduced IL-4 and IL-13 secreted by phytohemagglutinin(PHA)-induced primary cells in the mice spleen.It was found that the treatment of AMA with 2,4-din itrochlorobenzene-induced AD-like mice could promote the recovery of dermatitis,reduce the score of dermatitis severity and the scratching frequency,treat the skin lesions,reduce the epidermal thickness,decrease the infiltration of mast cells,reduce the IgE level in serum,decrease the expression levels of AD-related cytokines,increase protein and mRNA expression of FLG,and reduce the protein and mRNA expression of KLK7 in the skin tissues of AD-like mice.Conclusion:In conclusion,AMA inhibits inflammatory response at the cellular level,and AMA reduces the validation response of specific dermatitis mice,relieves pruritus,and repairs the damaged skin barrier.
