Registries and biobanks for juvenile dermatomyositis (JDM) have generated statistical power to help understand pathogenesis and determine treatment and long-term outcomes in this rare and heterogeneous disease. Genotype, autoantibodies, muscle histology and early clinical features may predict prognosis and guide personalised treatment. While corticosteroids and disease-modifying anti-rheumatic drugs improve outcomes, there remain children who experience refractory disease. Ongoing research into the aberrant immune response and novel biological targets is necessary. Best practice guidelines promote prompt stepwise treatment, and there is growing appreciation of the role of exercise in improving prognosis. Validated tools standardise assessment of disease activity and damage in musculoskeletal, mucocutaneous, pulmonary, cardiac, gastrointestinal and endocrine systems. Recently, an internationally agreed dataset for JDM has been defined for clinical practice and incorporation into registries. In the future, with bigger datasets, statistical models may guide stratification for personalised medicine and discern the most relevant outcome markers for research.
Background:Eczema herpeticum is a rare, severe, and disseminated infection of herpes simplex virus in the setting of eczematous skin diseases.Methods:We experienced a case of this disease in a 9-month-old infant characterized by a sudden onset of monomorphic vesicles on the head, right lower leg, and two hands. The infant has a 7-month history of atopic dermatitis and his condition was initially regarded as a complication of atopic dermatitis and bacterial infection. After treatment of cefoperazone and dexamethasone, the eruptions got worse. The diagnosis of eczema herpeticum was made according to the clinical features and further confirmed by the findings of herpes simplex virus type 2.Results:The infant was cured by oral treatment with valacyclovir.Conclusion:The case highlights that the awareness for the sign of eczema herpeticum when diagnosing children with a sudden onset of disseminated vesicles in the setting of chronic skin disease should be increased. Oral valacyclovir may be an effective and convenient treatment option for pediatric outpatients with eczema herpeticum.
Psoriasis is a chronic, immune-mediated inflammatory disease which pathogenesis is closely linked to γδ T cells. Recently, a critical role for butyrophilin 3A1 (BTN3A1) in mediating the activation of Vγ9Vδ2 T cells, which are reported to redistribute from blood to the perturbed skin lesions in psoriasis, has been proposed. Additional molecular partners, including RhoB and periplakin, have also been speculated to interact with BTN3A1 in modulating Vγ9Vδ2 T-cell activation. Immunohistochemical staining was performed to examine the expressions of BTN3A1, RhoB, and the plakin family members, including periplakin, epiplakin, and envoplakin in the psoriasis vulgaris lesions as compared with the normal control. The expressions of BTN3A1 and RhoB were found significantly upregulated in the psoriatic lesions. Besides, a downregulation of periplakin and an upregulation of epiplakin were noticed in the psoriasis vulgaris lesions. Our data suggest that BTN3A1 and RhoB might participate in the pathogenesis of psoriasis through Vγ9Vδ2 T-cell responses. In addition, a potential involvement of the plakin protein family, especially periplakin and epiplakin, in psoriasis pathology was proposed.
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