RESUMO
Background: Dermatomyositis (DM) is an idiopathic inflammatory myopathy that is clinically challenging to diagnose and has a poor prognosis. It is characterized by symmetric proximal muscle weakness, muscle tenderness, dysphagia, characteristic skin rash (heliotrope rash, Gottron's sign), elevated muscle enzyme levels, abnormal electromyography, and muscle biopsy findings. DM with positive anti-MDA5 antibodies is mainly characterized by Gottron's sign, skin ulcers, facial erythema, mechanic's hands, and V-sign. In this case, the patient presented with the rare manifestation of severe necrotic skin ulcers in association with Gottron's sign, prompting us to report this case. Case Presentation: A 45-year-old female was admitted to the hospital with systemic joint pain, fatigue, multiple ulcers, and purulent discharge on both hands. Her myositis-specific antibody profile revealed positive anti-MDA5 and anti-SSA/RO52 antibodies. Treatment included a combination of glucocorticoids, immunosuppressants, gastric and liver protection, infection control, and wound care. After two weeks of treatment, the patient showed improvement in symptoms. However, on the 24th day of hospitalization, the wound at the right elbow joint ruptured and became infected, requiring debridement and skin grafting in the appropriate department. Conclusion: There has been limited research and reported cases of dermatomyositis with coexistence of positive anti-MDA5 and anti-SSA/RO52 antibodies combined with severe skin ulcers. Therefore, we present this rare case and emphasize the need for close follow-up on pulmonary involvement and skin ulcer progression, as well as timely implementation of new treatment strategies to actively improve the prognosis.
RESUMO
PH domain leucine-rich repeats protein phosphatase 1(PHLPP1) belongs to a novel family of Ser/Thr protein phosphatases: PHLPP serves as tumor suppressor in several cancers. However, little knowledge about the expression of PHLPP1 in human glioma tumor tissue and its role in inflammation response in glioma cells was known. Glioma samples were obtained from a total of 37 patients including 16 males and 21 females with surgical removal of the brain tumor. PHLPP1 protein and inflammatory cytokines were measured by Western blot analysis and immunohistochemistry while mRNA was determined by RT-PCR. The levels of inflammatory cytokines including TNF-α, IL-17, IL-1ß in U251 glioma cells were evaluated by siRNA PHLPP1 and PHLPP1 addition. The loss of PHLPP1 expression occurs at high frequency in human gliomas. The highest mean values of PHLPP1 mRNA and protein were found in non-glioma brain tissues whereas the lowest mean values were found in those in glioblastoma with an increase of TNF-α, IL-17, IL-1ß (p<0.05). PHLPP1 expression in human glioma was associated negatively with the severity of the tumor and inflammatory cytokines. siRNA PHLPP1 could increase the levels of inflammatory cytokines in U251 glioma cells while PHLPP1 addition could inhibit significantly inflammatory cytokines. We concluded that PHLPP1 played a suppression role in inflammatory response of glioma. The present study indicated that PHLPP1 could be used as a predictor for the prediction of the patients or as a therapeutic target for the treatment of human glioma.
