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Progress is tracked with meaningful outcomes, such as improved standing tolerance, longer walking distance, fewer night flares, and better balance on stairs
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In summary, in DRESS, T cells in the skin and blood demonstrate non-clonal proliferation, distinct expression of chemokine receptors, co-stimulatory molecules, and positive gene regulation in the JAK-STAT signaling pathway.28,29 Clinical manifestations DRESS syndrome is characterized by the gradual involvement of multiple organs, which may include skin, hematological systems, and solid organs.19 Clinical manifestations are usually perceived two weeks to two months after starting a medication.22 DRESS begins with prodromes of flu-like malaise, pharyngitis, fever, and lymphadenopathy.30 The evolution can be slow and with the association of several symptoms, with fever (≥ 38.5 °C) occurring in 75%‒100% of patients and generally preceding the rash by several days.15,16,19 Antibiotics or radiocontrast media can trigger the reaction within approximately 14 days of exposure, while antiepileptics and allopurinol tend to have longer latency periods and may be related to more severe forms of the disease.26,31 Skin manifestations Although the dermatological manifestations are varied and affect more than 50% of the body surface, the most characteristic lesions in the initial phase are periorbital and facial edema (76% of cases) with micropustules.15,17,26,32,33 Maculopapular exanthema is the most common clinical sign, presenting in 99%‒100% of patients, sparing the palms and soles, with craniocaudal progression (Fig
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