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Sweet syndrome (SS), or acute febrile neutrophilic dermatosis, [1] [2] is a skin disease characterized by the sudden onset of fever, an elevated white blood cell count, and tender, red, well-demarcated papules and plaques that show dense infiltrates by neutrophil granulocytes on histologic examination.
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Reactive neutrophilic dermatoses are a spectrum of conditions mediated by neutrophils, and typically associated with underlying diseases, such as inflammatory bowel disease and hematologic malignancy.
Fever and skin conditions—particularly rashes resembling those seen in Sweet syndrome—are common signs. Other autoinflammatory conditions that can occur in individuals with VEXAS syndrome include periorbital angioedema, uveitis and scleritis, relapsing polychondritis, and polyarteritis nodosa. Inflammation may also affect the lungs. [7] [8]
Evans syndrome is an autoimmune disease in which an individual's immune system attacks their own red blood cells and platelets, the syndrome can include immune neutropenia. [ 1 ] [ 2 ] These immune cytopenias may occur simultaneously or sequentially.
This syndrome caused every subsequent generation in a bloodline to have their life expectancy shortened. When the amber lead concentration in the body is fatally high, white blotches appear on the victim's skin, their hair becomes white, and they feel intense chronic pain. The only known survivor of this syndrome is Trafalgar D. Water Law.
The median time interval between the onset of symptoms and the diagnosis was 6 years, with a range of 26 days to 14 years. This suggests that the symptoms of MAGIC syndrome may manifest relatively long after the initial onset of symptoms. During the course of MAGIC syndrome, the signs and symptoms of BD may typically occur before those of RP. [4]
Airedale NHS Foundation Trust, established 1 November 1991 as Airedale NHS Trust, [2] authorised as a foundation trust on 1 June 2010. [3]Alder Hey Children's NHS Foundation Trust, established 21 December 1990 as Royal Liverpool Children's Hospital and Community Services NHS Trust, [4] changed its name to The Royal Liverpool Children's National Health Service Trust on 15 March 1996, [5 ...