Abstract:

Adult-onset Still’s disease （AOSD） is a rare autoinflammatory disease characterized by fever， rash， arthritis， liver， spleen and lymph node enlargement， increased total number of peripheral white blood cells and neutrophil ratio. This paper reported a case of AOSD with dermal lymphadenitis （DL） complicated with hemophagocytic syndrome （HPS）， in order to improve the clinicians’ understanding for this complicated complication. The patient was a 48-year-old female who was admitted to the hospital with the complant of “intermittent fever with rash for 15 d”. After 10 d of active anti-infection treatment， the symptoms were not improved， and there were new large congestive edematous erythema on the face and trunk， muscle pain in limbs and joints， and spleen enlargement. Laboratory tests showed increased white blood cell count， significantly decreased platelet count， hypofibrinogenemia， elevated serum ferritin， and elevated soluble interleukin-2 receptor sCD25； DL was pathologically diagnosed by axillary lymph node biopsy. After excluding other diseases， the diagnosis was confirmed as AOSD with DL complicated with HPS. After diagnosis of HPS， the patient was treated with hemophagocytic lymphohistiocytosis（HLH）- 1994 regimen combined with rucotinib for 6 weeks， and the symptoms were improved； the patrent was discharged. The diagnosis of AOSD is particularly complex when complicated with the complications such as HPS， which requires carefully differential diagnosis， especially to exclude lymphoma. The cases of AOSD with DL are rare， and its etiology and pathogenesis need further study； early diagnosis and multidisciplinary collaboration are essential to improve the patient’s prognosis.

Key words: Adult-onset Still’s disease, Dermatopathic lymphadenitis, Hemophagocytic syndrome, Fever, Rash, Lymphadenectasis