The Analysis Of The Clinical And Pathological Features Of Adult Onset Still’s Disease

Adult-onset Still’s disease(AOSD)is a rare multi-systematic inflammatory disease with unknown etiology,which is classically characterized by intermittent high spiking fever,sore throat,evanescent rash,arthralgia/arthritis,lymphadenopathy and/or hepatosplenomegaly,leukocytosis with neutrophilia,hyper-ferritin,liver dysfunction,elevated erythrocyte sedimentation rate(ESR),elevated C-reactive protein(CRP)and negative antinuclear antibody(ANA)and rheumatoid factor(RF).Typical eruption is evanescent,salmon-like maculopapular,which occurs at the same time as fever and fades away as fever subsides.It was reported to appear in up to 87%of the patients with AOSD during the course of disease.And evanescent rash is included as one of the major diagnostic criteria of AOSD(Yamaguchi criteria).However,another atypical rash that differs from the classical eruptions has been highlighted in the recent literature,which clinically manifested as persistent pruritic eruptions(PPEs).They are extremely different from classic evanescent rash in clinical manifestations and histopathology.PPEs clinically manifested with persistent squamous erythema,papules or plaques with much itching on the trunk and extremities,with no significant correlation with fever.Histologically,PPEs show peculiar,distinctive distribution dyskeratosis in the upper epidermis and perivascular inflammatory cells infiltration in the superficial dermis.However,the clinical significance of PPEs needs further study.It has been reported that it is a mark of severe complications or malignant tumors in AOSD.At present,among all the AOSD cases diagnosed in our hospital,there were not only patients with evanescent rashes but also with PPEs.With the purpose of comparing the clinical,laboratory features including pathological features of AOSD patients with different types of lesions,we demonstrated a retrospective study.Part I The clinical characteristics of adult onset Still’s diseaseObjective:Investigating the clinical and laboratory features of AOSD patients with different types of cutaneous manifestations to analyze the disease condition and to guide clinical diagnostic and treatment.Method:Collect the clinical data of the 52 AOSD patient with lesions who were inpatient diagnosed the first time in our hospital from November 2015 to November 2017,including clinical manifestation,laboratory examination(including blood routine examination,liver function,coagulation function,ESR,CRP,serum ferritin,autoantibody series and infection markers),imaging examination(including chest CT and type-B ultrasonic)and other laboratory examinations such as bone marrow biopsy histopathology and lymph node biopsy histopathology.All these patients were divided into two groups according to the different type of lesions.One group is classic evanescent rash group,and the other is PPEs group.Result:In total,52 AOSD patients with cutaneous marnifestation were included in this study.There were 25 patients(25/52,48.1%)in the group of classic evanescent rash whose average onset age is 3 3± 10 years old,including 7 males and 18 females.There were 27(27/52,51.9%)patients in the group of PPEs whose average onset age is 49± 14 years old,including 5 males and 22 females;To make comparison between two groups,we found that the onset age of patients with PPEs were much higher than those with evanescent eruptions.The difference is statistically significant(p<0.0001).As for the laboratory examination,the levels of serum ferritin and D-dimer were significantly higher in the group of PPEs than those of group of evanescent rashes(p<0.05).In the group of PPEs,there were 2 cases(2/27,7.4%)of reactive hemophagocytic activation syndrome(RHS)who died in the end and 2 cases of lymphoma,one of which initially presented as RHS.There were no serious complications or death case in the group of evanescent rashes.Conclusion:AOSD patients with PPEs tend to be older.The serum ferritin and D-dimer levels of patients with PPEs were significantly higher than those of patients with evanescent rashes.And the proportion of patients with severe complications or malignant tumors was higher.Therefore,clinicians should be reminded that AOSD patients with PPEs should be treated more aggressively and followed up more closely.Partn Ⅱ The pathological features of lesions in AOSD patientsObjective:To elucidate the histopathological features of lesions of AOSD patients and to evaluate its diagnostic value.Method:The clinical data and histopathological manifestations of lesions from patients who first diagnosed AOSD and underwent skin biopsy in our hospital from November 2015 to November 2017 were retrospectively analyzed.And the histopathological findings of skin lesions were independently read and diagnosed by two dermatopathologists.Result:There were 3 out of 25 AOSD patients with evanescent rash and 15 out of 27 AOSD patients with PPEs performed skin biopsy.A total of 18 cases of AOSD patients were included in the histopathological study of the skin lesions.The histopathological features of the lesions of 3 AOSD patients with evanescent rash were no obvious abnormality in epidermis and sparse inflammatory cell infiltration in the superficial dermis(3/3,100%).In the histologic specimens of 15 AOSD patients with PPEs,singly or aggregated dyskeratotic/necrotic keratinocytes in the upper epidermis and perivascular interstitial inflammatory cells infiltration in the superficial dermis were observed(15/15,100%).There were interface changes,basal cell vacuolar degeneration,in 2 cases(2/15,13.3%).The inflammatory infiltrate was composed mainly of lymphocytes in 14 cases with sparse neutrophils infiltration(14/15,93.3%).Of the 14 cases,there were eosinophil infiltration in the upper dermis in 2 cases.And inflammatory infiltration was composed predominantly of neutrophils in 1 case with sparse lymphocytes and eosinophils(1/15,6.7%).And there were appearances of melanophages in the superficial dermis in 6 pathological sections(6/15,40%).Conclusion:The histological features of AOSD patients with evanescent rash is nonspecific,while the histology of PPEs has special pathological changes.There was presentation of singly or aggregated dyskeratotic/necrotic keratinocytes in the upper layers of the epidermis and perivascular and also interstitial inflammatory infiltrate in the upper and mid-dermis.And the inflammatory cell are mainly neutrophils with sparse lymphocytes and eosinophil.And scattered melanophages appeared in the superficial dermis.These features can be used as the diagnostic basis for histopathology of AOSD with PPEs.It is recommended that PPEs be included in the diagnostic criteria of AOSD.