The Analysis Of Clinical And Histopathological Features Of Sweet Syndrome

Part ? The clinical characteristics of Sweet syndrome Objective:To investigate the clinical and laboratory features,treatment and outcome of Sweet syndrome(SS)patients with and without hematologic malignancy to analyze the disease condition and to guide clinical diagnostic and treatment.Method:Collect the clinical data of the SS patient in our hospital from October 2010 to June2019,including clinical manifestation,laboratory examination [including blood routine examination,erythrocyte sedimentation rate(ESR),C-reactive protein(CRP),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: with or without hematologic malignancy.Results:In total,37 SS patients were included in this study(16 males and 21 females)with the average onset age of 53 years.Ten patients were classified as having malignancy-associated SS: nine with a hematologic malignancy including acute myeloid leukemia(AML,4/9,44%),myelodysplastic syndrome(MDS,4/9,44%),and multiple myeloma(MM,1/9,11%)and one with a solid tumor diagnosed as liver carcinoma.The mean hemoglobin and platelet levels were significantly lower in patients with hematologic malignancy than in those without(p = 0.007 and p = 0.013,respectively).Systemic corticosteroids were the most frequently used treatment(24/37,65%).In the group of SS with hematological malignancy,4 patients died(44%),while in the other group,the mortality was 14%.Conclusion:SS patients who have laboratory evidence of lower hemoglobin and platelet levels may have an increased risk of hematologic malignancy.Part ? The histopathological features of Sweet syndrome Objective:To elucidate the histopathological features of SS patients with and without hematologic malignancy and to evaluate its diagnostic value.Method:We respectively reviewed the medical records and histopathological manifestations of lesions from patients who diagnosed SS and underwent skin biopsy in our hospitalfrom October 2010 to June 2019.And the histopathological findings of skin lesions were independently read and diagnosed by two dermatopathologists.Results:All 37 SS patients underwent skin biopsy and were included in this study.In the respect of histopathological features,the main findings included a predominantly neutrophilic dermal infiltrate(37/37,100%),inflammatory cell infiltrate around the adnexa(19/37,51%),prominent dermal papillary edema(26/37,70%),acanthosis(20/37,54%)and collagen degeneration(16/37,43%).The cell infiltrate was usually diffuse(30/37,81%).Four cases had an infiltrate only in the superficial dermis(4/37,19%),51%(19/37)had involvement in the mid-dermis,and 38%(14/37)had involvement in the deep dermis,even the subcutis.Lymphocytes(10/37,27%),histiocytes(8/37,22%),eosinophils(4/37,11%),and plasmacytes(4/37,11%)were the other cells involved in the infiltrate.In the group of SS with hematologic malignancy,we observed leukemic cells in the dermis in one patient with SS and AML.Conclusion:The histopathological features of SS with and without hematologic malignancy were both shown a dense dermal neutrophilic infiltrate with edema.Therefore clinicians should not rely on histopathological findings only to assess whether SS patients have potential associated hematological malignancies.The presence of leukemic cells in the skin lesion is suggestive of a coexistence of leukemia cutis and SS.