| Cutaneous rosai-dorfman disease(CRDD)is a rare benign histiocytosis.It was considered as a distinct entity,based on the exclusive involvement of the skin,different demographic features and better prognosis,compared with systemic rosai-dorfman disease.The pathogenesis was still unclear,and was considered to be related to IgG4-related disease by some authors.This study primarily summarized the demographic features,clinical manifestation,histopathological features,treatment and prognosis of 75 CRDDs diagnosed by our institution in recent years,and analyzed their clinical and histological characteristics,in order to strengthen the understanding of this rare disease.We further detected the expression of IgG4+ and IgG+ plasma cells in CRDD and investigated its relationship with IgG4-related disease.Part 1 Clinical and histopathological features analyses of cutaneous rosai-dorfman diseaseObjective To analyze the clinical and histopathological features of cutaneous rosai-dorfman disease(CRDD),and provide reference for future clinical work.Methods Totally,75 patients with CRDD diagnosed by the Institute of Dermatology,Chinese Academy of Medical Sciences from 2004 to 2018 were enrolled.The clinical data,treatment and prognosis were retrospectively reviewed and analyzed.Furthermore,the histopathological and histochemical features of 44 patients who performed skin biopsy in our institution were collected and analyzed.Results The male-to-female ratio was 1.14:1 in the 75 patients.The average age at presentation was 44.1 years(standard deviation,11.5 years)with a mean disease duration of 8.6 months.Most cases have single(42.67%)lesion or multiple(>5)(32.00%)lesions.Lesions of 52 patients mainly presented as dark-red to brown papuloplaques or noduloplaques with irregular surface,and around satellite papules or nodules.There were 18 cases presented as isolated red nodule imitating inflammatory pseudotumor and 4 cases presented as multiple acneifonn papules.The surface of the lesions were intact except for one case in which surface ulceration was observed.The commonest involved site was face(58.67%),with cheek is the commonest site,followed by trunk and limbs.The lesions ranged from 5 to 150 millimeter in size,and generally asymptomatic(84.21%).Histopathologically,the lesions were mainly located in dennal,with 36 cases involved subcutaneous tissue.All of the 44 lesions showed varying numbers of proliferative polygonal histiocytic cells,lymphocytes,plasma cells,and neutrophils infiltration forming a diffuse(68.18%),nodular(18.18%),or focal(13.64%)infiltrate pattern.The RD cells had abundant granular and palely eosinophilic cytoplasm,and the most important feature was phagocytosis of intact lymphocytes,plasma cells,and neutrophils within the cytoplasm,a process termed as emperipolesis.Large polygonal RD cells were found within dilated vascular spaces in 4 cases.None of the lesions shown cellular pleomorphism with nuclear atypia and mitoses.Patch or focal infiltrate of numerous plasma cells was a constant feature in all cases.However,mainly lymphocytes infiltration were found in 8 cases.Germinal center was discovered in 3 lesions.In addition,neutrophils existed in all cases with varying degrees in which 13 lesions forming microabscess.33 lesions showed various degrees of fibrosis,with 3 lesions appearing as storifonn pattern.Follow-up course of 66 patients ranged from 2 to 140 months displayed that 14 patients received surgical resection,and one had recurrence at the original site after 20 months.There were 7 patients without any treatment,of which 3 cases were spontaneous regression with a mean of 5.3 months.The remaining 45 patients received various therapies including use of systemic corticosteroids,hydroxychloroquine,methotrexate or intralesional injection corticosteroids,local radiation and so on,with a combined remission rate of 97.78%,and complete remission was achieved in 22 patients after 2 to 22 months(mean 8.7 months)follow-up.However,7 patients experienced recurrence and one patients experienced persistence irrespective of treatment.None patients were found with extracutaneous involvement during the diagnosis and follow-up.Conclusions CRDD is rare.The clinical manifestation varied,and the existence of clusters of papules or nodules with satellite lesions is a diagnostic hint.Recognition of the typical histiocytes with emperipolesis and stains for S-100 protein,combined with numerous plasma cells may allow a correct diagnosis.The prognosis is good,and should be differentiated from varieties of benign or malignant diseases.Part 2 The expression of IgG4 in cutaneous rosai-dorfman disease tissueObjective To measure the expression of IgG4 and IgG positive plasma cells in the tissue of cutaneous rosai-dorfman disease(CRDD),and to explore its possible relationship with IgG4-related disease(IgG4-RD).Methods Formalin fixed paraffin embedded tissue samples of 44 patients with CRDD who performed skin biopsy in the Institute of Dermatology,Chinese Academy of Medical Sciences from 2009 to 2018 were collected,and immunostained with anti-IgG4 and anti-IgG antibodies.And the number of IgG4 and IgG positive plasma cells with the highest density per high-power field(HPF)and their ratios were counted.Results Various degrees of IgG4 and IgG positive plasma cells were found in all 44 cases.IgG4 positive plasma cells ranged from 2 to 131/HPF(mean 34/HPF),and IgG positive plasma cells ranged from 28 to 359/HPF(mean 151/HPF).None lesions had more than 200 IgG4 positive cells/HPF.The IgG4/IgG cells ratio ranged from 2.8%to 73.1%(mean 23.6%),and it more than 40%was found in only five cases.Conclusions There were various degrees of fibrosis and IgG4 positive plasma cells in different tissue of CRDD,yet cannot be diagnosed as IgG4-RD based on the combination of its clinical and histological changes.The possible role of IgG4 played in the pathogenesis of cutaneous rosai-dorfinan disease should be further explored. |
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