| BackgroundNeutrophilic urticarial dermatosis(NUD)is a rare entity characterized by urticaria-like rashes,and it histopathologically belongs to neutrophilic dermatoses Since it was nosologically defined in 2009,NUD has gradually been accepted as an entity.However,there are still few reports and analyses of the disease.ObjectAnalyze the clinical features of NUD and improve the understanding of the disease,in order to assist in diagnosis and treatment of the diseaseMethodsThree cases with neutrophilic urticarial dermatosis admitted to the dermatology department of Qilu Hospital of Shandong University from January 2018 to December 2019 were reported.NUD cases reported since 2009 were searched in the databases of CNKI,PUBMED and Wanfang,and 52 cases with complete clinical datas were extracted for retrospective analysis.Summerize the epidemiology,associated diseases,clinical manifestations,ancillary testing and treatment of NUD.At the same time,we summarized the pathogenesis,clinical manifestations,histopathological alterations and the pharmacologic treatment of NUD.ResultsRepot of three cases1.General information:All three patients were female,24,30,72 years old respectively.One patient had urticaria-like lesions for the first time.The other two patients presented with recurrent urticaria-like lesions.The main clinical manifestations are red spots and wheals2.Clinical manifestations:The main clinical manifestation were erythema and wheal,1 patient suffered from noticeable pruritus,while the others were lack of itching or only had slight pruritus.Fever and fatigue were encountered in 2 patients.No joint pain occurred in 3 cases.3.Associated diseases:All of the three patients were with systemic inflammation,but without any underlying systemic condition.4.Histopathology:Histopathological examination has the following common features:? the epidermis is normal to near-normal;? a dense perivascular and interstitial infiltrate of neutrophils with leukocytoclasia;?There are no signs of vasculitis or dermal edema.5.Laboratory test:Two cases showed neutrophilia.ESR increased rapidly in 2 cases,and CRP increased in 3 patients.2 patients had increased serum Immunoglobulin E.6.Treatment:Three patients were all treated with glucocorticoids and antihistamines.One patient had urticarial eruption for the first time with a good response to glucocorticoid and antihistamines.The rash basically resolved without pigmentation,and symptoms were relieved.The other 2 patients had persistent rash,and rash was aggravated after the glucocorticoid was stopped.The erythema obviously cleared after the neutrophil migration inhibitor was added.The inflammation index including WBC,ESR and CRP decreased significantly or back to normalLiterature review and analyses52 cases of NUD patients reported in related literature during 2009 and 2019.1.Age and gender:22 cases are male patients,and 30 cases are female.The male and female ratio is 1:1.36.The incidence is approximately the same in each sex.The youngest patient is a newborn,the maximum age is 74 years old,the average age is 37,and the median age is 40 years old.NUD occurs with a high prevalence in adults between 20 and 50 years of age,rarely in children and the elderly.2.Associated diseases:Among 52 patients,20 cases were Schnitzler syndrome,19 cases were systemic lupus erythematosus,4 cases were adult-onset still disease,2 cases were Cryopyrin-associated periodic syndrome.There were 1 case each of systemic juvenile idiopathic arthritis,poststreptococcal rheumatic disease,and serum sickness-like drug reaction.And 4 cases were without any systemic diseases3.Clinical manifestations:33 out of 52 patients occurred fever of varying degree.24 cases had joint pain.Forty-one patients had non-itching or mild itching erythema,8 cases felt itching and 3 cases had stabbling pain.Peripheral lymphadenopathy occurred in 7 cases.4.Histopathology:52 cases has the following common features:a dense perivascular and interstitial infiltrate of neutrophils with leukocytoclasia without vasculitis or dermal edema.33 cases had significant nuclear dust,17 cases were not described,2 cases were not accompanied with nuclear dust.One case was observed mild vacuolar change of the basal layer(SLE patients).3 cases had a small amount of eosinophils in dermis.5.Auxiliary testing:The neutrophil count and rate of peripheral blood were increased in 14 patients.12 patients were found to be anaemic.3 patients had elevated serum ferritin;35 patients had elevated ESR/CRP;Antinuclear antibody was positive in 16 patients;11 patients had anti-Ro/SSA and anti-La/SSB antibodies;21 patients had monoclonal hypergammaglobulinemia(IgM or IgG),20 of them were Schnitzler's syndrome,the other one was witout any systemic diseases.4 SLE patients had polyclonal hypergammaglobulinemia.6.Therapy:The criteria for drug efficacy:complete or partial improvement in rash,systemic symptoms,and inflammatory indicators after drug use.16 patients were treated with antihistamines,with a response rate of 31%;Systematic application of glucocorticoids in 21 patients had a response rate of 43%;12 patients were treated with immunosuppressive drugs with a response rate of 25%;Combinations of steroids with immunosuppressive drugs were used,and the respond rate is 100%;4 patients were treated with dapsone or colchicine with a response rate of 50%;21 patients received the anakinra,and all of them achieved complete remission.In addition,even for patients who responded well to hormones,most of them have repeated or worsened conditions during the hormone reduction,while anakinra can achieve complete remission after treatment.Conclusion1.NUD is an entity which can occur at any age,especially in young and middle-aged people.Women had a slightly higher prevalence.2.The rash is characterized by urticaria like lesions which include rose to red macule or slightly elevated plaque or papule.Extra-cutaneous signs are very frequen,especially fever,fatigue or arthralgia.3.At histopathology,a dermal neutrophilic infiltrate with substantial interstitial spreading accompanied by leukocytoclasis,but no fibrinoid necrosis of vascular walls can be found.4.Some patients have no response to antihistamine therapy.Whether the application of glucocorticoids depends on the patient's systemic systemic diseases and NUD.Neutrophil migration inhibitors such as dapsone or colchicine and the IL-1 antagonist are the main drugs options for NUD5,NUD is associated with systemic diseases,including Schnitzler syndrome,SLE,AOSD and CAPS.Thus clinical attentions should be paid to screening for systemic diseases. |
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