Linear IgA Bullous Dermatosis In A Neonate:a Case Report And Literature Review

BackgroundLinear IgA bullous dermatosis(LABD)is a chronic acquired,self-limited autoimmune bullous disease(AIBD)characterized by linear deposition of IgA in the basement membrane zone(BMZ).The etiology of LABD is unknown,most of which are idiopathic.Others may be related to drugs,infections,autoimmune diseases,tumors,physical and chemical factors,etc.And the exact pathogenesis is still unclear.LABD is more common in preschool children and the elderly over 60 years old,and can be divided into child type and adult type according to the age of onset.Child type of LABD has a rapid onset,and the face and perineum are easily affected.It gradually affects the trunk,limbs and other parts.The typical clinical manifestations are blisters arranged in a sausage-like ring on the basis of normal skin or erythema.However,in adult patients with LABD,the lesions are pleomorphic and mucosal damages are common.The pathological changes of this disease are manifested as subepidermal blisters,but they lack specificity.Direct immunofluorescence(DIF)examination shows homogeneous linear IgA deposition along the basement membrane zone,which is the main basis for the diagnosis of LABD.In the treatment of LABD,different treatment methods should be selected according to the severity of the patients’ condition,and excessive treatments ought to be avoided.Newborn refers to a baby that is less than 28 days after the birth of the fetus from the mother.LABD cases are not very common in children and adults,and the disease is even rarer in newborns.So far,there are only a few reports in foreign literature,and there is no report of neonatal LABD in domestic literature.ObjectivesThis article aims to report a clinical case of neonatal linear IgA bullous dermatosis,and to retrospectively analyze the case data of literature published at home and abroad to discuss its epidemiology,clinical features,pathological manifestations,treatments and prognosis,so as to improve the understanding of neonatal LABD and avoid missed diagnosis and misdiagnosis.Methods1.The clinical manifestations,laboratory examinations,histopathological and immunopathological data of a case of neonatal LABD diagnosed in our hospital were reported and analyzed.2.Chinese literature search:search for Chinese case reports on neonatal LABD published in Chinese databases such as CNKI,Wanfang database and VIP database(search interval:January 1,1970 to December 31,2020).3.English literature search:English case reports about neonatal LABD were searched in English databases such as PubMed,GeenMedical and Google Academic.The retrieval time is from January 1,1970 to December 31,2020.4.Inclusion criteria:The clinical data were complete,and the case reports literature were diagnosed as neonatal LABD(onset age≤28 days).Exclusion criteria:①The age of onset was over 28 days;②Documents in which the basic information of patients were not clearly recorded.5.Summarize the domestic and foreign literature reports in neonatal LABD,and discuss the general conditions,clinical performances,laboratory examinations,disease course,treatments and prognosis of the patients.SPSS22.0 was used for statistical analysis.Results1.The case of LABD in a neonate we reported had the following characteristics:The patient was a 10-day-old boy,who had systemic erythema and blisters for 6 days.Erythema and tension blisters of varying sizes were scattered or fused all over the body,and some blisters were ulcerated and eroded.The oral mucosa had blood blisters and erosions,with red erosions visible.There was no obvious abnormality in the examination of each system.Histopathological biopsy showed subepidermal blisters containing neutrophils and a small amount of eosinophils;direct immunofluorescence examination showed homogeneous linear IgA deposition along the basement membrane zone.Serum anti-BP180 antibody and anti-BP230 antibody were negative.Neonatal LABD was diagnosed.We gave the patient mupirocin ointment for external use twice a day,and strengthen skin care and wound protection.His skin blisters disappeared completely without any new skin lesions.However,his mucosal lesions gradually aggravated;and the mucosal injuries were relieved after we added antibiotics.Currently,the patient is still in follow-up.2.A comprehensive analysis of the clinical data of neonatal LABD reported in the literature shows that there were 11 cases of neonatal LABD reported in the past 50 years(all reported in foreign literature),combined with 1 case reported in our hospital,a total of 12 cases.The characteristics of neonatal LABD were summarized as follows:(1)Gender ratio:There were 10 males and 2 females,and the ratio of male to female was 5:1.(2)Age:The onset age was from delivery to 10 days after birth,with the median age of 3 days.(3)Status of the mothers:Among the 12 cases of neonatal LABD,all the mothers of the infants had no history of LABD.The mothers of 3 cases had a history of infection and medication during pregnancy or delivery.(4)Location of neonatal LABD:more common in the face and perineum,and then gradually developed to the head and neck,trunk and limbs,etc.(5)Characteristics of skin lesions:The typical clinical manifestations of LABD are blisters arranged in a ring on the basis of normal skin or erythema,which are like"string of pearls".(6)Mucosal involvements:11 of the 12 cases of neonatal LABD had mucosal involvements of varying degrees,and the eyes,oral cavity,and airway mucosas were easily affected.(7)Course of illness:ranged from 7 days to 11 months.The course of disease in most patients is within 2 months.(8)Histopathology and immunopathology of LABD:Histopathological manifestations were subepidermal blisters,and direct immunofluorescence examination showed homogeneous linear IgA deposition along the basement membrane zone,which could be confirmed.(9)Treatments:Mild patients can be controlled by topical glucocorticoid emulsion,and patients with severe symptoms and mucosal involvements need systematic application of glucocorticoid,dapsone,immunoglobulin,etc.(10)Prognosis:LABD is a self-limited disease.Mucosal lesions often leaves different degrees of damage,and severe cases can lead to death.Conclusions1.Neonatal LABD is extremely rare in clinical practice,which is prone to be missed and misdiagnosed.Therefore,it is necessary to improve clinicians’understanding of this disease.2.In addition to skin damages in neonatal LABD,mucosa is also frequently involved,and the care of mucosa should be strengthened.3.LABD is a self-limited disease,and excessive treatments should be avoided.