| BackgroundAs a rare chronic acquired autoimmune subepidermal bullous dermatosis,linear IgA bullous dermatosis(LABD)can be the classification basis of the children LABD and adults LABD complying to the age of onset,which has a pleomorphism of lesions,characterized by circular or semicyclic lesions with blisters,similar to the string of pearls.The histopathology of LABD is not peculiar,and the immunopathogenesis shows a linear deposition of IgA in the basement membrane.Some mistakes such as missed diagnosis and misdiagnosis are frequently raised in LABD clinically,due to the rare clinical disease,pleomorphic skin manifestation,distinguishing from many kinds of diseases,clinician’s insufficient recognition on LABD and other factors,which usually results in delay or the improper treatment.ObjectiveTo provide the references to the clinician,the clinical manifestations,histopathology,immunopathology,misdiagnosis,treatment and prognosis of LABD were analyzed,which was conducive to the early identification and diagnosis,so as to treat timely,improve the prognosis and reduce misdiagnosis rate.Objects and Methods25 cases of LABD diagnosed and treated in Dermatology & STD,First Affiliated Hospital of Zhengzhou University from January 2011 to March 2018 were collected,and the patients who were included should meet the clinical and pathological diagnostic criteria for LABD.Data of age,gender,skin performance,histopathological results,immunofluorescence results,misdiagnosis and causes,treatment and prognosis were collected and analyzed.Results1.Age: in the children group,the youngest was 1 year and 10 months,and the oldest was 12 years old.There existed 7 cases of patients less than 5 years old and 9 cases of 5~10 years old.In adult group,the minimum age was 31 years,and the maximum age was 62 years old,2 cases of patients less than 40 years old,4 cases of patients aged from 40 to 60 years old,4 cases of patients over 60 years old,and 1 case of patients over 60 years old.The data clearly indicated that the number of children was significantly higher than that of adults.2.Gender: There were 13 males and 12 females among the 25 patients with LABD,male patients were more than female patients with the ratio of 1.08: 1.Among them,18 cases were involved in the children group,including 10 cases of boys and 8 cases of girls(ratio of male to female was 1.25).Moreover,there were 7 adult patients,including 4 female and 3 male(ratio of male to female was 0.75).The results implied that there was no significant difference in gender distribution between adult group and children group(P<0.155).3.Location and characteristics of skin lesions: the lesions in the children group manifested the circumferential or multi-ring edematous erythema and papules in the head,torso,limbs and perineum,whose boundary was surrounded by blisters,the blister wall was tense,the blister fluid was clear,and the Nikolsky sign was negative,that is,the skin damage like "string of pearls",with various degree of itching.In adult group,most of the lesions involved trunk,and the lesions were erythema,papules and blisters.Blisters of varying sizes occurred on normal skin or erythema with clear blister fluid and negative Nikolsky sign,with mild itching.Ring lesions with blisters were less common in adult than in children.4.Histopathology: histopathological examination was performed in 25 patients,whose results showed that the subepidermal blister was mainly the neutrophil infiltration with eosinophil infiltration.Histopathology was nonspecific,similar to herpes dermatitis and bullous pemphigoid.5.Immunopathogenesis: direct immunofluorescence examination was performed in 25 patients,with the results showed that linear IgA was deposited in the basement membrane zone.6.Misdiagnosis: 20 of 25 patients were misdiagnosed,with the misdiagnosis rate of 80%.Among them,6 patients in children group were misdiagnosed as herpetiformis dermatitis,3 cases were misdiagnosed as papular rash,1 case was misdiagnosed as impetigo,1 case was misdiagnosed as erythema multiforme,and 3 cases were misdiagnosed as eczema.2 cases of adult group were misdiagnosed as bullous goid,2 cases were misdiagnosed as eczema,1 case was misdiagnosed as herpes-like dermatitis,and 1 case was misdiagnosed as cutaneous vasculitis.7.Treatment: in this study,25 patients with mild condition and only skin damage were given glucocorticoid,while those in severe condition were given glucocorticoid for the whole body.The dosage of glucocorticoid should be 0.5-1 mg/kg/d,with the treatment of anti-inflammatory,anti-sensitive,anti-itching,and antiinfective therapy.In the children group,the average treatment time was 14 days(10<X<16,S=14±1.0),and the course of disease was 2.2 years(1<X<5,S=2.2±0.8).In adult group,the average treatment time was 17 days(12<X<21,S=17±1.3),and the course of disease was 3.1 years(1<X<5,S=3.11±0.6).The results indicated that there was no significant difference in the average treatment time between the children group and the adult group(p=0.016)and the course of disease in the adult group was more than that in the child group(p=0.073).8.Prognosis: 25 patients was generally better after treatment,with no new skin lesions during medication.The blisters on the head and trunk,crusts dried up,some crusts had fallen off,and the area of erythema was obviously reduced and the color was lighter,without any obvious symptoms.The clinical lab indices of blood routine,urine routine,liver function,renal function and electrolyte were not abnormal.During the follow-up period,the patients had a great life quality with slight adverse reactions.Conclusion1.Linear IgA bullous dermatosis is rare clinically and easy to be missed and misdiagnosed.Therefore,it is necessary to improve the recognition of linear IgA bullous disease by clinicians.2.Direct immunofluorescence shows that linear IgA deposited in the basement membrane is an important basis for the diagnosis and differential diagnosis of linear IgA bullous dermatosis. |
PDF Full Text Request