Unlabelled: Several small blisters were noticed on the forehead and the trunk of a newborn boy on day 1. The blisters gradually enlarged and spread over the whole body including the oral mucosa. A skin biopsy was performed twice and subepidermal bullae with polymorphonuclear and mononuclear cell infiltration were demonstrated. Direct immunofluorescence showed linear IgA, IgG and C3 depositions along the basement membrane zone and this finding led to a diagnosis of linear IgA bullous dermatosis. So far, internationally, only one case has ever been reported on the disease at neonatal onset. The skin lesions spontaneously regressed and the mucosal lesions were controlled with diaminodiphenylsulfone.

Conclusion: In neonates with prolonged blistering, autoimmune disease such as linear IgA bullous dermatosis should be considered within the differential diagnosis and an immunofluorescence study must be performed.

Download full-text PDF

Source

Publication Analysis

Top Keywords

linear iga
16
iga bullous
12
bullous dermatosis
12
neonatal onset
8
linear
4
dermatosis neonatal
4
onset case
4
case report
4
report review
4
review literature
4

Similar Publications

Linear IgA bullous dermatosis (LABD) is a rare subepidermal blistering disorder characterized by the presence of linear IgA deposits at the basement membrane zone (BMZ) by direct immunofluorescence (DIF). This entity was first described by Chorzelski and Jablonska from Warsaw Center of Bullous Diseases, Poland. The disease affects children and adults, whereby they differ in terms of clinical picture and course.

View Article and Find Full Text PDF

Case report: a rare concurrence of dense deposit disease in an adolescent patient with IgA nephropathy.

BMC Pediatr

January 2025

Department of Traditional Chinese Medicine, Shengli Clinical Medical College of Fujian Medical University, Fujian Provincial Hospital, Fuzhou, China.

Background: Dense deposit disease (DDD) is a rare renal disorder major affecting adolescents and children, characterized by an absence of distinctive clinical symptoms. Its coexistence with other renal conditions complicates both diagnosis and treatment in clinical practice.

Case Presentation: We described a 15-year-old male adolescent presenting with nephrotic syndrome as the initial manifestation, with urinalysis indicating significantly elevated protein and erythrocytes.

View Article and Find Full Text PDF

Purpose: This study seeks to investigate the fundamental molecular processes through which histone deacetylase 9 (HDAC9) governs the proliferation of glomerular mesangial cells in the context of immunoglobulin A nephropathy (IgAN) and to identify novel targets for clinical research on IgAN.

Methods: Data from high-throughput RNA sequencing for IgAN were procured from the Gene Expression Omnibus database to assess the expression profiles and clinical diagnostic significance of histone deacetylase family proteins (HDACs). Blood samples from 20 IgAN patients were employed in RT-qPCR analysis, and the spearman linear regression method was utilized to analyze the clinical correlation.

View Article and Find Full Text PDF

Background And Objectives: Gut microbial symbionts have been shown to influence the development of autoimmunity in multiple sclerosis (MS). Emerging research points to an important relationship between the microbial-IgA interface and MS pathophysiology. IgA-secreting B cells are observed in the MS brain, and shifts in gut bacteria-IgA binding have been described in some patients with MS.

View Article and Find Full Text PDF

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!