Linear IgA bullous dermatosis (LABD), also known as linear IgA disease, is a rare, idiopathic or drug-induced autoimmune blistering disease described by the linear deposition of IgA at the dermoepidermal junction. In spite of the clinical manifestation of this disorder is difficult to diagnose from dermatitis herpetiformis, the different immunopathologic findings in LABD and the lack of an associated gluten-sensitive enteropathy approbate the status of LABD as a separate disease.
Panel test:
● Immunopathology:
o DIF shows linear (rather than granular) IgA deposition along the basement membrane, and a 97kDa antigen (LAD-1) has been detected in most cases.
● Autoantibodies:
– Antibody against to a domain of the BPAg2 antigen (180kDa).
References:
1. Fortuna G, Marinkovich MP. Linear immunoglobulin A bullous dermatosis. Clin Dermatol 2012; 30:38.
2. Mintz EM, Morel KD. Clinical features, diagnosis, and pathogenesis of chronic bullous disease of childhood. Dermatol Clin 2011; 29:459.
3. Gluth MB, Witman PM, Thompson DM. Upper aerodigestive tract complications in a neonate with linear IgA bullous dermatosis. Int J Pediatr Otorhinolaryngol 2004; 68:965.
4. Zhao CY, Chiang YZ, Murrell DF. Neonatal Autoimmune Blistering Disease: A Systematic Review. Pediatr Dermatol 2016; 33:367.
