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linear IgA disease
Pathology:
- subepidermal blistering disorder
- tissue-bound & circulating IgA autoantibodies to the dermal-epidermal junction
- both the 120 kD linear IgA disease antigen & the 97 kD linear IgA disease antigen of COL17A1, represent major antigenic targets of autoantibodies in patients with linear IgA disease
- the IgA autoantibodies preferentially react with 97 kD & the 120 kD forms, but not with the full-length COL17A1, suggesting that the cleavage of the ectodomain generates novel autoantigenic epitopes
Laboratory:
- see ARUP consult [2]
General
vesiculobullous dermatitis
References
- UniProt :accession Q9UMD9
- ARUP Consult: Linear IgA Disease
The Physician's Guide to Laboratory Test Selection & Interpretation
https://www.arupconsult.com/content/linear-iga-disease
- Chaudhari S, Mobini N.
Linear IgA Bullous Dermatosis: A Rare Clinicopathologic Entity with
an Unusual Presentation.
J Clin Aesthet Dermatol. 2015 Oct;8(10):43-6.
PMID: 26557220 Free PMC Article
- Chen S, Mattei P, Fischer M, Gay JD, Milner SM, Price LA.
Linear IgA bullous dermatosis.
Eplasty. 2013 Jul 2;13:ic49. Print 2013.
PMID: 23882302 Free PMC Article
- Haeberle MT, James WD
Linear IgA Dermatosis
Medscape: (eMedicine)
https://emedicine.medscape.com/article/1063590-overview