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Linear IgA deposits along the epidermal basement membrane zone

MedGen UID:
1614816
•Concept ID:
C4531057
•
Anatomical Abnormality
HPO: HP:0031539

Definition

Presence of IgA antibodies in the dermoepidermal junction that are distributed in a linear pattern. This feature can be appreciated by immunofluorescence microscopy. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVLinear IgA deposits along the epidermal basement membrane zone

Recent clinical studies

Etiology

A subepidermal blistering dermatosis associated with coexistent IgG and IgA anti-dermal basement membrane zone antibodies; demonstration of IgG antibodies reactive against a 200-kDa dermal antigen.
Watanabe M, Tsunoda T, Tagami H
Eur J Dermatol 2002 Nov-Dec;12(6):603-6. PMID: 12459541
IgA-mediated epidermolysis bullosa acquisita: two cases and review of the literature.
Vodegel RM, de Jong MC, Pas HH, Jonkman MF
J Am Acad Dermatol 2002 Dec;47(6):919-25. doi: 10.1067/mjd.2002.125079. PMID: 12451379
IgA antibodies in chronic bullous disease of childhood react with 97 kDa basement membrane zone protein.
Zone JJ, Taylor TB, Kadunce DP, Chorzelski TP, Schachner LA, Huff JC, Meyer LJ, Petersen MJ
J Invest Dermatol 1996 Jun;106(6):1277-80. doi: 10.1111/1523-1747.ep12348993. PMID: 8752670

Diagnosis

A Case of Anti-BP180-type Mucous Membrane Pemphigoid with IgG and IgA Autoantibodies Showing Distinct Reactivities.
Minakawa S, Matsuzaki Y, Hashimoto T, Ishii N, Nishie W, Nakazawa M, Sawamura D
Acta Dermatovenerol Croat 2022 Jul;30(1):54-56. PMID: 36153720
Trimethoprim-sulfamethoxazole-induced linear IgA bullous disease presenting as toxic epidermal necrolysis.
Baltazard T, Dhaille F, Duvert-Lehembre S, Lok C, Chaby G
Dermatol Online J 2017 Aug 15;23(8) PMID: 29469749
A toxic epidermal necrolysis-like presentation of linear IgA bullous dermatosis treated with dapsone.
Nguyen JK, Koshelev MV, Gill BJ, Boulavsky J, Diwan AH, Dao H Jr
Dermatol Online J 2017 Aug 15;23(8) PMID: 29469740
Vancomycin-associated linear IgA disease mimicking toxic epidermal necrolysis.
Pereira AR, Moura LH, Pinheiro JR, Pasin VP, Enokihara MM, Porro AM
An Bras Dermatol 2016 Sep-Oct;91(5 suppl 1):35-38. doi: 10.1590/abd1806-4841.20164665. PMID: 28300888Free PMC Article
IgA-mediated epidermolysis bullosa acquisita: two cases and review of the literature.
Vodegel RM, de Jong MC, Pas HH, Jonkman MF
J Am Acad Dermatol 2002 Dec;47(6):919-25. doi: 10.1067/mjd.2002.125079. PMID: 12451379

Therapy

Trimethoprim-sulfamethoxazole-induced linear IgA bullous disease presenting as toxic epidermal necrolysis.
Baltazard T, Dhaille F, Duvert-Lehembre S, Lok C, Chaby G
Dermatol Online J 2017 Aug 15;23(8) PMID: 29469749
A toxic epidermal necrolysis-like presentation of linear IgA bullous dermatosis treated with dapsone.
Nguyen JK, Koshelev MV, Gill BJ, Boulavsky J, Diwan AH, Dao H Jr
Dermatol Online J 2017 Aug 15;23(8) PMID: 29469740
Vancomycin-associated linear IgA disease mimicking toxic epidermal necrolysis.
Pereira AR, Moura LH, Pinheiro JR, Pasin VP, Enokihara MM, Porro AM
An Bras Dermatol 2016 Sep-Oct;91(5 suppl 1):35-38. doi: 10.1590/abd1806-4841.20164665. PMID: 28300888Free PMC Article
Drug-associated linear IgA disease mimicking toxic epidermal necrolysis.
Khan I, Hughes R, Curran S, Marren P
Clin Exp Dermatol 2009 Aug;34(6):715-7. Epub 2008 Dec 2 doi: 10.1111/j.1365-2230.2008.03011.x. PMID: 19077099
IgA-mediated epidermolysis bullosa acquisita: two cases and review of the literature.
Vodegel RM, de Jong MC, Pas HH, Jonkman MF
J Am Acad Dermatol 2002 Dec;47(6):919-25. doi: 10.1067/mjd.2002.125079. PMID: 12451379

Prognosis

Anti-BP180-type mucous membrane pemphigoid: report of two cases.
Wada M, Sato J, Shindoh M, Ujiie H, Natsuga K, Nishie W, Shimizu H, Kitagawa Y
Odontology 2016 Jan;104(1):114-8. Epub 2014 Oct 30 doi: 10.1007/s10266-014-0181-2. PMID: 25355555
Drug-associated linear IgA disease mimicking toxic epidermal necrolysis.
Khan I, Hughes R, Curran S, Marren P
Clin Exp Dermatol 2009 Aug;34(6):715-7. Epub 2008 Dec 2 doi: 10.1111/j.1365-2230.2008.03011.x. PMID: 19077099
A subepidermal blistering dermatosis associated with coexistent IgG and IgA anti-dermal basement membrane zone antibodies; demonstration of IgG antibodies reactive against a 200-kDa dermal antigen.
Watanabe M, Tsunoda T, Tagami H
Eur J Dermatol 2002 Nov-Dec;12(6):603-6. PMID: 12459541
IgA-mediated epidermolysis bullosa acquisita: two cases and review of the literature.
Vodegel RM, de Jong MC, Pas HH, Jonkman MF
J Am Acad Dermatol 2002 Dec;47(6):919-25. doi: 10.1067/mjd.2002.125079. PMID: 12451379

Clinical prediction guides

A Case of Anti-BP180-type Mucous Membrane Pemphigoid with IgG and IgA Autoantibodies Showing Distinct Reactivities.
Minakawa S, Matsuzaki Y, Hashimoto T, Ishii N, Nishie W, Nakazawa M, Sawamura D
Acta Dermatovenerol Croat 2022 Jul;30(1):54-56. PMID: 36153720
Leukocytoclastic vasculitis in association with linear epidermal basement membrane zone immunoglobulin deposition: Linear vasculitis.
Magro CM, Mo JH, Kerns MJ
Clin Dermatol 2022 Nov-Dec;40(6):639-650. Epub 2022 Jul 28 doi: 10.1016/j.clindermatol.2022.07.011. PMID: 35907580
Symmetrical Skin Lesions on the Gluteal Region in a Patient with Anti-Laminin-332 Mucous Membrane Pemphigoid.
Maki N, Demitsu T, Nagato H, Okada O, Yoneda K, Hashimoto T, Hasunuma N, Osada I, Manabe M
Acta Dermatovenerol Croat 2021 Jul;29(2):105-107. PMID: 34477077
Linear immunoglobulin A/G bullous dermatosis associated with ulcerative colitis.
Onoe A, Matsuura D, Terui T, Ishii N, Hashimoto T, Ochiai T
J Dermatol 2017 Nov;44(11):1295-1298. Epub 2017 Jun 17 doi: 10.1111/1346-8138.13934. PMID: 28623863
Epidermolysis bullosa acquisita. Immunofluorescence, electron microscopic and immunoelectron microscopic studies in four patients.
Nieboer C, Boorsma DM, Woerdeman MJ, Kalsbeek GL
Br J Dermatol 1980 Apr;102(4):383-92. doi: 10.1111/j.1365-2133.1980.tb06550.x. PMID: 6992836

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