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Linear IgG deposits along the epidermal basement membrane zone

MedGen UID:
1623438
•Concept ID:
C4531056
•
Finding
HPO: HP:0031540

Definition

Presence of IgG 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 IgG deposits along the epidermal basement membrane zone

Conditions with this feature

Autoimmune disease, multisystem, infantile-onset, 2
MedGen UID:
934735
•Concept ID:
C4310768
•
Disease or Syndrome
Any autoimmune disease, multisystem, infantile-onset in which the cause of the disease is a mutation in the ZAP70 gene.
See: Condition Record
Autoimmune disease, multisystem, infantile-onset, 2

Recent clinical studies

Etiology

Immunofluorescence serration pattern analysis as a diagnostic criterion in antilaminin-332 mucous membrane pemphigoid: immunopathological findings and clinical experience in 10 Dutch patients.
Terra JB, Pas HH, Hertl M, Dikkers FG, Kamminga N, Jonkman MF
Br J Dermatol 2011 Oct;165(4):815-22. Epub 2011 Sep 15 doi: 10.1111/j.1365-2133.2011.10474.x. PMID: 21692774
False-positive intercellular indirect immunofluorescence test due to anti-blood group antibodies in a patient with epidermolysis bullosa acquisita.
Li W, Zhao X, Shi R, Wang X, Zheng J
Int J Dermatol 2011 Apr;50(4):462-6. doi: 10.1111/j.1365-4632.2010.04532.x. PMID: 21413961
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
Anti-BP180 autoantibodies as a marker of poor prognosis in bullous pemphigoid: a cohort analysis of 94 elderly patients.
Bernard P, Bedane C, Bonnetblanc JM
Br J Dermatol 1997 May;136(5):694-8. PMID: 9205501

Diagnosis

Levofloxacin-Associated Bullous Pemphigoid in a Hemodialysis Patient After Kidney Transplant Failure.
Miao J, Gibson LE, Craici IM
Am J Case Rep 2022 Dec 29;23:e938476. doi: 10.12659/AJCR.938476. PMID: 36578185Free PMC Article
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
Lichen planus pemphigoides associated with Chinese herbs.
Xu HH, Xiao T, He CD, Jin GY, Wang YK, Gao XH, Chen HD
Clin Exp Dermatol 2009 Apr;34(3):329-32. Epub 2008 Aug 9 doi: 10.1111/j.1365-2230.2008.02900.x. PMID: 18699839
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
Anti-BP180 autoantibodies as a marker of poor prognosis in bullous pemphigoid: a cohort analysis of 94 elderly patients.
Bernard P, Bedane C, Bonnetblanc JM
Br J Dermatol 1997 May;136(5):694-8. PMID: 9205501

Therapy

Levofloxacin-Associated Bullous Pemphigoid in a Hemodialysis Patient After Kidney Transplant Failure.
Miao J, Gibson LE, Craici IM
Am J Case Rep 2022 Dec 29;23:e938476. doi: 10.12659/AJCR.938476. PMID: 36578185Free PMC Article
Possible paraneoplastic syndrome case of bullous pemphigoid with immunoglobulin G anti-BP180 C-terminal domain antibodies associated with psoriasis and primary macroglobulinemia.
Maki N, Demitsu T, Umemoto N, Nagashima K, Nakamura T, Kakurai M, Nakamura S, Yamada T, Ishii N, Hashimoto T
J Dermatol 2016 May;43(5):571-4. Epub 2015 Oct 28 doi: 10.1111/1346-8138.13170. PMID: 26507447
Lichen planus pemphigoides associated with Chinese herbs.
Xu HH, Xiao T, He CD, Jin GY, Wang YK, Gao XH, Chen HD
Clin Exp Dermatol 2009 Apr;34(3):329-32. Epub 2008 Aug 9 doi: 10.1111/j.1365-2230.2008.02900.x. PMID: 18699839
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
Bullous pemphigoid induced by PUVA therapy.
Perl S, Rappersberger K, Födinger D, Anegg B, Hönigsmann H, Ortel B
Dermatology 1996;193(3):245-7. doi: 10.1159/000246255. PMID: 8944349

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
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
A child with localized vulval pemphigoid and IgG autoantibodies targeting the C-terminus of collagen XVII/BP180.
Schumann H, Amann U, Tasanen K, Müller S, Zillikens D, Metze D, Luger T, Bruckner-Tuderman L, Bonsmann G
Br J Dermatol 1999 Jun;140(6):1133-8. doi: 10.1046/j.1365-2133.1999.02891.x. PMID: 10354084
Anti-BP180 autoantibodies as a marker of poor prognosis in bullous pemphigoid: a cohort analysis of 94 elderly patients.
Bernard P, Bedane C, Bonnetblanc JM
Br J Dermatol 1997 May;136(5):694-8. PMID: 9205501

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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