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Linear C3 deposits along the epidermal basement membrane zone

MedGen UID:
1611994
•Concept ID:
C4531055
•
Anatomical Abnormality
HPO: HP:0031541

Definition

Presence of complement C3 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 C3 deposits along the epidermal basement membrane zone

Recent clinical studies

Etiology

Pemphigoid gestationis.
Lipozenčić J, Ljubojevic S, Bukvić-Mokos Z
Clin Dermatol 2012 Jan-Feb;30(1):51-5. doi: 10.1016/j.clindermatol.2011.03.009. PMID: 22137226
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
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
Lichen Planus Pemphigoides Associated With PD-1 and PD-L1 Inhibitors: A Case Series and Review of the Literature.
Boyle MM, Ashi S, Puiu T, Reimer D, Sokumbi O, Soltani K, Onajin O
Am J Dermatopathol 2022 May 1;44(5):360-367. doi: 10.1097/DAD.0000000000002139. PMID: 35120032
Lichen Planus Pemphigoides Mimicking Toxic Epidermal Necrolysis.
Ondhia C, Kaur C, Mee J, Natkunarajah J, Singh M
Am J Dermatopathol 2019 Nov;41(11):e144-e147. doi: 10.1097/DAD.0000000000001461. PMID: 31145106
Pemphigoid gestationis.
Lipozenčić J, Ljubojevic S, Bukvić-Mokos Z
Clin Dermatol 2012 Jan-Feb;30(1):51-5. doi: 10.1016/j.clindermatol.2011.03.009. PMID: 22137226
The predominance of IgG4 in prodromal bullous pemphigoid.
Lamb PM, Patton T, Deng JS
Int J Dermatol 2008 Feb;47(2):150-3. doi: 10.1111/j.1365-4632.2008.03361.x. PMID: 18211485

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
Drug-induced linear IgA bullous dermatosis in a patient with a vancomycin-impregnated cement spacer.
Riemenschneider K, Diiorio DA, Zic JA, Livingood MR, Fine JD, Powers JG, Zwerner JP, Tkaczyk E
Cutis 2018 Apr;101(4):293-296. PMID: 29763476Free PMC Article
Pemphigoid gestationis.
Lipozenčić J, Ljubojevic S, Bukvić-Mokos Z
Clin Dermatol 2012 Jan-Feb;30(1):51-5. doi: 10.1016/j.clindermatol.2011.03.009. PMID: 22137226
Localized linear IgA dermatosis induced by UV light-treatment for herpes zoster.
He C, Xu H, Xiao T, Geng L, Chen HD
Int J Dermatol 2007 May;46(5):500-2. doi: 10.1111/j.1365-4632.2007.02966.x. PMID: 17472682
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
Pemphigoid gestationis.
Lipozenčić J, Ljubojevic S, Bukvić-Mokos Z
Clin Dermatol 2012 Jan-Feb;30(1):51-5. doi: 10.1016/j.clindermatol.2011.03.009. PMID: 22137226
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
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
Nonbullous pemphigoid: prodrome of bullous pemphigoid or a distinct pemphigoid variant?
Strohal R, Rappersberger K, Pehamberger H, Wolff K
J Am Acad Dermatol 1993 Aug;29(2 Pt 2):293-9. doi: 10.1016/0190-9622(93)70179-w. PMID: 8340501

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
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
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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