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Linear IgA Dermatosis

MedGen UID:
473106
•Concept ID:
C0406650
•
Disease or Syndrome
Synonyms: Dermatoses, Linear IgA; Dermatosis, Linear IgA; IgA Dermatoses, Linear; IgA Dermatosis, Linear; Linear IgA Bullous Dermatosis; Linear IgA Dermatoses
SNOMED CT: Linear immunoglobulin A bullous dermatosis (95330001); IgA - Linear immunoglobulin A bullous dermatosis (95330001); LAD - Linear IgA disease (95330001); Linear IgA disease (95330001); Linear IgA dermatosis (95330001); Linear immunoglobulin A dermatosis (95330001)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0018748
Orphanet: ORPHA46488

Definition

A rare, acquired autoimmune bullous skin disease characterized by annular, grouped blisters on the skin and, frequently, mucous membranes with linear deposition of immunoglobulin A along the basement membrane zone (BMZ). [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVLinear IgA Dermatosis
Follow this link to review classifications for Linear IgA Dermatosis in Orphanet.

Professional guidelines

PubMed

S2k guidelines on diagnosis and treatment of linear IgA dermatosis initiated by the European Academy of Dermatology and Venereology.
Caux F, Patsatsi A, Karakioulaki M, Antiga E, Baselga E, Borradori L, Caproni M, Cardones AR, Chandran NS, Dräger S, Drenovska K, Goebeler M, Günther C, Hofmann SC, Ioannides D, Joly P, Marinović B, Mariotti EB, Marzano AV, Morel KD, Murrell DF, Prost C, Sárdy M, Setterfield J, Skiljevic D, Uzun S, Vassileva S, Zambruno G, Schmidt E
J Eur Acad Dermatol Venereol 2024 Jun;38(6):1006-1023. Epub 2024 Feb 29 doi: 10.1111/jdv.19880. PMID: 38421060
Pemphigoid diseases: pathogenesis, diagnosis, and treatment.
Kasperkiewicz M, Zillikens D, Schmidt E
Autoimmunity 2012 Feb;45(1):55-70. Epub 2011 Sep 19 doi: 10.3109/08916934.2011.606447. PMID: 21923615
Intravenous immunoglobulin in the treatment of autoimmune bullous dermatoses: an update.
Czernik A, Toosi S, Bystryn JC, Grando SA
Autoimmunity 2012 Feb;45(1):111-8. Epub 2011 Sep 19 doi: 10.3109/08916934.2011.606452. PMID: 21923613

Recent clinical studies

Etiology

State-of-the-art diagnosis of autoimmune blistering diseases.
van Beek N, Holtsche MM, Atefi I, Olbrich H, Schmitz MJ, Pruessmann J, Vorobyev A, Schmidt E
Front Immunol 2024;15:1363032. Epub 2024 Jun 6 doi: 10.3389/fimmu.2024.1363032. PMID: 38903493Free PMC Article
S2k guidelines on diagnosis and treatment of linear IgA dermatosis initiated by the European Academy of Dermatology and Venereology.
Caux F, Patsatsi A, Karakioulaki M, Antiga E, Baselga E, Borradori L, Caproni M, Cardones AR, Chandran NS, Dräger S, Drenovska K, Goebeler M, Günther C, Hofmann SC, Ioannides D, Joly P, Marinović B, Mariotti EB, Marzano AV, Morel KD, Murrell DF, Prost C, Sárdy M, Setterfield J, Skiljevic D, Uzun S, Vassileva S, Zambruno G, Schmidt E
J Eur Acad Dermatol Venereol 2024 Jun;38(6):1006-1023. Epub 2024 Feb 29 doi: 10.1111/jdv.19880. PMID: 38421060
Stevens-johnson Syndrome and Toxic Epidermal Necrolysis: An Overview of Diagnosis, Therapy Options and Prognosis of Patients.
Garg VK, Buttar HS, Bhat SA, Ainur N, Priya T, Kashyap D, Tuli HS
Recent Adv Inflamm Allergy Drug Discov 2023;17(2):110-120. doi: 10.2174/2772270817666230821102441. PMID: 37605396
Blistering diseases in the mature patient.
Lakoš Jukić I, Jerković Gulin S, Marinović B
Clin Dermatol 2018 Mar-Apr;36(2):231-238. Epub 2017 Oct 3 doi: 10.1016/j.clindermatol.2017.10.014. PMID: 29566927
Toxic epidermal necrolysis and Stevens-Johnson syndrome.
Harr T, French LE
Orphanet J Rare Dis 2010 Dec 16;5:39. doi: 10.1186/1750-1172-5-39. PMID: 21162721Free PMC Article

Diagnosis

State-of-the-art diagnosis of autoimmune blistering diseases.
van Beek N, Holtsche MM, Atefi I, Olbrich H, Schmitz MJ, Pruessmann J, Vorobyev A, Schmidt E
Front Immunol 2024;15:1363032. Epub 2024 Jun 6 doi: 10.3389/fimmu.2024.1363032. PMID: 38903493Free PMC Article
S2k guidelines on diagnosis and treatment of linear IgA dermatosis initiated by the European Academy of Dermatology and Venereology.
Caux F, Patsatsi A, Karakioulaki M, Antiga E, Baselga E, Borradori L, Caproni M, Cardones AR, Chandran NS, Dräger S, Drenovska K, Goebeler M, Günther C, Hofmann SC, Ioannides D, Joly P, Marinović B, Mariotti EB, Marzano AV, Morel KD, Murrell DF, Prost C, Sárdy M, Setterfield J, Skiljevic D, Uzun S, Vassileva S, Zambruno G, Schmidt E
J Eur Acad Dermatol Venereol 2024 Jun;38(6):1006-1023. Epub 2024 Feb 29 doi: 10.1111/jdv.19880. PMID: 38421060
Stevens-johnson Syndrome and Toxic Epidermal Necrolysis: An Overview of Diagnosis, Therapy Options and Prognosis of Patients.
Garg VK, Buttar HS, Bhat SA, Ainur N, Priya T, Kashyap D, Tuli HS
Recent Adv Inflamm Allergy Drug Discov 2023;17(2):110-120. doi: 10.2174/2772270817666230821102441. PMID: 37605396
Norfloxacin-Induced Linear IgA Dermatosis.
Jakhar D, Singal A, Sharma S, Kotru M
Skinmed 2020;18(6):374-377. Epub 2020 Dec 1 PMID: 33397569
Toxic epidermal necrolysis and Stevens-Johnson syndrome.
Harr T, French LE
Orphanet J Rare Dis 2010 Dec 16;5:39. doi: 10.1186/1750-1172-5-39. PMID: 21162721Free PMC Article

Therapy

Stevens-johnson Syndrome and Toxic Epidermal Necrolysis: An Overview of Diagnosis, Therapy Options and Prognosis of Patients.
Garg VK, Buttar HS, Bhat SA, Ainur N, Priya T, Kashyap D, Tuli HS
Recent Adv Inflamm Allergy Drug Discov 2023;17(2):110-120. doi: 10.2174/2772270817666230821102441. PMID: 37605396
Norfloxacin-Induced Linear IgA Dermatosis.
Jakhar D, Singal A, Sharma S, Kotru M
Skinmed 2020;18(6):374-377. Epub 2020 Dec 1 PMID: 33397569
Lichen Planus Pemphigoides: From Lichenoid Inflammation to Autoantibody-Mediated Blistering.
Hübner F, Langan EA, Recke A
Front Immunol 2019;10:1389. Epub 2019 Jul 2 doi: 10.3389/fimmu.2019.01389. PMID: 31312198Free PMC Article
Toxic epidermal necrolysis and Stevens-Johnson syndrome.
Harr T, French LE
Orphanet J Rare Dis 2010 Dec 16;5:39. doi: 10.1186/1750-1172-5-39. PMID: 21162721Free PMC Article
Colchicine revisited.
Bhat A, Naguwa SM, Cheema GS, Gershwin ME
Ann N Y Acad Sci 2009 Sep;1173:766-73. doi: 10.1111/j.1749-6632.2009.04674.x. PMID: 19758227

Prognosis

State-of-the-art diagnosis of autoimmune blistering diseases.
van Beek N, Holtsche MM, Atefi I, Olbrich H, Schmitz MJ, Pruessmann J, Vorobyev A, Schmidt E
Front Immunol 2024;15:1363032. Epub 2024 Jun 6 doi: 10.3389/fimmu.2024.1363032. PMID: 38903493Free PMC Article
Retrospective analysis of autoimmune bullous diseases in Middle Franconia.
Sollfrank L, Schönfelder V, Sticherling M
Front Immunol 2023;14:1256617. Epub 2023 Oct 10 doi: 10.3389/fimmu.2023.1256617. PMID: 37881435Free PMC Article
Stevens-johnson Syndrome and Toxic Epidermal Necrolysis: An Overview of Diagnosis, Therapy Options and Prognosis of Patients.
Garg VK, Buttar HS, Bhat SA, Ainur N, Priya T, Kashyap D, Tuli HS
Recent Adv Inflamm Allergy Drug Discov 2023;17(2):110-120. doi: 10.2174/2772270817666230821102441. PMID: 37605396
Blistering diseases in the mature patient.
Lakoš Jukić I, Jerković Gulin S, Marinović B
Clin Dermatol 2018 Mar-Apr;36(2):231-238. Epub 2017 Oct 3 doi: 10.1016/j.clindermatol.2017.10.014. PMID: 29566927
Toxic epidermal necrolysis and Stevens-Johnson syndrome.
Harr T, French LE
Orphanet J Rare Dis 2010 Dec 16;5:39. doi: 10.1186/1750-1172-5-39. PMID: 21162721Free PMC Article

Clinical prediction guides

Stevens-johnson Syndrome and Toxic Epidermal Necrolysis: An Overview of Diagnosis, Therapy Options and Prognosis of Patients.
Garg VK, Buttar HS, Bhat SA, Ainur N, Priya T, Kashyap D, Tuli HS
Recent Adv Inflamm Allergy Drug Discov 2023;17(2):110-120. doi: 10.2174/2772270817666230821102441. PMID: 37605396
Type XVII collagen: Relevance of distinct epitopes, complement-independent effects, and association with neurological disorders in pemphigoid disorders.
Opelka B, Schmidt E, Goletz S
Front Immunol 2022;13:948108. Epub 2022 Aug 10 doi: 10.3389/fimmu.2022.948108. PMID: 36032160Free PMC Article
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
Toxic epidermal necrolysis and Stevens-Johnson syndrome.
Harr T, French LE
Orphanet J Rare Dis 2010 Dec 16;5:39. doi: 10.1186/1750-1172-5-39. PMID: 21162721Free PMC Article
Diagnosis of adult linear IgA dermatosis by immunoelectronmicroscopy in 16 patients with linear IgA deposits.
Prost C, De Leca AC, Combemale P, Labeille B, Martin N, Cosnes A, Guillaume JC, Venencie PY, Verret JL, Dubertret L
J Invest Dermatol 1989 Jan;92(1):39-45. doi: 10.1111/1523-1747.ep13070851. PMID: 2642511

Recent systematic reviews

Phototherapy in the treatment of cutaneous herpesvirus manifestations.
Kelley JP, Rashid RM
Cutis 2011 Sep;88(3):140-8. PMID: 22017068

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