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IgA pemphigus

MedGen UID:
697519
•Concept ID:
C1274167
•
Disease or Syndrome
Synonym: Immunoglobulin A pemphigus
SNOMED CT: IgA pemphigus (402717008); Immunoglobulin A pemphigus (402717008)
 
Monarch Initiative: MONDO:0034127
Orphanet: ORPHA555905

Definition

A rare autoimmune bullous skin disease characterized by painful and pruritic vesiculopustular eruptions resulting from circulating IgA antibodies against keratinocyte cell surface components. The lesions are typically found at the periphery of erythematous annular plaques and favor intertriginous regions. Histologically and immunologically, IgA pemphigus can be subdivided into subcorneal pustular dermatosis and intraepidermal neutrophilic IgA dermatosis. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVIgA pemphigus

Professional guidelines

PubMed

Dermoscopic approach for differential diagnosing of autoimmune bullous disease: pemphigus vulgaris, pemphigus foliaceus, and IgA pemphigus.
Gharib K, Nassar A, Youssef A, Bessar H
Int J Dermatol 2023 Jun;62(6):770-775. Epub 2023 Mar 3 doi: 10.1111/ijd.16623. PMID: 36866791
Clinical Patterns, Survival, Comorbidities, and Treatment Regimens in 149 Patients With Pemphigus in Tuscany (Italy): A 12-Year Hospital-Based Study.
Quintarelli L, Coi A, Maglie R, Corrà A, Mariotti EB, Aimo C, Ruffo di Calabria V, Verdelli A, Bianchi B, Del Bianco E, Antiga E, Caproni M
Front Immunol 2022;13:895490. Epub 2022 Jul 8 doi: 10.3389/fimmu.2022.895490. PMID: 35880183Free PMC Article
Treatment of pemphigus.
Camisa C, Warner M
Dermatol Nurs 1998 Apr;10(2):115-8, 123-31. PMID: 9697444

Recent clinical studies

Etiology

Superficial and Bullous Neutrophilic Dermatoses: Sneddon-Wilkinson, IgA Pemphigus, and Bullous Lupus.
Manjaly P, Sanchez K, Gregoire S, Ly S, Kamal K, Mostaghimi A
Dermatol Clin 2024 Apr;42(2):307-315. Epub 2023 Sep 7 doi: 10.1016/j.det.2023.08.010. PMID: 38423689
An Updated Review of Pemphigus Diseases.
Malik AM, Tupchong S, Huang S, Are A, Hsu S, Motaparthi K
Medicina (Kaunas) 2021 Oct 9;57(10) doi: 10.3390/medicina57101080. PMID: 34684117Free PMC Article
Intraepithelial autoimmune blistering dermatoses: Clinical features and diagnosis.
Montagnon CM, Tolkachjov SN, Murrell DF, Camilleri MJ, Lehman JS
J Am Acad Dermatol 2021 Jun;84(6):1507-1519. Epub 2021 Mar 5 doi: 10.1016/j.jaad.2020.11.075. PMID: 33684498
Pemphigus group: overview, epidemiology, mortality, and comorbidities.
Kridin K
Immunol Res 2018 Apr;66(2):255-270. doi: 10.1007/s12026-018-8986-7. PMID: 29479654
IgA pemphigus foliaceus: a case report.
Zillikens D, Miller K, Hartmann AA, Burg G
Dermatologica 1990;181(4):304-7. doi: 10.1159/000247833. PMID: 2073960

Diagnosis

An Updated Review of Pemphigus Diseases.
Malik AM, Tupchong S, Huang S, Are A, Hsu S, Motaparthi K
Medicina (Kaunas) 2021 Oct 9;57(10) doi: 10.3390/medicina57101080. PMID: 34684117Free PMC Article
Immune serological diagnosis of pemphigus.
Solimani F, Meier K, Zimmer CL, Hashimoto T
Ital J Dermatol Venerol 2021 Apr;156(2):151-160. Epub 2020 Nov 23 doi: 10.23736/S2784-8671.20.06788-7. PMID: 33228340
A case of tinea incognito mimicking subcorneal pustular dermatosis.
Kalkan G, Demirseren DD, Güney CA, Aktaş A
Dermatol Online J 2020 Feb 15;26(2) PMID: 32239896
Pemphigus group: overview, epidemiology, mortality, and comorbidities.
Kridin K
Immunol Res 2018 Apr;66(2):255-270. doi: 10.1007/s12026-018-8986-7. PMID: 29479654
Generalized pustular psoriasis induced by systemic steroid dose reduction.
Westphal DC, Schettini AP, Souza PP, Castiel J, Chirano CA, Santos M
An Bras Dermatol 2016 Sep-Oct;91(5):664-666. doi: 10.1590/abd1806-4841.20163804. PMID: 27828647Free PMC Article

Therapy

A case of tinea incognito mimicking subcorneal pustular dermatosis.
Kalkan G, Demirseren DD, Güney CA, Aktaş A
Dermatol Online J 2020 Feb 15;26(2) PMID: 32239896
Oral pemphigus.
Giannetti L, Generali L, Bertoldi C
G Ital Dermatol Venereol 2018 Jun;153(3):383-388. Epub 2018 Mar 6 doi: 10.23736/S0392-0488.18.05887-X. PMID: 29512980
Generalized pustular psoriasis induced by systemic steroid dose reduction.
Westphal DC, Schettini AP, Souza PP, Castiel J, Chirano CA, Santos M
An Bras Dermatol 2016 Sep-Oct;91(5):664-666. doi: 10.1590/abd1806-4841.20163804. PMID: 27828647Free PMC Article
Immunoglobulin A pemphigus involving the perianal skin and oral mucosa: an unusual presentation.
Erdag G, Qureshi HS, Greer KE, Patterson JW
Cutis 2007 Sep;80(3):218-20. PMID: 17956010
IgA pemphigus occurring in a 1-month-old infant.
Suzuki M, Karube S, Kobori Y, Usui K, Murata S, Kato H, Nakagawa H
J Am Acad Dermatol 2003 Feb;48(2 Suppl):S22-4. doi: 10.1067/mjd.2003.119. PMID: 12582378

Prognosis

Immune serological diagnosis of pemphigus.
Solimani F, Meier K, Zimmer CL, Hashimoto T
Ital J Dermatol Venerol 2021 Apr;156(2):151-160. Epub 2020 Nov 23 doi: 10.23736/S2784-8671.20.06788-7. PMID: 33228340
Pemphigus group: overview, epidemiology, mortality, and comorbidities.
Kridin K
Immunol Res 2018 Apr;66(2):255-270. doi: 10.1007/s12026-018-8986-7. PMID: 29479654
IgA pemphigus.
Tsuruta D, Ishii N, Hamada T, Ohyama B, Fukuda S, Koga H, Imamura K, Kobayashi H, Karashima T, Nakama T, Dainichi T, Hashimoto T
Clin Dermatol 2011 Jul-Aug;29(4):437-42. doi: 10.1016/j.clindermatol.2011.01.014. PMID: 21679872
Aggressive immunosuppressive therapy for a refractory case of IgA pemphigus.
Sibley Hash K, Rencic A, Hernandez MI, Hashimoto T, Nousari HC
Arch Dermatol 2002 Jun;138(6):744-6. doi: 10.1001/archderm.138.6.744. PMID: 12056953
The new pemphigus variants.
Robinson ND, Hashimoto T, Amagai M, Chan LS
J Am Acad Dermatol 1999 May;40(5 Pt 1):649-71; quiz 672-3. doi: 10.1016/s0190-9622(99)70145-3. PMID: 10321591

Clinical prediction guides

A case of tinea incognito mimicking subcorneal pustular dermatosis.
Kalkan G, Demirseren DD, Güney CA, Aktaş A
Dermatol Online J 2020 Feb 15;26(2) PMID: 32239896
Pemphigus group: overview, epidemiology, mortality, and comorbidities.
Kridin K
Immunol Res 2018 Apr;66(2):255-270. doi: 10.1007/s12026-018-8986-7. PMID: 29479654
IgA pemphigus.
Tsuruta D, Ishii N, Hamada T, Ohyama B, Fukuda S, Koga H, Imamura K, Kobayashi H, Karashima T, Nakama T, Dainichi T, Hashimoto T
Clin Dermatol 2011 Jul-Aug;29(4):437-42. doi: 10.1016/j.clindermatol.2011.01.014. PMID: 21679872
Autoimmunity against desmosomal cadherins in pemphigus.
Amagai M
J Dermatol Sci 1999 Jun;20(2):92-102. doi: 10.1016/s0923-1811(99)00016-x. PMID: 10379702
Intercellular IgA dermatosis.
Ongenae KC, Temmerman LJ, Vermander F, Naeyaert JM
Eur J Dermatol 1999 Mar;9(2):85-94. PMID: 10066953

Recent systematic reviews

IgA pemphigus: A systematic review.
Kridin K, Patel PM, Jones VA, Cordova A, Amber KT
J Am Acad Dermatol 2020 Jun;82(6):1386-1392. Epub 2019 Dec 5 doi: 10.1016/j.jaad.2019.11.059. PMID: 31812619

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