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Subepidermal blistering

MedGen UID:
347341
Concept ID:
C1856956
Finding
Synonym: Subepidermal blisters
 
HPO: HP:0033804

Definition

A type of blistering in which the lesions are located beneath the epidermis. [from HPO]

Conditions with this feature

Epidermolysis bullosa, junctional 2A, intermediate
MedGen UID:
1807376
Concept ID:
C5676936
Disease or Syndrome
Intermediate junctional epidermolysis bullosa 2A (JEB2A) is an autosomal recessive blistering disease of skin and mucous membranes. Blistering is less severe than in severe JEB (see 226700). The plane of skin cleavage is through the lamina lucida of the cutaneous basement membrane zone. Oral mucosa may be involved and nail bed blistering has been reported. Blistering does not affect the life span of affected individuals (summary by Has et al., 2020). For a discussion of genetic heterogeneity of the subtypes of JEB, see JEB1A (226650). Reviews Has et al. (2020) reviewed the clinical and genetic aspects, genotype-phenotype correlations, disease-modifying factors, and natural history of epidermolysis bullosa.

Professional guidelines

PubMed

Borradori L, Van Beek N, Feliciani C, Tedbirt B, Antiga E, Bergman R, Böckle BC, Caproni M, Caux F, Chandran NS, Cianchini G, Daneshpazhooh M, De D, Didona D, Di Zenzo GM, Dmochowski M, Drenovska K, Ehrchen J, Goebeler M, Groves R, Günther C, Horvath B, Hertl M, Hofmann S, Ioannides D, Itzlinger-Monshi B, Jedličková J, Kowalewski C, Kridin K, Lim YL, Marinovic B, Marzano AV, Mascaro JM, Meijer JM, Murrell D, Patsatsi K, Pincelli C, Prost C, Rappersberger K, Sárdy M, Setterfield J, Shahid M, Sprecher E, Tasanen K, Uzun S, Vassileva S, Vestergaard K, Vorobyev A, Vujic I, Wang G, Wozniak K, Yayli S, Zambruno G, Zillikens D, Schmidt E, Joly P
J Eur Acad Dermatol Venereol 2022 Oct;36(10):1689-1704. Epub 2022 Jun 29 doi: 10.1111/jdv.18220. PMID: 35766904
Bernard P, Antonicelli F
Am J Clin Dermatol 2017 Aug;18(4):513-528. doi: 10.1007/s40257-017-0264-2. PMID: 28247089
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

Recent clinical studies

Etiology

Tan WH, Sim J, Sultana R, Lee HY
Dermatology 2023;239(1):5-11. Epub 2022 Oct 27 doi: 10.1159/000526600. PMID: 36302346
Mai Y, Izumi K, Mai S, Ujiie H
Front Immunol 2022;13:963401. Epub 2022 Aug 8 doi: 10.3389/fimmu.2022.963401. PMID: 36003369Free PMC Article
Pratasava V, Sahni VN, Suresh A, Huang S, Are A, Hsu S, Motaparthi K
Medicina (Kaunas) 2021 Oct 4;57(10) doi: 10.3390/medicina57101061. PMID: 34684098Free PMC Article
Moro F, Fania L, Sinagra JLM, Salemme A, Di Zenzo G
Biomolecules 2020 Oct 10;10(10) doi: 10.3390/biom10101432. PMID: 33050407Free PMC Article
Bernard P, Antonicelli F
Am J Clin Dermatol 2017 Aug;18(4):513-528. doi: 10.1007/s40257-017-0264-2. PMID: 28247089

Diagnosis

Borradori L, Van Beek N, Feliciani C, Tedbirt B, Antiga E, Bergman R, Böckle BC, Caproni M, Caux F, Chandran NS, Cianchini G, Daneshpazhooh M, De D, Didona D, Di Zenzo GM, Dmochowski M, Drenovska K, Ehrchen J, Goebeler M, Groves R, Günther C, Horvath B, Hertl M, Hofmann S, Ioannides D, Itzlinger-Monshi B, Jedličková J, Kowalewski C, Kridin K, Lim YL, Marinovic B, Marzano AV, Mascaro JM, Meijer JM, Murrell D, Patsatsi K, Pincelli C, Prost C, Rappersberger K, Sárdy M, Setterfield J, Shahid M, Sprecher E, Tasanen K, Uzun S, Vassileva S, Vestergaard K, Vorobyev A, Vujic I, Wang G, Wozniak K, Yayli S, Zambruno G, Zillikens D, Schmidt E, Joly P
J Eur Acad Dermatol Venereol 2022 Oct;36(10):1689-1704. Epub 2022 Jun 29 doi: 10.1111/jdv.18220. PMID: 35766904
Pratasava V, Sahni VN, Suresh A, Huang S, Are A, Hsu S, Motaparthi K
Medicina (Kaunas) 2021 Oct 4;57(10) doi: 10.3390/medicina57101061. PMID: 34684098Free PMC Article
Moro F, Fania L, Sinagra JLM, Salemme A, Di Zenzo G
Biomolecules 2020 Oct 10;10(10) doi: 10.3390/biom10101432. PMID: 33050407Free PMC Article
Bernard P, Antonicelli F
Am J Clin Dermatol 2017 Aug;18(4):513-528. doi: 10.1007/s40257-017-0264-2. PMID: 28247089
Goletz S, Hashimoto T, Zillikens D, Schmidt E
J Am Acad Dermatol 2014 Jul;71(1):185-91. Epub 2014 Apr 24 doi: 10.1016/j.jaad.2014.02.036. PMID: 24767733

Therapy

Tan WH, Sim J, Sultana R, Lee HY
Dermatology 2023;239(1):5-11. Epub 2022 Oct 27 doi: 10.1159/000526600. PMID: 36302346
Borradori L, Van Beek N, Feliciani C, Tedbirt B, Antiga E, Bergman R, Böckle BC, Caproni M, Caux F, Chandran NS, Cianchini G, Daneshpazhooh M, De D, Didona D, Di Zenzo GM, Dmochowski M, Drenovska K, Ehrchen J, Goebeler M, Groves R, Günther C, Horvath B, Hertl M, Hofmann S, Ioannides D, Itzlinger-Monshi B, Jedličková J, Kowalewski C, Kridin K, Lim YL, Marinovic B, Marzano AV, Mascaro JM, Meijer JM, Murrell D, Patsatsi K, Pincelli C, Prost C, Rappersberger K, Sárdy M, Setterfield J, Shahid M, Sprecher E, Tasanen K, Uzun S, Vassileva S, Vestergaard K, Vorobyev A, Vujic I, Wang G, Wozniak K, Yayli S, Zambruno G, Zillikens D, Schmidt E, Joly P
J Eur Acad Dermatol Venereol 2022 Oct;36(10):1689-1704. Epub 2022 Jun 29 doi: 10.1111/jdv.18220. PMID: 35766904
Hofmann SC, Juratli HA, Eming R
J Dtsch Dermatol Ges 2018 Nov;16(11):1339-1358. doi: 10.1111/ddg.13688. PMID: 30395395
Cobos G, Mu E, Cohen J, Beasley J, Brinster N, Femia A
Dermatol Online J 2017 Dec 15;23(12) PMID: 29447655
Bernard P, Antonicelli F
Am J Clin Dermatol 2017 Aug;18(4):513-528. doi: 10.1007/s40257-017-0264-2. PMID: 28247089

Prognosis

Aromolo IF, Maronese CA, Moltrasio C, Genovese G, Marzano AV
Int J Dermatol 2022 Nov;61(11):1353-1358. Epub 2022 Jan 20 doi: 10.1111/ijd.16045. PMID: 35049061
Zhou T, Peng B, Geng S
Front Immunol 2021;12:718073. Epub 2021 Aug 24 doi: 10.3389/fimmu.2021.718073. PMID: 34504496Free PMC Article
Moro F, Fania L, Sinagra JLM, Salemme A, Di Zenzo G
Biomolecules 2020 Oct 10;10(10) doi: 10.3390/biom10101432. PMID: 33050407Free PMC Article
Nguyen T, Kwan JM, Ahmed AR
Dermatology 2014;229(2):88-96. Epub 2014 May 22 doi: 10.1159/000362208. PMID: 24861031
Ahmed AR, Kurgis BS, Rogers RS 3rd
J Am Acad Dermatol 1991 Jun;24(6 Pt 1):987-1001. doi: 10.1016/0190-9622(91)70159-y. PMID: 1869688

Clinical prediction guides

Tan WH, Sim J, Sultana R, Lee HY
Dermatology 2023;239(1):5-11. Epub 2022 Oct 27 doi: 10.1159/000526600. PMID: 36302346
Zhou T, Peng B, Geng S
Front Immunol 2021;12:718073. Epub 2021 Aug 24 doi: 10.3389/fimmu.2021.718073. PMID: 34504496Free PMC Article
Kridin K
Immunol Res 2018 Feb;66(1):6-17. doi: 10.1007/s12026-017-8975-2. PMID: 29159697
Amber KT, Murrell DF, Schmidt E, Joly P, Borradori L
Clin Rev Allergy Immunol 2018 Feb;54(1):26-51. doi: 10.1007/s12016-017-8633-4. PMID: 28779299
Bernard P, Antonicelli F
Am J Clin Dermatol 2017 Aug;18(4):513-528. doi: 10.1007/s40257-017-0264-2. PMID: 28247089

Recent systematic reviews

Jiirasutat N, Pongchareon P, Weschawalit S
Int J Dermatol 2024 May;63(5):572-579. Epub 2024 Jan 12 doi: 10.1111/ijd.16970. PMID: 38217028
Aromolo IF, Maronese CA, Moltrasio C, Genovese G, Marzano AV
Int J Dermatol 2022 Nov;61(11):1353-1358. Epub 2022 Jan 20 doi: 10.1111/ijd.16045. PMID: 35049061
Wilson A, Murrell DF
Clin Exp Dermatol 2022 May;47(5):873-881. Epub 2022 Jan 2 doi: 10.1111/ced.15059. PMID: 34919291

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