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Bullous pyoderma gangrenosum

MedGen UID:
590609
Concept ID:
C0406687
Disease or Syndrome
Synonyms: Bullous pyoderma; bullous pyoderma gangrenosum; Phemphigoid pyoderma gangrenosum
SNOMED CT: Bullous pyoderma (238985006); Bullous pyoderma gangrenosum (238985006)
 
Monarch Initiative: MONDO:0035237
Orphanet: ORPHA538869

Definition

A rare subtype of pyoderma gangrenosum disease characterized by grouped vesicles that rapidly spread and coalesce to form large bullae, which evolve into ulcerations that have an erythematous peripheral halo and central necrosis, mainly affecting the upper limbs and face. Lymphoproliferative diseases are frequently associated, thus prognosis is often compromised. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVBullous pyoderma gangrenosum

Professional guidelines

PubMed

Janowska A, Oranges T, Fissi A, Davini G, Romanelli M, Dini V
Dermatol Ther 2020 May;33(3):e13412. Epub 2020 May 5 doi: 10.1111/dth.13412. PMID: 32291879
George C, Deroide F, Rustin M
Clin Med (Lond) 2019 May;19(3):224-228. doi: 10.7861/clinmedicine.19-3-224. PMID: 31092515Free PMC Article
Ahronowitz I, Harp J, Shinkai K
Am J Clin Dermatol 2012 Jun 1;13(3):191-211. doi: 10.2165/11595240-000000000-00000. PMID: 22356259

Recent clinical studies

Etiology

Rafael MR, Fernandes CM, Machado JM, Rodrigues PA, Cardoso OJ, Afonso A, Sousa AB, Pacheco FM, Proença RM
J Eur Acad Dermatol Venereol 2003 Jul;17(4):449-51. doi: 10.1046/j.1468-3083.2003.00766.x. PMID: 12834459

Diagnosis

Vacas AS, Bollea-Garlatti ML, Torre AC, Galimberti RL
An Bras Dermatol 2018 Jan-Feb;93(1):133-134. doi: 10.1590/abd1806-4841.20187031. PMID: 29641716Free PMC Article
Sener S, Sarac G, Altunisik N, Bayindir Y
Niger J Clin Pract 2016 Nov-Dec;19(6):837-839. doi: 10.4103/1119-3077.187327. PMID: 27811462
Miyata T, Yashiro M, Hayashi M, Kamata K, Katsuoka K
J Dermatol 2012 Dec;39(12):1006-9. Epub 2012 Oct 5 doi: 10.1111/j.1346-8138.2012.01672.x. PMID: 23039059
Ho KK, Otridge BW, Vandenberg E, Powell FC
J Am Acad Dermatol 1992 Nov;27(5 Pt 2):804-8. doi: 10.1016/0190-9622(92)70252-b. PMID: 1469131
Perry HO, Winkelmann RK
Arch Dermatol 1972 Dec;106(6):901-5. PMID: 4508849

Therapy

Aktaş Karabay E, Aksu Cerman A, Kıvanc Altunay İ, Yalçın Ö
Am J Dermatopathol 2017 Jun;39(6):476-478. doi: 10.1097/DAD.0000000000000801. PMID: 27893467
Miyata T, Yashiro M, Hayashi M, Kamata K, Katsuoka K
J Dermatol 2012 Dec;39(12):1006-9. Epub 2012 Oct 5 doi: 10.1111/j.1346-8138.2012.01672.x. PMID: 23039059
Sakiyama M, Kobayashi T, Nagata Y, Fujimoto N, Satoh T, Tajima S
J Dermatol 2012 Dec;39(12):1010-5. Epub 2012 Sep 14 doi: 10.1111/j.1346-8138.2012.01676.x. PMID: 22974277
Fox LP, Geyer AS, Husain S, Grossman ME
Leuk Lymphoma 2006 Jan;47(1):147-50. doi: 10.1080/10428190500254299. PMID: 16321840
Callen JP
Dermatol Clin 1985 Jan;3(1):153-63. PMID: 3912087

Prognosis

Vacas AS, Bollea-Garlatti ML, Torre AC, Galimberti RL
An Bras Dermatol 2018 Jan-Feb;93(1):133-134. doi: 10.1590/abd1806-4841.20187031. PMID: 29641716Free PMC Article
Koester G, Tarnower A, Levisohn D, Burgdorf W
J Am Acad Dermatol 1993 Nov;29(5 Pt 2):875-8. doi: 10.1016/0190-9622(93)70261-q. PMID: 8408832
Ho KK, Otridge BW, Vandenberg E, Powell FC
J Am Acad Dermatol 1992 Nov;27(5 Pt 2):804-8. doi: 10.1016/0190-9622(92)70252-b. PMID: 1469131
Hay CR, Messenger AG, Cotton DW, Bleehen SS, Winfield DA
J Clin Pathol 1987 Apr;40(4):387-92. doi: 10.1136/jcp.40.4.387. PMID: 3473088Free PMC Article
Perry HO, Winkelmann RK
Arch Dermatol 1972 Dec;106(6):901-5. PMID: 4508849

Clinical prediction guides

Vacas AS, Bollea-Garlatti ML, Torre AC, Galimberti RL
An Bras Dermatol 2018 Jan-Feb;93(1):133-134. doi: 10.1590/abd1806-4841.20187031. PMID: 29641716Free PMC Article
Altunay IK, Sezgin SA, Ileri U, Ekmekçi TR, Kuran I, Köşlü A, Baş L
Int J Dermatol 2001 May;40(5):327-9. doi: 10.1046/j.1365-4362.2001.01091.x. PMID: 11554994

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