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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)
 
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
Akay N, Boyvat A, Heper AO, Soykan I, Arica IE, Bektas M, Ensari A, Cokca F
Clin Exp Dermatol 2006 May;31(3):384-6. doi: 10.1111/j.1365-2230.2006.02093.x. PMID: 16681583
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
Perry HO, Winkelmann RK
Arch Dermatol 1972 Dec;106(6):901-5. PMID: 4508849

Therapy

Diab R, Rakhshan A, Kaddah A, Abdollahimajd F, Mirzaei HR
Anticancer Drugs 2022 Oct 1;33(9):970-974. Epub 2022 Aug 9 doi: 10.1097/CAD.0000000000001338. PMID: 35946554
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
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
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
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

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
Fox LP, Geyer AS, Husain S, Grossman ME
Leuk Lymphoma 2006 Jan;47(1):147-50. doi: 10.1080/10428190500254299. PMID: 16321840
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
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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