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Acute generalized exanthematous pustulosis

MedGen UID:
209091
Concept ID:
C0877055
Disease or Syndrome
Synonyms: Acute Generalised Exanthematous Pustulosis; Acute Generalized Exanthematous Pustulosis; Pustulosis, Exanthematous, Acute Generalized
SNOMED CT: AGEP - acute generalized exanthematous pustulosis (702617007); Acute generalized exanthematous pustulosis (702617007)
Modes of inheritance:
Non-Mendelian inheritance
MedGen UID:
109109
Concept ID:
C0600599
Genetic Function
Source: Orphanet
A mode of inheritance that depends on genetic determinants in more than one gene.
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0017384
Orphanet: ORPHA293173

Definition

A rare toxic dermatosis disease characterized by the rapid development of numerous, nonfollicular, sterile, pinhead-sized pustules on an edematous and erythematous base, predominantly occurring on the trunk, intertriginous and flexural areas, with rare, mostly oral, mucosal involvement. Acute onset of fever (>38°C), peripheral blood leukocytosis, and mild eosinophilia are accompanying features. Systemic involvement, with hepatic, renal or pulmonary dysfunction, occasionally occurs. Histologically reveals characteristic spongiform, subcorneal and/or intraepidermal, pustules, as well as marked edema of the papillary dermis, a perivascularly accentuated, neutrophil-rich inflammatory infiltrate, and intrapustular or intradermal eosinophils. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAcute generalized exanthematous pustulosis
Follow this link to review classifications for Acute generalized exanthematous pustulosis in Orphanet.

Professional guidelines

PubMed

Parisi R, Shah H, Navarini AA, Muehleisen B, Ziv M, Shear NH, Dodiuk-Gad RP
Am J Clin Dermatol 2023 Jul;24(4):557-575. Epub 2023 May 8 doi: 10.1007/s40257-023-00779-3. PMID: 37156992Free PMC Article
Stadler PC, Oschmann A, Kerl-French K, Maul JT, Oppel EM, Meier-Schiesser B, French LE
Dermatology 2023;239(3):328-333. Epub 2023 Jan 26 doi: 10.1159/000529218. PMID: 36702114
Owen CE, Jones JM
Med Clin North Am 2021 Jul;105(4):577-597. doi: 10.1016/j.mcna.2021.04.001. PMID: 34059239

Recent clinical studies

Etiology

Wilkerson RG
Immunol Allergy Clin North Am 2023 Aug;43(3):473-489. doi: 10.1016/j.iac.2022.10.005. PMID: 37394254
Wilkerson RG
Emerg Med Clin North Am 2022 Feb;40(1):39-55. Epub 2021 Oct 29 doi: 10.1016/j.emc.2021.09.001. PMID: 34782090
Zhang J, Lei Z, Xu C, Zhao J, Kang X
Clin Rev Allergy Immunol 2021 Dec;61(3):282-298. Epub 2021 Jul 17 doi: 10.1007/s12016-021-08859-0. PMID: 34273058Free PMC Article
Duong TA, Valeyrie-Allanore L, Wolkenstein P, Chosidow O
Lancet 2017 Oct 28;390(10106):1996-2011. Epub 2017 May 2 doi: 10.1016/S0140-6736(16)30378-6. PMID: 28476287
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

Wilkerson RG
Immunol Allergy Clin North Am 2023 Aug;43(3):473-489. doi: 10.1016/j.iac.2022.10.005. PMID: 37394254
Parisi R, Shah H, Navarini AA, Muehleisen B, Ziv M, Shear NH, Dodiuk-Gad RP
Am J Clin Dermatol 2023 Jul;24(4):557-575. Epub 2023 May 8 doi: 10.1007/s40257-023-00779-3. PMID: 37156992Free PMC Article
Stadler PC, Oschmann A, Kerl-French K, Maul JT, Oppel EM, Meier-Schiesser B, French LE
Dermatology 2023;239(3):328-333. Epub 2023 Jan 26 doi: 10.1159/000529218. PMID: 36702114
Sussman M, Napodano A, Huang S, Are A, Hsu S, Motaparthi K
Medicina (Kaunas) 2021 Sep 23;57(10) doi: 10.3390/medicina57101004. PMID: 34684041Free PMC Article
Duong TA, Valeyrie-Allanore L, Wolkenstein P, Chosidow O
Lancet 2017 Oct 28;390(10106):1996-2011. Epub 2017 May 2 doi: 10.1016/S0140-6736(16)30378-6. PMID: 28476287

Therapy

Sibaud V, Sollena P
Ann Dermatol Venereol 2023 Sep;150(3):208-212. Epub 2023 Aug 14 doi: 10.1016/j.annder.2022.11.013. PMID: 37586898
Dilley M, Geng B
Clin Rev Allergy Immunol 2022 Jun;62(3):463-475. Epub 2021 Dec 15 doi: 10.1007/s12016-021-08878-x. PMID: 34910281Free PMC Article
Owen CE, Jones JM
Med Clin North Am 2021 Jul;105(4):577-597. doi: 10.1016/j.mcna.2021.04.001. PMID: 34059239
Szatkowski J, Schwartz RA
J Am Acad Dermatol 2015 Nov;73(5):843-8. Epub 2015 Sep 6 doi: 10.1016/j.jaad.2015.07.017. PMID: 26354880
Harr T, French LE
Orphanet J Rare Dis 2010 Dec 16;5:39. doi: 10.1186/1750-1172-5-39. PMID: 21162721Free PMC Article

Prognosis

Sussman M, Napodano A, Huang S, Are A, Hsu S, Motaparthi K
Medicina (Kaunas) 2021 Sep 23;57(10) doi: 10.3390/medicina57101004. PMID: 34684041Free PMC Article
Owen CE, Jones JM
Med Clin North Am 2021 Jul;105(4):577-597. doi: 10.1016/j.mcna.2021.04.001. PMID: 34059239
Guvenir H, Arikoglu T, Vezir E, Misirlioglu ED
Curr Pharm Des 2019;25(36):3840-3854. doi: 10.2174/1381612825666191107162921. PMID: 31696807
Duong TA, Valeyrie-Allanore L, Wolkenstein P, Chosidow O
Lancet 2017 Oct 28;390(10106):1996-2011. Epub 2017 May 2 doi: 10.1016/S0140-6736(16)30378-6. PMID: 28476287
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

Sibaud V, Sollena P
Ann Dermatol Venereol 2023 Sep;150(3):208-212. Epub 2023 Aug 14 doi: 10.1016/j.annder.2022.11.013. PMID: 37586898
Alvarado SA, Muñoz-Mendoza D, Bahna SL
Ann Allergy Asthma Immunol 2018 Nov;121(5):552-560. Epub 2018 May 25 doi: 10.1016/j.anai.2018.05.022. PMID: 29803714
Castelain F, Humbert P
Curr Drug Saf 2012 Nov 1;7(5):332-8. doi: 10.2174/157488612805076516. PMID: 23441982
Harr T, French LE
Orphanet J Rare Dis 2010 Dec 16;5:39. doi: 10.1186/1750-1172-5-39. PMID: 21162721Free PMC Article
Halevy S
Curr Opin Allergy Clin Immunol 2009 Aug;9(4):322-8. doi: 10.1097/ACI.0b013e32832cf64e. PMID: 19458527

Recent systematic reviews

Al Muqarrab F, Schwartz RA, Almohssen AA
Acta Dermatovenerol Croat 2022 Dec;30(4):227-236. PMID: 36919389
Lei H, Deng H, Liu X, Li Z, Wang C
J Clin Pharm Ther 2022 Dec;47(12):2008-2013. Epub 2022 Jul 31 doi: 10.1111/jcpt.13738. PMID: 35909299
Safa I, Ines L, Noureddine L, Meriem J, Manel N, Belhajali H, Faten Z, Zili J
Dermatol Ther 2021 Sep;34(5):e15087. Epub 2021 Aug 25 doi: 10.1111/dth.15087. PMID: 34351040
Torres-Navarro I, de Unamuno-Bustos B, Botella-Estrada R
J Eur Acad Dermatol Venereol 2021 Mar;35(3):607-614. Epub 2020 Sep 30 doi: 10.1111/jdv.16894. PMID: 32846030
Vassallo C, Derlino F, Brazzelli V, D'Ospina RD, Borroni G
G Ital Dermatol Venereol 2014 Jun;149(3):281-90. PMID: 24819755

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