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Neutrophilic dermatosis

MedGen UID:
923154
•Concept ID:
C1142272
•
Disease or Syndrome
Synonym: Neutrophilic Dermatosis
SNOMED CT: Neutrophilic dermatosis (724834006)

Definition

A type of dermatosis characterized by dense infiltration of neutrophils in the skin without evidence of infection or vasculitis. It may be secondary to an underlying systemic illness. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVNeutrophilic dermatosis

Professional guidelines

PubMed

Diagnosis and novel clinical treatment strategies for pyoderma gangrenosum.
Goldust M, Hagstrom EL, Rathod D, Ortega-Loayza AG
Expert Rev Clin Pharmacol 2020 Feb;13(2):157-161. Epub 2020 Jan 6 doi: 10.1080/17512433.2020.1709825. PMID: 31875484
Pyoderma gangrenosum - a guide to diagnosis and management .
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
PAPA, PASH and PAPASH Syndromes: Pathophysiology, Presentation and Treatment.
Cugno M, Borghi A, Marzano AV
Am J Clin Dermatol 2017 Aug;18(4):555-562. doi: 10.1007/s40257-017-0265-1. PMID: 28236224

Recent clinical studies

Etiology

UBA1 Variations in Neutrophilic Dermatosis Skin Lesions of Patients With VEXAS Syndrome.
Zakine E, Schell B, Battistella M, Vignon-Pennamen MD, Chasset F, Mahévas T, Cordoliani F, Adès L, Sébert M, Delaleu J, Jachiet M, Lepelletier C, Lemaire P, Chauvel C, Dhouaieb B, Kim R, Cassius C, Georgin-Lavialle S, Mekinian A, Bagot M, Braun T, Rousset L, Begon E, de Masson A, Fenaux P, Clappier E, Bouaziz JD
JAMA Dermatol 2021 Nov 1;157(11):1349-1354. doi: 10.1001/jamadermatol.2021.3344. PMID: 34495287Free PMC Article
JAK-inhibitors. New players in the field of immune-mediated diseases, beyond rheumatoid arthritis.
Fragoulis GE, McInnes IB, Siebert S
Rheumatology (Oxford) 2019 Feb 1;58(Suppl 1):i43-i54. doi: 10.1093/rheumatology/key276. PMID: 30806709Free PMC Article
Neutrophilic dermatoses: Pyoderma gangrenosum and other bowel- and arthritis-associated neutrophilic dermatoses.
Ashchyan HJ, Nelson CA, Stephen S, James WD, Micheletti RG, Rosenbach M
J Am Acad Dermatol 2018 Dec;79(6):1009-1022. Epub 2018 Apr 11 doi: 10.1016/j.jaad.2017.11.063. PMID: 29653213
Neutrophilic dermatoses: Pathogenesis, Sweet syndrome, neutrophilic eccrine hidradenitis, and Behçet disease.
Nelson CA, Stephen S, Ashchyan HJ, James WD, Micheletti RG, Rosenbach M
J Am Acad Dermatol 2018 Dec;79(6):987-1006. Epub 2018 Apr 11 doi: 10.1016/j.jaad.2017.11.064. PMID: 29653210
JAK1/2 inhibition with baricitinib in the treatment of autoinflammatory interferonopathies.
Sanchez GAM, Reinhardt A, Ramsey S, Wittkowski H, Hashkes PJ, Berkun Y, Schalm S, Murias S, Dare JA, Brown D, Stone DL, Gao L, Klausmeier T, Foell D, de Jesus AA, Chapelle DC, Kim H, Dill S, Colbert RA, Failla L, Kost B, O'Brien M, Reynolds JC, Folio LR, Calvo KR, Paul SM, Weir N, Brofferio A, Soldatos A, Biancotto A, Cowen EW, Digiovanna JJ, Gadina M, Lipton AJ, Hadigan C, Holland SM, Fontana J, Alawad AS, Brown RJ, Rother KI, Heller T, Brooks KM, Kumar P, Brooks SR, Waldman M, Singh HK, Nickeleit V, Silk M, Prakash A, Janes JM, Ozen S, Wakim PG, Brogan PA, Macias WL, Goldbach-Mansky R
J Clin Invest 2018 Jul 2;128(7):3041-3052. Epub 2018 Jun 11 doi: 10.1172/JCI98814. PMID: 29649002Free PMC Article

Diagnosis

New Practical Aspects of Sweet Syndrome.
Joshi TP, Friske SK, Hsiou DA, Duvic M
Am J Clin Dermatol 2022 May;23(3):301-318. Epub 2022 Feb 14 doi: 10.1007/s40257-022-00673-4. PMID: 35157247Free PMC Article
Pyoderma gangrenosum.
Maverakis E, Marzano AV, Le ST, Callen JP, Brüggen MC, Guenova E, Dissemond J, Shinkai K, Langan SM
Nat Rev Dis Primers 2020 Oct 8;6(1):81. doi: 10.1038/s41572-020-0213-x. PMID: 33033263
Neutrophilic urticarial dermatosis: A review.
Gusdorf L, Lipsker D
Ann Dermatol Venereol 2018 Dec;145(12):735-740. Epub 2018 Sep 14 doi: 10.1016/j.annder.2018.06.010. PMID: 30224079
Skin manifestations of rheumatoid arthritis.
Lora V, Cerroni L, Cota C
G Ital Dermatol Venereol 2018 Apr;153(2):243-255. Epub 2018 Jan 24 doi: 10.23736/S0392-0488.18.05872-8. PMID: 29368864
Periodic fever syndromes.
Lachmann HJ
Best Pract Res Clin Rheumatol 2017 Aug;31(4):596-609. Epub 2018 Jan 11 doi: 10.1016/j.berh.2017.12.001. PMID: 29773275

Therapy

New Practical Aspects of Sweet Syndrome.
Joshi TP, Friske SK, Hsiou DA, Duvic M
Am J Clin Dermatol 2022 May;23(3):301-318. Epub 2022 Feb 14 doi: 10.1007/s40257-022-00673-4. PMID: 35157247Free PMC Article
Neutrophilic urticarial dermatosis: A review.
Gusdorf L, Lipsker D
Ann Dermatol Venereol 2018 Dec;145(12):735-740. Epub 2018 Sep 14 doi: 10.1016/j.annder.2018.06.010. PMID: 30224079
Neutrophilic dermatoses: Pathogenesis, Sweet syndrome, neutrophilic eccrine hidradenitis, and Behçet disease.
Nelson CA, Stephen S, Ashchyan HJ, James WD, Micheletti RG, Rosenbach M
J Am Acad Dermatol 2018 Dec;79(6):987-1006. Epub 2018 Apr 11 doi: 10.1016/j.jaad.2017.11.064. PMID: 29653210
Skin manifestations of rheumatoid arthritis.
Lora V, Cerroni L, Cota C
G Ital Dermatol Venereol 2018 Apr;153(2):243-255. Epub 2018 Jan 24 doi: 10.23736/S0392-0488.18.05872-8. PMID: 29368864
Periodic fever syndromes.
Lachmann HJ
Best Pract Res Clin Rheumatol 2017 Aug;31(4):596-609. Epub 2018 Jan 11 doi: 10.1016/j.berh.2017.12.001. PMID: 29773275

Prognosis

VEXAS syndrome: a new paradigm for adult-onset monogenic autoinflammatory diseases.
Vitale A, Caggiano V, Bimonte A, Caroni F, Tosi GM, Fabbiani A, Renieri A, Bocchia M, Frediani B, Fabiani C, Cantarini L
Intern Emerg Med 2023 Apr;18(3):711-722. Epub 2023 Jan 20 doi: 10.1007/s11739-023-03193-z. PMID: 36662445Free PMC Article
Sweet Syndrome in the Pediatric Population.
McClanahan D, Funk T, Small A
Dermatol Clin 2022 Apr;40(2):179-190. Epub 2022 Mar 4 doi: 10.1016/j.det.2021.12.005. PMID: 35366971
Dermatological Toxicities of Bruton's Tyrosine Kinase Inhibitors.
Sibaud V, Beylot-Barry M, Protin C, Vigarios E, Recher C, Ysebaert L
Am J Clin Dermatol 2020 Dec;21(6):799-812. doi: 10.1007/s40257-020-00535-x. PMID: 32613545
Extracutaneous involvement of pyoderma gangrenosum.
Borda LJ, Wong LL, Marzano AV, Ortega-Loayza AG
Arch Dermatol Res 2019 Aug;311(6):425-434. Epub 2019 Mar 29 doi: 10.1007/s00403-019-01912-1. PMID: 30923901
Sweet's syndrome.
Limdiwala PG, Parikh SJ, Shah JS
Indian J Dent Res 2014 May-Jun;25(3):401-5. doi: 10.4103/0970-9290.138358. PMID: 25099003

Clinical prediction guides

Pyoderma Gangrenosum: Treatment Options.
Dissemond J, Marzano AV, Hampton PJ, Ortega-Loayza AG
Drugs 2023 Sep;83(14):1255-1267. Epub 2023 Aug 23 doi: 10.1007/s40265-023-01931-3. PMID: 37610614Free PMC Article
New Practical Aspects of Sweet Syndrome.
Joshi TP, Friske SK, Hsiou DA, Duvic M
Am J Clin Dermatol 2022 May;23(3):301-318. Epub 2022 Feb 14 doi: 10.1007/s40257-022-00673-4. PMID: 35157247Free PMC Article
Pyoderma gangrenosum: A systematic review of the molecular characteristics of disease.
Flora A, Kozera E, Frew JW
Exp Dermatol 2022 Apr;31(4):498-515. Epub 2022 Feb 15 doi: 10.1111/exd.14534. PMID: 35114021
JAK1/2 inhibition with baricitinib in the treatment of autoinflammatory interferonopathies.
Sanchez GAM, Reinhardt A, Ramsey S, Wittkowski H, Hashkes PJ, Berkun Y, Schalm S, Murias S, Dare JA, Brown D, Stone DL, Gao L, Klausmeier T, Foell D, de Jesus AA, Chapelle DC, Kim H, Dill S, Colbert RA, Failla L, Kost B, O'Brien M, Reynolds JC, Folio LR, Calvo KR, Paul SM, Weir N, Brofferio A, Soldatos A, Biancotto A, Cowen EW, Digiovanna JJ, Gadina M, Lipton AJ, Hadigan C, Holland SM, Fontana J, Alawad AS, Brown RJ, Rother KI, Heller T, Brooks KM, Kumar P, Brooks SR, Waldman M, Singh HK, Nickeleit V, Silk M, Prakash A, Janes JM, Ozen S, Wakim PG, Brogan PA, Macias WL, Goldbach-Mansky R
J Clin Invest 2018 Jul 2;128(7):3041-3052. Epub 2018 Jun 11 doi: 10.1172/JCI98814. PMID: 29649002Free PMC Article
Drug-Induced Pyoderma Gangrenosum: A Review.
Wang JY, French LE, Shear NH, Amiri A, Alavi A
Am J Clin Dermatol 2018 Feb;19(1):67-77. doi: 10.1007/s40257-017-0308-7. PMID: 28624960

Recent systematic reviews

Chronic Atypical Neutrophilic Dermatosis With Lipodystrophy and Elevated Temperature Syndrome: A Systemic Review.
Altom A, Khader SAE, Gad AG, Anadani R, Dang DP, Ansar F, Chaudhari J, Crespo-Quezada J, Huy NT
Am J Dermatopathol 2023 Jun 1;45(6):355-370. Epub 2023 Feb 17 doi: 10.1097/DAD.0000000000002345. PMID: 37191371
The 2021 European Alliance of Associations for Rheumatology/American College of Rheumatology Points to Consider for Diagnosis and Management of Autoinflammatory Type I Interferonopathies: CANDLE/PRAAS, SAVI, and AGS.
Cetin Gedik K, Lamot L, Romano M, Demirkaya E, Piskin D, Torreggiani S, Adang LA, Armangue T, Barchus K, Cordova DR, Crow YJ, Dale RC, Durrant KL, Eleftheriou D, Fazzi EM, Gattorno M, Gavazzi F, Hanson EP, Lee-Kirsch MA, Montealegre Sanchez GA, Neven B, Orcesi S, Ozen S, Poli MC, Schumacher E, Tonduti D, Uss K, Aletaha D, Feldman BM, Vanderver A, Brogan PA, Goldbach-Mansky R
Arthritis Rheumatol 2022 May;74(5):735-751. Epub 2022 Mar 21 doi: 10.1002/art.42087. PMID: 35315249
Pyoderma gangrenosum: A systematic review of the molecular characteristics of disease.
Flora A, Kozera E, Frew JW
Exp Dermatol 2022 Apr;31(4):498-515. Epub 2022 Feb 15 doi: 10.1111/exd.14534. PMID: 35114021
The 2021 European Alliance of Associations for Rheumatology/American College of Rheumatology points to consider for diagnosis and management of autoinflammatory type I interferonopathies: CANDLE/PRAAS, SAVI and AGS.
Cetin Gedik K, Lamot L, Romano M, Demirkaya E, Piskin D, Torreggiani S, Adang LA, Armangue T, Barchus K, Cordova DR, Crow YJ, Dale RC, Durrant KL, Eleftheriou D, Fazzi EM, Gattorno M, Gavazzi F, Hanson EP, Lee-Kirsch MA, Montealegre Sanchez GA, Neven B, Orcesi S, Ozen S, Poli MC, Schumacher E, Tonduti D, Uss K, Aletaha D, Feldman BM, Vanderver A, Brogan PA, Goldbach-Mansky R
Ann Rheum Dis 2022 May;81(5):601-613. Epub 2022 Jan 27 doi: 10.1136/annrheumdis-2021-221814. PMID: 35086813Free PMC Article
Is neutrophilic dermatosis a manifestation of familial Mediterranean fever?
Delplanque M, Ducharme-Bénard S, Moguelet P, Chasset F, Grateau G, Georgin-Lavialle S, Bachmeyer C
Scand J Rheumatol 2022 Jan;51(1):42-49. Epub 2021 Jun 23 doi: 10.1080/03009742.2021.1904588. PMID: 34159892

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