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Acute febrile neutrophilic dermatosis(AFND)

MedGen UID:
43097
Concept ID:
C0085077
Disease or Syndrome
Synonyms: AFND; Gomm Button disease; Sweet Syndrome
SNOMED CT: Febrile neutrophilic dermatosis (84625002); Acute febrile neutrophilic dermatosis (84625002); Sweet disease (84625002); Sweet syndrome (84625002)
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.
 
Gene (location): MEFV (16p13.3)
 
Monarch Initiative: MONDO:0011959
OMIM®: 608068
Orphanet: ORPHA3243

Definition

Acute febrile neutrophilic dermatosis (AFND) is an autosomal dominant autoinflammatory disorder characterized by onset of recurrent fever and dermatologic abnormalities in childhood. Laboratory studies show elevated acute-phase reactants and activation of the inflammatory response, particularly IL1B (147720). Additional more variable features may include myalgia and arthralgia (summary by Masters et al., 2016). [from OMIM]

Clinical features

From HPO
Arthralgia
MedGen UID:
13917
Concept ID:
C0003862
Sign or Symptom
Joint pain.
Myalgia
MedGen UID:
68541
Concept ID:
C0231528
Sign or Symptom
Pain in muscle.
Primary dilated cardiomyopathy
MedGen UID:
2880
Concept ID:
C0007193
Disease or Syndrome
Familial dilated cardiomyopathy is a genetic form of heart disease. It occurs when heart (cardiac) muscle becomes thin and weakened in at least one chamber of the heart, causing the open area of the chamber to become enlarged (dilated). As a result, the heart is unable to pump blood as efficiently as usual. To compensate, the heart attempts to increase the amount of blood being pumped through the heart, leading to further thinning and weakening of the cardiac muscle. Over time, this condition results in heart failure.\n\nIt usually takes many years for symptoms of familial dilated cardiomyopathy to cause health problems. They typically begin in mid-adulthood, but can occur at any time from infancy to late adulthood. Signs and symptoms of familial dilated cardiomyopathy can include an irregular heartbeat (arrhythmia), shortness of breath (dyspnea), extreme tiredness (fatigue), fainting episodes (syncope), and swelling of the legs and feet. In some cases, the first sign of the disorder is sudden cardiac death. The severity of the condition varies among affected individuals, even in members of the same family.
Small vessel vasculitis
MedGen UID:
868710
Concept ID:
C4023113
Disease or Syndrome
A type of vasculitis (inflammation of blood vessel walls) that affects blood vessels that are smaller than arteries, i.e., arterioles, venules, and capilllaries.
Anemia
MedGen UID:
1526
Concept ID:
C0002871
Disease or Syndrome
A reduction in erythrocytes volume or hemoglobin concentration.
Cystic acne
MedGen UID:
507614
Concept ID:
C0010666
Disease or Syndrome
A severe type of acne characterized by the formation of cysts enclosing a mixture of keratin and sebum.
Panniculitis
MedGen UID:
45301
Concept ID:
C0030326
Disease or Syndrome
Inflammation of subcutaneous adipose tissue.
Acne inversa
MedGen UID:
57993
Concept ID:
C0162836
Disease or Syndrome
A chronic skin condition involving the inflammation of the apocrine sweat glands, forming pimple-like bumps known as abscesses.
Increased circulating interleukin 6 concentration
MedGen UID:
901461
Concept ID:
C4280771
Finding
The concentration of interleukin-6 in the blood circulation is above the upper limit of normal.
Elevated erythrocyte sedimentation rate
MedGen UID:
57727
Concept ID:
C0151632
Finding
An increased erythrocyte sedimentation rate (ESR). The ESR is a test that measures the distance that erythrocytes have fallen after one hour in a vertical column of anticoagulated blood under the influence of gravity. The ESR is a nonspecific finding. An elevation may indicate inflammation or may be caused by any condition that elevates fibrinogen.
Recurrent fever
MedGen UID:
811468
Concept ID:
C3714772
Sign or Symptom
Periodic (episodic or recurrent) bouts of fever.
Elevated circulating C-reactive protein concentration
MedGen UID:
892906
Concept ID:
C4023452
Finding
An abnormal elevation of the C-reactive protein level in the blood circulation.
Erythema
MedGen UID:
11999
Concept ID:
C0041834
Disease or Syndrome
Redness of the skin, caused by hyperemia of the capillaries in the lower layers of the skin.
Pyoderma gangrenosum
MedGen UID:
43224
Concept ID:
C0085652
Disease or Syndrome
A deep skin ulcer with a well defined border, which is usually violet or blue. The ulcer edge is often undermined (worn and damaged) and the surrounding skin is erythematous and indurated. The ulcer often starts as a small papule or collection of papules, which break down to form small ulcers with a so called cat's paw appearance. These coalesce and the central area then undergoes necrosis to form a single ulcer.

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAcute febrile neutrophilic dermatosis
Follow this link to review classifications for Acute febrile neutrophilic dermatosis in Orphanet.

Professional guidelines

PubMed

Hrin ML, Williams J, Bowers NL, Pichardo RO, Jorizzo JL, Feldman SR, Huang WW
J Cutan Med Surg 2022 Sep-Oct;26(5):532-533. Epub 2022 Jul 13 doi: 10.1177/12034754221111992. PMID: 35822306
Dabade TS, Davis MD
Dermatol Ther 2011 Mar-Apr;24(2):273-84. doi: 10.1111/j.1529-8019.2011.01403.x. PMID: 21410617
Hisanaga K, Iwasaki Y, Itoyama Y; Neuro-Sweet Disease Study Group
Neurology 2005 May 24;64(10):1756-61. doi: 10.1212/01.WNL.0000161848.34159.B5. PMID: 15911805

Recent clinical studies

Etiology

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
Baartman B, Kosari P, Warren CC, Ali S, Jorizzo JL, Sato M, Kurup SK
Dermatology 2014;228(3):193-7. Epub 2014 Mar 1 doi: 10.1159/000357729. PMID: 24603349
Marzano AV, Menicanti C, Crosti C, Trevisan V
G Ital Dermatol Venereol 2013 Apr;148(2):185-96. PMID: 23588144
Dabade TS, Davis MD
Dermatol Ther 2011 Mar-Apr;24(2):273-84. doi: 10.1111/j.1529-8019.2011.01403.x. PMID: 21410617
Wallach D, Vignon-Pennamen MD
J Am Acad Dermatol 2006 Dec;55(6):1066-71. Epub 2006 Oct 18 doi: 10.1016/j.jaad.2006.07.016. PMID: 17097401

Diagnosis

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
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
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
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
Cohen PR
Orphanet J Rare Dis 2007 Jul 26;2:34. doi: 10.1186/1750-1172-2-34. PMID: 17655751Free PMC Article

Therapy

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
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
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
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
Kim MJ, Choe YH
Eur J Pediatr 2010 Dec;169(12):1439-44. Epub 2010 May 6 doi: 10.1007/s00431-010-1201-z. PMID: 20445990

Prognosis

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
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
Baartman B, Kosari P, Warren CC, Ali S, Jorizzo JL, Sato M, Kurup SK
Dermatology 2014;228(3):193-7. Epub 2014 Mar 1 doi: 10.1159/000357729. PMID: 24603349
Dabade TS, Davis MD
Dermatol Ther 2011 Mar-Apr;24(2):273-84. doi: 10.1111/j.1529-8019.2011.01403.x. PMID: 21410617
Ratzinger G, Burgdorf W, Zelger BG, Zelger B
Am J Dermatopathol 2007 Apr;29(2):125-33. doi: 10.1097/01.dad.0000249887.59810.76. PMID: 17414432

Clinical prediction guides

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
Anzalone CL, Cohen PR
Curr Opin Hematol 2013 Jan;20(1):26-35. doi: 10.1097/MOH.0b013e32835ad132. PMID: 23207661
Dabade TS, Davis MD
Dermatol Ther 2011 Mar-Apr;24(2):273-84. doi: 10.1111/j.1529-8019.2011.01403.x. PMID: 21410617
Cohen PR, Talpaz M, Kurzrock R
J Clin Oncol 1988 Dec;6(12):1887-97. doi: 10.1200/JCO.1988.6.12.1887. PMID: 3058878
Cohen PR, Kurzrock R
Am J Med 1987 Jun;82(6):1220-6. doi: 10.1016/0002-9343(87)90229-4. PMID: 3300306

Recent systematic reviews

Kakaletsis N, Kaiafa G, Savopoulos C, Iliadis F, Perifanis V, Tzalokostas V, Grekou A, Giannouli A, Hatzitolios AI
Acta Haematol 2014;132(2):220-5. doi: 10.1159/000357933. PMID: 24714374

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