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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
Modes of inheritance:
Inheritance with unknown mechanism
MedGen UID:
831588
Concept ID:
CN227389
Intellectual Product
Source: Orphanet
Describes an inherited disorder with unknown mode of inheritance.
SNOMED CT: Febrile neutrophilic dermatosis (84625002); Acute febrile neutrophilic dermatosis (84625002); Sweet's disease (84625002); Sweet's syndrome (84625002); Sweet disease (84625002); Sweet syndrome (84625002)
 
OMIM®: 608068
Orphanet: ORPHA3243

Definition

A rare syndrome characterized by fever, skin papules and plaques, and leukocytosis. Morphologically, the skin lesions show neutrophilic infiltrates and dermal edema. It may occur in the absence of underlying conditions. It may also be associated with the presence of cancer or may be a side effect of medications. [from NCI]

Clinical features

Inflammatory abnormality of the eye
MedGen UID:
866622
Concept ID:
C4020969
Anatomical Abnormality
Inflammation of the eye, parts of the eye or the periorbital region.
Anemia
MedGen UID:
1526
Concept ID:
C0002871
Disease or Syndrome
If you have anemia, your blood does not carry enough oxygen to the rest of your body. The most common cause of anemia is not having enough iron. Your body needs iron to make hemoglobin. Hemoglobin is an iron-rich protein that gives the red color to blood. It carries oxygen from the lungs to the rest of the body. Anemia has three main causes: blood loss, lack of red blood cell production, and high rates of red blood cell destruction. Conditions that may lead to anemia include. -Heavy periods. -Pregnancy. -Ulcers. -Colon polyps or colon cancer. -Inherited disorders. -A diet that does not have enough iron, folic acid or vitamin B12. -Blood disorders such as sickle cell anemia and thalassemia, or cancer. -Aplastic anemia, a condition that can be inherited or acquired. -G6PD deficiency, a metabolic disorder. Anemia can make you feel tired, cold, dizzy, and irritable. You may be short of breath or have a headache. Your doctor will diagnose anemia with a physical exam and blood tests. Treatment depends on the kind of anemia you have. NIH: National Heart, Lung, and Blood Institute.
Leukocytosis
MedGen UID:
9736
Concept ID:
C0023518
Pathologic Function
An abnormal increase in the number of leukocytes in the blood.
Thrombocytopenia
MedGen UID:
52737
Concept ID:
C0040034
Finding
A reduction in the number of circulating thrombocytes.
Hematuria
MedGen UID:
5488
Concept ID:
C0018965
Finding
The presence of blood in the urine. Hematuria may be gross hematuria (visible to the naked eye) or microscopic hematuria (detected by dipstick or microscopic examination of the urine).
Proteinuria
MedGen UID:
10976
Concept ID:
C0033687
Finding
Increased levels of protein in the urine.
Renal failure syndrome
MedGen UID:
11177
Concept ID:
C0035078
Disease or Syndrome
Healthy kidneys clean your blood by removing excess fluid, minerals, and wastes. They also make hormones that keep your bones strong and your blood healthy. But if the kidneys are damaged, they don't work properly. Harmful wastes can build up in your body. Your blood pressure may rise. Your body may retain excess fluid and not make enough red blood cells. This is called kidney failure. If your kidneys fail, you need treatment to replace the work they normally do. The treatment options are dialysis or a kidney transplant. Each treatment has benefits and drawbacks. No matter which treatment you choose, you'll need to make some changes in your life, including how you eat and plan your activities. But with the help of healthcare providers, family, and friends, most people with kidney failure can lead full and active lives. NIH: National Institute of Diabetes and Digestive and Kidney Diseases.
Glomerulopathy
MedGen UID:
451033
Concept ID:
C0268731
Disease or Syndrome
Inflammatory or noninflammatory diseases affecting the glomeruli of the nephron.
Migraine
MedGen UID:
57451
Concept ID:
C0149931
Disease or Syndrome
Migraine is the most common type of chronic, episodic headache, as summarized by Featherstone (1985). One locus for migraine with or without aura (MGR1) has been identified on chromosome 4q24. Other loci for migraine have been identified on 6p21.1-p12.2 (MGR3; 607498), 14q21.2-q22.3 (MGR4; 607501), 19p13 (MGR5; 607508), 1q31 (MGR6; 607516), 15q11-q13 (MGR7; 609179), 5q21 (with or without aura, MGR8, 609570; with aura, MGR9, 609670), 17p13 (MGR10; 610208), 18q12 (MGR11; 610209), 10q22-q23 (MGR12; 611706), and the X chromosome (MGR2; 300125). Mutation in the KCNK18 gene (613655) on chromosome 10q25 causes migraine with aura (MGR13; 613656). A subtype of autosomal dominant migraine with aura (MA), familial hemiplegic migraine (FHM; see 141500), is caused by mutation in the CACNA1A gene (601011) on chromosome 19p13 (FHM1; 141500), by mutation in the ATP1A2 gene (182340) on chromosome 1q21 (FHM2; 602481), or by mutation in the SCN1A gene (182389) on chromosome 2q24 (FHM3; 609634). Another locus for FHM has been mapped to chromosome 1q31 (FHM4; see 607516). There is evidence that a polymorphism in the estrogen receptor gene (ESR1; 133430.0005) and a polymorphism in the TNF gene (191160.0004) may confer susceptibility to migraine. A polymorphism in the endothelin receptor type A gene (EDNRA; 131243.0001) may confer resistance to migraine.
Pulmonary infiltrates
MedGen UID:
116009
Concept ID:
C0235896
Finding
A finding indicating the presence of an inflammatory or neoplastic cellular infiltrate in the lung parenchyma.
Recurrent respiratory infections
MedGen UID:
812812
Concept ID:
C3806482
Finding
An increased susceptibility to respiratory infections as manifested by a history of recurrent respiratory infections.
Splenomegaly
MedGen UID:
52469
Concept ID:
C0038002
Finding
Enlargement of the spleen.
Malabsorption
MedGen UID:
811453
Concept ID:
C3714745
Finding
Impaired ability to absorb one or more nutrients from the intestine.
Muscle pain
MedGen UID:
68541
Concept ID:
C0231528
Sign or Symptom
Pain in a muscle or group of muscles.
Exanthema
MedGen UID:
8732
Concept ID:
C0015230
Finding
A rash is an area of irritated or swollen skin. Many rashes are itchy, red, painful, and irritated. Some rashes can also lead to blisters or patches of raw skin. Rashes are a symptom of many different medical problems. Other causes include irritating substances and allergies. Certain genes can make people more likely to get rashes. Contact dermatitis is a common type of rash. It causes redness, itching, and sometimes small bumps. You get the rash where you have touched an irritant, such as a chemical, or something you are allergic to, like poison ivy. Some rashes develop right away. Others form over several days. Although most rashes clear up fairly quickly, others are long-lasting and need long-term treatment. Because rashes can be caused by many different things, it's important to figure out what kind you have before you treat it. If it is a bad rash, if it does not go away, or if you have other symptoms, you should see your health care provider. Treatments may include moisturizers, lotions, baths, cortisone creams that relieve swelling, and antihistamines, which relieve itching.
Leukocytosis
MedGen UID:
9736
Concept ID:
C0023518
Pathologic Function
An abnormal increase in the number of leukocytes in the blood.
Splenomegaly
MedGen UID:
52469
Concept ID:
C0038002
Finding
Enlargement of the spleen.
Pustule
MedGen UID:
488804
Concept ID:
C0241157
Finding
A vesicle filled with leukocytes
Recurrent respiratory infections
MedGen UID:
812812
Concept ID:
C3806482
Finding
An increased susceptibility to respiratory infections as manifested by a history of recurrent respiratory infections.
Inflammatory abnormality of the eye
MedGen UID:
866622
Concept ID:
C4020969
Anatomical Abnormality
Inflammation of the eye, parts of the eye or the periorbital region.
Body Temperature Changes
MedGen UID:
626
Concept ID:
C0005904
Sign or Symptom
An abnormality of temperature homeostasis.
Hematuria
MedGen UID:
5488
Concept ID:
C0018965
Finding
The presence of blood in the urine. Hematuria may be gross hematuria (visible to the naked eye) or microscopic hematuria (detected by dipstick or microscopic examination of the urine).
Proteinuria
MedGen UID:
10976
Concept ID:
C0033687
Finding
Increased levels of protein in the urine.
Joint pain
MedGen UID:
13917
Concept ID:
C0003862
Sign or Symptom
Joint pain.
Abnormality of the oral cavity
MedGen UID:
871391
Concept ID:
C4025887
Anatomical Abnormality
Abnormality of the oral cavity, i.e., the opening or hollow part of the mouth.
Exanthema
MedGen UID:
8732
Concept ID:
C0015230
Finding
A rash is an area of irritated or swollen skin. Many rashes are itchy, red, painful, and irritated. Some rashes can also lead to blisters or patches of raw skin. Rashes are a symptom of many different medical problems. Other causes include irritating substances and allergies. Certain genes can make people more likely to get rashes. Contact dermatitis is a common type of rash. It causes redness, itching, and sometimes small bumps. You get the rash where you have touched an irritant, such as a chemical, or something you are allergic to, like poison ivy. Some rashes develop right away. Others form over several days. Although most rashes clear up fairly quickly, others are long-lasting and need long-term treatment. Because rashes can be caused by many different things, it's important to figure out what kind you have before you treat it. If it is a bad rash, if it does not go away, or if you have other symptoms, you should see your health care provider. Treatments may include moisturizers, lotions, baths, cortisone creams that relieve swelling, and antihistamines, which relieve itching.
Skin ulcer
MedGen UID:
52369
Concept ID:
C0037299
Disease or Syndrome
A discontinuity of the skin exhibiting complete loss of the epidermis and often portions of the dermis and even subcutaneous fat.
skin bullae
MedGen UID:
451011
Concept ID:
C0241054
Finding
The presence of one or more bullae on the skin, defined as fluid-filled blisters more than 5 mm in diameter with thin walls.
Pustule
MedGen UID:
488804
Concept ID:
C0241157
Finding
A vesicle filled with leukocytes
Hyperkeratosis
MedGen UID:
209030
Concept ID:
C0870082
Disease or Syndrome
Hyperkeratosis is thickening of the outer layer of the skin, the stratum corneum, which is composed of large, polyhedral, plate-like envelopes filled with keratin which are the dead cells that have migrated up from the stratum granulosum.
Hyperpigmented macules
MedGen UID:
375013
Concept ID:
C1842774
Finding
A hyperpigmented circumscribed area of change in normal skin color without elevation or depression of any size.

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.

Recent clinical studies

Etiology

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. [Epub ahead of print] PMID: 24603349
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. [Epub ahead of print] PMID: 17097401
DiCaudo DJ, Ortiz KJ, Mengden SJ, Lim KK
Arch Dermatol 2005 Jul;141(7):881-4. doi: 10.1001/archderm.141.7.881. PMID: 16027305
Singh RK
J Assoc Physicians India 2002 Oct;50:1322-3. PMID: 12568224
Clemmensen OJ, Menné T, Brandrup F, Thomsen K, Lange Wantzin G
Acta Derm Venereol 1989;69(1):52-8. PMID: 2563609

Diagnosis

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. [Epub ahead of print] PMID: 24603349
Yong AS, Lee KY, Murphy J, Phillips M, Rushbrook S, Garioch JJ
Postgrad Med J 2013 Dec;89(1058):731-2. Epub 2013 Sep 18 doi: 10.1136/postgradmedj-2013-132162. [Epub ahead of print] PMID: 24048149
Gaeta M, Mileto A, Musumeci O, Vaccaro M, Monici MC, Sergi M, Blandino A, Rodolico C
Skeletal Radiol 2011 Jun;40(6):779-82. Epub 2011 Feb 6 doi: 10.1007/s00256-011-1104-8. [Epub ahead of print] PMID: 21298430
Larsson LG, Baum J
J Rheumatol 1985 Oct;12(5):1000-3. PMID: 4087247
Callen JP
Dermatol Clin 1985 Jan;3(1):153-63. PMID: 3912087

Therapy

Kumar P, Das A, Mondal A
Indian Pediatr 2014 May;51(5):420. PMID: 24953595
Yong AS, Lee KY, Murphy J, Phillips M, Rushbrook S, Garioch JJ
Postgrad Med J 2013 Dec;89(1058):731-2. Epub 2013 Sep 18 doi: 10.1136/postgradmedj-2013-132162. [Epub ahead of print] PMID: 24048149
Grelle JL, Halloush RA, Khasawneh FA
BMJ Case Rep 2013 Aug 16;2013 doi: 10.1136/bcr-2013-200405. PMID: 23955987Free PMC Article
Gaeta M, Mileto A, Musumeci O, Vaccaro M, Monici MC, Sergi M, Blandino A, Rodolico C
Skeletal Radiol 2011 Jun;40(6):779-82. Epub 2011 Feb 6 doi: 10.1007/s00256-011-1104-8. [Epub ahead of print] PMID: 21298430
Larsson LG, Baum J
J Rheumatol 1985 Oct;12(5):1000-3. PMID: 4087247

Prognosis

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. [Epub ahead of print] PMID: 24603349
DiCaudo DJ, Ortiz KJ, Mengden SJ, Lim KK
Arch Dermatol 2005 Jul;141(7):881-4. doi: 10.1001/archderm.141.7.881. PMID: 16027305
Singh RK
J Assoc Physicians India 2002 Oct;50:1322-3. PMID: 12568224
Rahav G, Moses A, Michaeli J
Cutis 1989 Aug;44(2):157-9. PMID: 2758865
Gunawardena DA, Gunawardena KA, Ratnayaka RM, Vasanthanathan NS
Br J Dermatol 1975 Apr;92(4):363-73. PMID: 1156550

Clinical prediction guides

Anzalone CL, Cohen PR
Curr Opin Hematol 2013 Jan;20(1):26-35. doi: 10.1097/MOH.0b013e32835ad132. PMID: 23207661
DiCaudo DJ, Ortiz KJ, Mengden SJ, Lim KK
Arch Dermatol 2005 Jul;141(7):881-4. doi: 10.1001/archderm.141.7.881. PMID: 16027305
Masuda T, Abe Y, Arata J, Nagao Y
J Dermatol 1994 May;21(5):341-6. PMID: 8051321
Soppi E, Nousiainen T, Seppa A, Lahtinen R
Br J Haematol 1989 Sep;73(1):43-7. PMID: 2679862
Sweet RD
Br J Dermatol 1968 Dec;80(12):800-5. PMID: 5706798

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. [Epub ahead of print] PMID: 24714374
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

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