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Vibratory urticaria(VBU)

MedGen UID:
510413
Concept ID:
C0157743
Disease or Syndrome
Synonym: VBU
SNOMED CT: Vibratory urticaria (51247001)
 
Gene (location): ADGRE2 (19p13.12)
 
HPO: HP:0410138
Monarch Initiative: MONDO:0006618
OMIM®: 125630

Definition

Autosomal dominant vibratory urticaria is characterized by localized hives and systemic manifestations in response to dermal vibration, with coincident degranulation of mast cells and increased histamine levels in serum (Boyden et al., 2016). [from OMIM]

Clinical features

From HPO
Flushing
MedGen UID:
5234
Concept ID:
C0016382
Sign or Symptom
Recurrent episodes of redness of the skin together with a sensation of warmth or burning of the affected areas of skin.
Urticaria
MedGen UID:
22587
Concept ID:
C0042109
Disease or Syndrome
Raised, well-circumscribed areas of erythema and edema involving the dermis and epidermis. Urticaria is intensely pruritic, and blanches completely with pressure.
Facial erythema
MedGen UID:
65986
Concept ID:
C0239488
Finding
Redness of the skin of the face, caused by hyperemia of the capillaries in the lower layers of the skin.
Dermatographic urticaria
MedGen UID:
488854
Concept ID:
C0343065
Disease or Syndrome
An exaggerated whealing tendency when the skin is stroked, that is, formation of red, itchy bumps and lines on the skin as a result of pressure on the skin (for instance, stroking the skin with a pen or tongue depressor).

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVVibratory urticaria

Professional guidelines

PubMed

Kulthanan K, Ungprasert P, Tapechum S, Rujitharanawong C, Kiratiwongwan R, Munprom K, Terhorst-Molawi D, Maurer M
J Allergy Clin Immunol Pract 2021 Feb;9(2):971-984. Epub 2020 Sep 19 doi: 10.1016/j.jaip.2020.09.009. PMID: 32961312
Maurer M, Metz M, Brehler R, Hillen U, Jakob T, Mahler V, Pföhler C, Staubach P, Treudler R, Wedi B, Magerl M
J Allergy Clin Immunol 2018 Feb;141(2):638-649. Epub 2017 Jul 24 doi: 10.1016/j.jaci.2017.06.032. PMID: 28751232

Recent clinical studies

Etiology

Maurer M, Fluhr JW, Khan DA
J Allergy Clin Immunol Pract 2018 Jul-Aug;6(4):1119-1130. doi: 10.1016/j.jaip.2018.03.007. PMID: 30033913

Diagnosis

Kulthanan K, Ungprasert P, Tapechum S, Rujitharanawong C, Kiratiwongwan R, Munprom K, Terhorst-Molawi D, Maurer M
J Allergy Clin Immunol Pract 2021 Feb;9(2):971-984. Epub 2020 Sep 19 doi: 10.1016/j.jaip.2020.09.009. PMID: 32961312
Pozderac I, Lugović-Mihić L, Artuković M, Stipić-Marković A, Kuna M, Ferček I
Acta Dermatovenerol Alp Pannonica Adriat 2020 Sep;29(3):141-148. PMID: 32975301
Naranjo AN, Bandara G, Bai Y, Smelkinson MG, Tobío A, Komarow HD, Boyden SE, Kastner DL, Metcalfe DD, Olivera A
J Invest Dermatol 2020 Nov;140(11):2210-2220.e5. Epub 2020 Mar 25 doi: 10.1016/j.jid.2020.03.936. PMID: 32222457Free PMC Article
Maurer M, Fluhr JW, Khan DA
J Allergy Clin Immunol Pract 2018 Jul-Aug;6(4):1119-1130. doi: 10.1016/j.jaip.2018.03.007. PMID: 30033913
Abajian M, Schoepke N, Altrichter S, Zuberbier T, Maurer M
Immunol Allergy Clin North Am 2014 Feb;34(1):73-88. Epub 2013 Oct 28 doi: 10.1016/j.iac.2013.09.010. PMID: 24262690

Therapy

Naranjo AN, Bandara G, Bai Y, Smelkinson MG, Tobío A, Komarow HD, Boyden SE, Kastner DL, Metcalfe DD, Olivera A
J Invest Dermatol 2020 Nov;140(11):2210-2220.e5. Epub 2020 Mar 25 doi: 10.1016/j.jid.2020.03.936. PMID: 32222457Free PMC Article
Le QT, Lyons JJ, Naranjo AN, Olivera A, Lazarus RA, Metcalfe DD, Milner JD, Schwartz LB
J Exp Med 2019 Oct 7;216(10):2348-2361. Epub 2019 Jul 23 doi: 10.1084/jem.20190701. PMID: 31337736Free PMC Article
Boyden SE, Desai A, Cruse G, Young ML, Bolan HC, Scott LM, Eisch AR, Long RD, Lee CC, Satorius CL, Pakstis AJ, Olivera A, Mullikin JC, Chouery E, Mégarbané A, Medlej-Hashim M, Kidd KK, Kastner DL, Metcalfe DD, Komarow HD
N Engl J Med 2016 Feb 18;374(7):656-63. Epub 2016 Feb 3 doi: 10.1056/NEJMoa1500611. PMID: 26841242Free PMC Article

Prognosis

Boyden SE, Desai A, Cruse G, Young ML, Bolan HC, Scott LM, Eisch AR, Long RD, Lee CC, Satorius CL, Pakstis AJ, Olivera A, Mullikin JC, Chouery E, Mégarbané A, Medlej-Hashim M, Kidd KK, Kastner DL, Metcalfe DD, Komarow HD
N Engl J Med 2016 Feb 18;374(7):656-63. Epub 2016 Feb 3 doi: 10.1056/NEJMoa1500611. PMID: 26841242Free PMC Article

Clinical prediction guides

Gatica-Ortega ME, Sánchez-Matas I, Sánchez-Muñoz L, Pastor-Nieto MA
Occup Med (Lond) 2022 Dec 7;72(8):566-569. doi: 10.1093/occmed/kqac092. PMID: 36130167
Boyden SE, Desai A, Cruse G, Young ML, Bolan HC, Scott LM, Eisch AR, Long RD, Lee CC, Satorius CL, Pakstis AJ, Olivera A, Mullikin JC, Chouery E, Mégarbané A, Medlej-Hashim M, Kidd KK, Kastner DL, Metcalfe DD, Komarow HD
N Engl J Med 2016 Feb 18;374(7):656-63. Epub 2016 Feb 3 doi: 10.1056/NEJMoa1500611. PMID: 26841242Free PMC Article

Recent systematic reviews

Kulthanan K, Ungprasert P, Tapechum S, Rujitharanawong C, Kiratiwongwan R, Munprom K, Terhorst-Molawi D, Maurer M
J Allergy Clin Immunol Pract 2021 Feb;9(2):971-984. Epub 2020 Sep 19 doi: 10.1016/j.jaip.2020.09.009. PMID: 32961312

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