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Livedoid vasculopathy

MedGen UID:
575376
Concept ID:
C0343081
Disease or Syndrome
Synonyms: Atrophic Blanche, Idiopathic; Blanches, Idiopathic Atrophic; Idiopathic Atrophic Blanche; Livedo Reticularis with Summer Ulceration; Livedo Vasculitides; Livedo Vasculitis; Livedoid Vasculitis; Livedoid Vasculopathies; Livedoid Vasculopathy; Vasculitis, Livedo; Vasculitis, Livedoid; Vasculopathy, Livedoid; White Atrophy
 
Monarch Initiative: MONDO:0025514
Orphanet: ORPHA542643

Definition

A rare vascular skin disease characterized by recurrent focal non-inflammatory thrombosis of dermal venulae, predominantly of the lower extremities, resulting in a cutaneous response manifested as pruritus and painful papules and erythematous plaques. The lesions evolve into hemorrhagic vesicles or bullae, which rupture and turn into painful ulcers merging into reticulate, confluent, geometric, and painful ulcerations. During a period of a few months, the ulcerations change to porcelain-white atrophic scars with punctate telangiectasia (so-called atrophie blanche). In active disease, lesions in different stages coexist. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVLivedoid vasculopathy

Professional guidelines

PubMed

Song X, Tu P
JAMA Dermatol 2022 May 1;158(5):587-589. doi: 10.1001/jamadermatol.2022.0241. PMID: 35353137
Micieli R, Alavi A
JAMA Dermatol 2018 Feb 1;154(2):193-202. doi: 10.1001/jamadermatol.2017.4374. PMID: 29141075
Vasudevan B, Neema S, Verma R
Indian J Dermatol Venereol Leprol 2016 Sep-Oct;82(5):478-88. doi: 10.4103/0378-6323.183635. PMID: 27297279

Recent clinical studies

Etiology

Apti Sengun O, Ergun T, Guctekin T, Alibaz Oner F
Microvasc Res 2023 Nov;150:104591. Epub 2023 Aug 4 doi: 10.1016/j.mvr.2023.104591. PMID: 37543163
Gao Y, Jin H
Int Wound J 2023 May;20(5):1498-1505. Epub 2022 Nov 4 doi: 10.1111/iwj.14004. PMID: 36333819Free PMC Article
Gao Y, Jin H
Dermatol Ther 2021 Sep;34(5):e15051. Epub 2021 Jul 18 doi: 10.1111/dth.15051. PMID: 34197012
Micieli R, Alavi A
JAMA Dermatol 2018 Feb 1;154(2):193-202. doi: 10.1001/jamadermatol.2017.4374. PMID: 29141075
Criado PR, Espinell DP, Barreto P, Di Giacomo TH, Sotto MN
Med Hypotheses 2015 Nov;85(5):670-4. Epub 2015 Aug 29 doi: 10.1016/j.mehy.2015.08.009. PMID: 26328479

Diagnosis

Gaspar P, Sciascia S, Tektonidou MG
Rheumatology (Oxford) 2024 Feb 6;63(SI):SI24-SI36. doi: 10.1093/rheumatology/kead571. PMID: 38320589Free PMC Article
Soulages A, Maisonobe T, Auzou P, Petit A, Allenbach Y, Barète S, Skopinski S, Ribeiro E, Jullié ML, Lamant L, Brevet F, Soulages X, Vallat JM, Martin-Négrier ML, Solé G, Duval F, Carla L, Le Masson G, Mathis S
J Neurol 2022 Jul;269(7):3779-3788. Epub 2022 Feb 15 doi: 10.1007/s00415-022-11007-z. PMID: 35166926
Micieli R, Alavi A
JAMA Dermatol 2018 Feb 1;154(2):193-202. doi: 10.1001/jamadermatol.2017.4374. PMID: 29141075
Zampeli E, Moutsopoulos HM
Arthritis Rheumatol 2017 Oct;69(10):2096. Epub 2017 Aug 15 doi: 10.1002/art.40153. PMID: 28511282
Llamas-Velasco M, Alegría V, Santos-Briz Á, Cerroni L, Kutzner H, Requena L
Am J Dermatopathol 2017 Sep;39(9):637-662. doi: 10.1097/DAD.0000000000000766. PMID: 27759698

Therapy

Gao Y, Jin H
Dermatol Ther 2021 Sep;34(5):e15051. Epub 2021 Jul 18 doi: 10.1111/dth.15051. PMID: 34197012
Rujitharanawong C, Chularojanamontri L, Trakanwittayarak S, Pinkaew S, Nuttawong S
Dermatol Ther 2021 Jan;34(1):e14569. Epub 2020 Nov 29 doi: 10.1111/dth.14569. PMID: 33219608
Micieli R, Alavi A
JAMA Dermatol 2018 Feb 1;154(2):193-202. doi: 10.1001/jamadermatol.2017.4374. PMID: 29141075
Vasudevan B, Neema S, Verma R
Indian J Dermatol Venereol Leprol 2016 Sep-Oct;82(5):478-88. doi: 10.4103/0378-6323.183635. PMID: 27297279
Kerk N, Goerge T
J Dtsch Dermatol Ges 2013 May;11(5):407-10. Epub 2013 Feb 26 doi: 10.1111/ddg.12064. PMID: 23437985

Prognosis

Gao Y, Jin H
Australas J Dermatol 2022 Aug;63(3):e200-e205. Epub 2022 May 30 doi: 10.1111/ajd.13884. PMID: 35635484
Rujitharanawong C, Chularojanamontri L, Trakanwittayarak S, Pinkaew S, Nuttawong S
Dermatol Ther 2021 Jan;34(1):e14569. Epub 2020 Nov 29 doi: 10.1111/dth.14569. PMID: 33219608
Micieli R, Alavi A
JAMA Dermatol 2018 Feb 1;154(2):193-202. doi: 10.1001/jamadermatol.2017.4374. PMID: 29141075
Martinez-Mera C, Fraga J, Capusan TM, Herrero-Moyano M, Godoy-Trapero A, Gonzales Perez Y, Llamas-Velasco M
G Ital Dermatol Venereol 2017 Dec;152(6):615-637. Epub 2017 Jul 28 doi: 10.23736/S0392-0488.17.05727-3. PMID: 28845953
Kerk N, Goerge T
Vasa 2013 Sep;42(5):317-22. doi: 10.1024/0301-1526/a000296. PMID: 23989066

Clinical prediction guides

Soulages A, Maisonobe T, Auzou P, Petit A, Allenbach Y, Barète S, Skopinski S, Ribeiro E, Jullié ML, Lamant L, Brevet F, Soulages X, Vallat JM, Martin-Négrier ML, Solé G, Duval F, Carla L, Le Masson G, Mathis S
J Neurol 2022 Jul;269(7):3779-3788. Epub 2022 Feb 15 doi: 10.1007/s00415-022-11007-z. PMID: 35166926
Gao Y, Jin H
Dermatol Ther 2021 Sep;34(5):e15051. Epub 2021 Jul 18 doi: 10.1111/dth.15051. PMID: 34197012
Rujitharanawong C, Chularojanamontri L, Trakanwittayarak S, Pinkaew S, Nuttawong S
Dermatol Ther 2021 Jan;34(1):e14569. Epub 2020 Nov 29 doi: 10.1111/dth.14569. PMID: 33219608
Micieli R, Alavi A
JAMA Dermatol 2018 Feb 1;154(2):193-202. doi: 10.1001/jamadermatol.2017.4374. PMID: 29141075
Kerk N, Goerge T
Vasa 2013 Sep;42(5):317-22. doi: 10.1024/0301-1526/a000296. PMID: 23989066

Recent systematic reviews

Gao Y, Jin H
Dermatol Ther 2021 Sep;34(5):e15051. Epub 2021 Jul 18 doi: 10.1111/dth.15051. PMID: 34197012
Gao Y, Jin H
Int Wound J 2021 Oct;18(5):616-625. Epub 2021 Mar 8 doi: 10.1111/iwj.13563. PMID: 33686673Free PMC Article
Micieli R, Alavi A
JAMA Dermatol 2018 Feb 1;154(2):193-202. doi: 10.1001/jamadermatol.2017.4374. PMID: 29141075

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