Livedoid Vasculopathy: A Review of 5 Clinical Cases
Livedoid vasculopathy is an unusual thrombo-occlusive dermatosis that manifests with punctiform erythemato-purpuric lesions and extremely painful ulcers, preferably in the lower third of the legs. We report five patients with livedoid vasculopathy, three men and two women, aged between 24 and 54 years, with no relevant personal background. The diagnosis was confirmed by biopsy in four cases. Study of thrombophilias revealed heterozygosity of the MTHFR gene in two cases. Treatment was diversified and included among others, venothropics, anticoagulants, antithrombotics, immunosuppressants and intravenous immunoglobulin. Only one patient has unsatisfactory control of the disease. Livedoid vasculopathy etiopathogenesis remains uncertain. It may occur in the context of hereditary or acquired thrombophilia, autoimmunity or neoplasms. These changes should be excluded, but most cases remain idiopathic, which contributes to the therapeutic difficulty. The authors intend to emphasize the importance of the Dermatologist in the diagnosis of a probably under-recognized disease that causes significant morbidity.
Criado PR, Rivitti EA, Sotto MN, Valente NY, Aoki
V, Carvalho JF, et al. Livedoid vasculopathy: an intringuing
cutaneous disease. An Bras Dermatol.
Poletti NM, Moreno González JL, Santacruz Torres
A. Vasculopatía livedoide: significado actual. Comunicación
de dos casos. Dermatología Rev Mex.
Papi M, Didona B, De Pita O, Frezzolini A, Di Giulio S,
De Matteis W, et al. Livedo vasculopathy vs small vessel
cutaneous vasculitis: cytokine and platelet P-selectin studies.
Arch Dermatol. 1998;134:447-52.
Feldaker M, Hines EA Jr., Kierland RR. Livedo reticularis
with summer ulcerations. AMA Arch Derm.
Di Giacomo TB, Hussein TP, Souza DG, Criado PR. Frequency
of thrombophilia determinant factors in patients
with livedoid vasculopathy and treatment with anticoagulant
drugs--a prospective study. J Eur Acad Dermatol
Gray HR, Graham JH, Johnson W, Burgoon CF Jr.
Atrophie blanche: periodic painful ulcers of lower extremities.
A clinical and histopathological entity. Arch
Alavi A, Hafner J, Dutz JP, Mayer D, Sibbald RG, Criado
PR, et al. Livedoid vasculopathy: an in-depth analysis
using a modified Delphi approach. J Am Acad Dermatol.
Alix JJ, Hadjivassiliou M, Ali R, Slater D, Messenger AG,
Rao DG. Sensory ganglionopathy with livedoid vasculopathy
controlled by immunotherapy. Muscle Nerve.
Toth C, Trotter M, Clark A, Zochodne D. Mononeuropathy
multiplex in association with livedoid vasculitis.
Muscle Nerve. 2003;28:634-9.
Kim JE, Park SY, Sinn DI, Kim SM, Hong YH, Park KS, et
al. Ischemic neuropathy associated with livedoid vasculitis.
J Clin Neurol. 2011;7:233-6.
Maessen-Visch MB, Koedam MI, Hamulyak K,
Neumann HA. Atrophie blanche. Int J Dermatol.
Kutzner GH. Therapeutic use of low molecular weight
heparin for capillaritis alba. Phlebologie. 2008;37:259–
Woo KY, Abbott LK, Librach L. Evidence-based approach
to manage persistent wound-related pain. Curr Opin
Support Palliat Care. 2013;7:86-94.
Hairston BR, Davis MD, Pittelkow MR, Ahmed I. Livedoid
vasculopathy: further evidence for procoagulant pathogenesis.
Arch Dermatol. 2006;142:1413-8.
Deng A, Gocke CD, Hess J, Heyman M, Paltiel M, Gaspari
A. Livedoid vasculopathy associated with plasminogen
activator inhibitor-1 promoter homozygosity
(4G/4G) treated successfully with tissue plasminogen
activator. Arch Dermatol. 2006;142:1466-9.
Tuchinda C, Leenutaphong V, Sudtim S, Lim HW. Refractory
livedoid vasculitis responding to PUVA: a report
of four cases. Photodermatol Photoimmunol Photomed.
Kreuter A, Gambichler T, Breuckmann F, Bechara FG,
Rotterdam S, Stucker M, et al. Pulsed intravenous immunoglobulin
therapy in livedoid vasculitis: an open trial
evaluating 9 consecutive patients. J Am Acad Dermatol.
Yang CH, Ho HC, Chan YS, Liou LB, Hong HS, Yang LC.
Intractable livedoid vasculopathy successfully treated
with hyperbaric oxygen. Br J Dermatol. 2003;149:647-
Rampf J, Sunderkotter C, Hirschfeld G, Scharffetter-
-Kochanek K, Weiss JM. Methylenetetrahydrofolate
reductase polymorphism associated with moderate
hyperhomocysteinaemia in a patient with livedo vasculopathy:
treatment with vitamin supplementation
and low molecular weight heparin. Br J Dermatol.
Copyright (c) 2018 Journal of the Portuguese Society of Dermatology and Venereology
This work is licensed under a Creative Commons Attribution 4.0 International License.
All articles in this journal are Open Access and meet the requirements of funding bodies or academic institutions. Each article published in the Journal is published under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License (CC BY-NC-ND 4.0). Articles can be read, downloaded, printed, and shared.
Submission of an article for publication implies the authors’ consent to publication under the applicable Creative Commons license and the terms and conditions of the Publisher’s Licensing Agreement.
It is the author's responsibility to obtain permission to reproduce illustrations, tables, etc. from other publications.
Upon acceptance of an article, authors will be asked to complete a 'Journal Publishing Agreement'. An e-mail will be sent to the corresponding author confirming receipt of the manuscript together with a 'Journal Publishing Agreement' form or a link to the online version of this agreement.
Author rights: As an author you (or your employer or institution) have certain rights to reuse your work.
The Journal reserves the right to use plagiarism detection software on any submitted material.