Acta Dermato-Venereologica 98-9CompleteContent | Page 8
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CLINICAL REPORT
Livedoid Vasculopathy: A French Observational Study Including
Therapeutic Options
Emma GARDETTE 1 , Philippe MOGUELET 2 , Jean David BOUAZIZ 3 , Dan LIPSKER 4 , Olivier DEREURE 5 , François LE PELLETIER 6 ,
Catherine LOK 7 , Thierry MAISONOBE 8 , Didier BESSIS 5 , Jacqueline CONARD 9 , Camille FRANCES 1 and Stéphane BARETE 10,11
Departments of Dermatology: 1 CHU Tenon, APHP, 3 CHU Saint Louis, APHP, Paris, 4 CHU de Strasbourg, Strasbourg, 5 CHU de Montpellier,
Montpellier, 7 CHU d’Amiens, Amiens, Departments of Histopathology: 2 CHU Tenon, APHP and 6 Groupe Hospitalier Pitié-Salpêtrière, APHP,
8
Department of Neurophysiology, Groupe Hospitalier Pitié-Salpêtrière, APHP, Paris, 9 Department of Hemostasis and Vascular Biology,
CHU Cochin, APHP, 10 Unit of Dermatology, Groupe Hospitalier Pitié-Salpêtrière, APHP, and 11 Inflammation-Immunopathology-Biotherapy
Department, Sorbonne Universités, UPMC Univ Paris, INSERM-UMRS 959, DHU i2B, Paris, France
Livedoid vasculopathy is a rare thrombotic cutaneous
disease. This observational study aimed to assess the
clinical and biological features of livedoid vasculopa-
thy and the efficacy of treatments. Patients enrolled
had typical livedoid vasculopathy both clinically and
histologically. Investigation of thrombophilia was per-
formed. Electromyography was undertaken in the pre-
sence of symptoms suggesting peripheral neuropathy.
Eighteen women and 8 men were included, with a
mean age of 35.5 years at onset. Twenty patients had
at least one thrombophilia factor. Ten patients had a
peripheral neuropathy with 2 of these patients demon-
strating a specific thrombo-occlusive vasculopathy on
muscle biopsy. Anticoagulation with low molecular
weight heparin was the most prescribed therapy and
was associated with the best outcome (effective in 14
patients). Eight patients had severe disease refractory
to anticoagulation and required intravenous immuno
globulins, producing a good response in 6 patients.
Key words: livedoid vasculopathy; peripheral neuropathy;
throm
bosis of dermal vessels; thrombophilia; low molecular
weight heparin; intravenous immunoglobulins.
Accepted May 4, 2018; Epub ahead of print May 8, 2018
Acta Derm Venereol 2018; 98: 842–847.
Corr: Dr. Stéphane Barete, MD, PhD, Department of Dermatology, Hôpital
Pitié-Salpêtrière, 47-83 Boulevard de l’Hôpital, FR-75013 Paris, France.
E-mail: [email protected].
L
ivedoid vasculopathy (LV) is a chronic disease mani-
festing as recurrent necrotic and painful lower limb
ulcerations. These resolve leaving atrophic porcelain-
white scars with surrounding telangiectasias known
as atrophie blanche (AB). The estimated incidence is
1:100,000 individuals, predominantly affecting young
to middle-aged females, with a sex ratio of 3:1 (1, 2).
Histopathology reveals a vaso-occlusive disorder with
intraluminal thrombosis of dermal vessels without leu-
kocytoclastic vasculitis (1, 3). The exact mechanism of
this entity is unknown but underlying thrombophilia with
abnormalities of coagulation or fibrinolysis, is observed
in up to 50% of patients (2, 4). Plasminogen activator
inhibitor-1 (PAI-1) is a major inhibitor of fibrinolysis.
The 4G/5G polymorphism of the PAI-1 promoter gene
results in enhanced transcription and the 4G allele is
doi: 10.2340/00015555-2965
Acta Derm Venereol 2018; 98: 842–847
SIGNIFICANCE
Livedoid vasculopathy is a chronic thrombotic disease of the
skin microcirculation resulting in painful ulcers mainly af-
fecting the lower legs. This study presents a French cohort
of patients with livedoid vasculopathy. It describes clinical,
histological characteristics and outcome of patients. It no-
tably shows a frequent thrombophilia background. Nerve
damage can be associated with cutaneous manifestations.
These data also confirm that heparin or oral anticoagulants
are able to achieve a complete response. Refractory cases
can be treated with intravenous immunoglobulins.
associated with increased PAI-1 levels. Impaired fib-
rinolysis through PAI-1 involvement was observed in
several studies, related to increased levels of PAI-1
antigen (5), enhanced activity (6) or 4G polymorphism
of the promoter gene (7).
Recently, a few cases have reported peripheral neuro-
pathy in association with LV, most often mononeuritis
multiplex (8–15).
Most treatments are based on anticoagulation (1, 3).
Several retrospective studies and case reports have shown
a good response to intravenous immunoglobulins (IVIG)
in refractory patients (16–19). However, in the absence
of large prospective controlled studies, there is no recom-
mendation on the dose or duration of medication and as
such the optimal therapeutic regimen is unknown.
We performed an observational multicenter study in
France reviewing the diagnosis and management of pa-
tients with LV. We aimed to assess the clinical and histo-
logical features of LV, to identify related coagulopathies
including plasma antigenic levels and 4G polymorphism
of PAI-1, neurological involvement, therapeutic mana-
gement and patient outcome.
METHODS
Study design and inclusion criteria
This observational study was conducted in 6 different French
dermatology departments between 2006 and 2015. Patients
included in the study presented with typical LV based on both
clinical symptoms (recurrent ulcers of the leg, livedo reticularis,
AB) and histology (thrombosis of dermal vessels). The exclusion
This is an open access article under the CC BY-NC license. www.medicaljournals.se/acta
Journal Compilation © 2018 Acta Dermato-Venereologica.