Forum for Nordic Dermato-Venereology Nr 3, 2018 | Page 15

Nicolas Kluger – Livedo reticularis and livedo racemosa
Table III. Non-exhaustive list of diseases responsible for livedo
racemosa (1–2)
Vasculitis
• Any vasculitis can be responsible for a livedo racemosa (vasculitis
associated with a connectivitis, systemic and cutaneous polyarteritis
nodosa, mixed cryoglobulinaemia type II and III, deficiency of
ADA2, etc.)
Thrombosis
• Coagulation disorders (protein S, protein C, anti-thrombin III
deficiencies, factor V Leiden mutation; factor II (prothrombin)
mutation, antiphospholipid syndrome, heparin and anti-vitamin K
necrosis, disseminated intravascular coagulation, etc.)
• Myeloproliferative disorders (thrombocythaemia, polycythaemia vera)
• Cryoproteins (type I cryoglobulinaemia, cryofibrinogenaemia, cold
agglutinins, paraproteinaemia)
• Primary oxaliuria (oxalosis)
• Cutaneous calcifications (calciphylaxis, primary hyperparathyroidism).
• Livedoid vasculopathy
• Sneddon’s syndrome
Embolization disorders
• Fibrino-cruorinal emboli (intracardiac or vascular thrombus)
• Septic embolization (intracardiac or vascular)
• Cholesterol emboli
• Cardiac myxoma
• Gas emboli (decompression sickness)
• Fat emboli (severe trauma and multiple bone fractures)
• Tumoural and metastatic emboli (lymphoma; breast)
• Nicolau’s livedoid dermatitis (embolia cutis medicamentosa) and
accidental intra-arterial injection (intramuscular; sclerotherapy)
• Intravenous drug abuse (buprenorphine), cocaine
of possible causes of pathological livedo racemosa is wide. A
non-exhaustive list of causes of livedo racemosa is summarized
in Table III. PubMed and other databases should be consulted
for more in-depth references regarding each disease (13–15).
Management of livedo racemosa
The management of livedo racemosa is directly related to
the underlying disease. Management will rely on correction
of the cause, the adjunction of corticosteroids and other im-
munosuppressive treatments, anticoagulation, antiplatelet or
antivitamin K agents. An algorithm for the management of
livedo racemosa is suggested in Fig. 6.
References
1. Kluger N, Molinari E, Francès C. Livedo in adults. Ann Dermatol
Venereol 2005; 132: 710–717.
2. Gibbs MB, English JC, Zirwas MJ. Livedo reticularis: an update. J
Am Acad Dermatol 2005; 52: 1009–1019.
3. Herrero C, Guilabert A, Mascaró-Galy JM. Actas Dermosifiliogr
2008; 99: 598–607.
4. Parsi K, Partsch H, Rabe E, Ramelet AA. Reticulate eruptions. Part
1: Vascular networks and physiology. Australas J Dermatol 2011;
52: 159–166.
5. Kluger N, Merlet-Albrand S, Guillot B, Bessis D. A pigmented ery-
thema of the thighs. Rev Med Interne 2009; 30: 349–350.
6. Bachmeyer C, Bensaid P, Bégon E. Laptop computer as a modern
cause of erythema ab igne. J Eur Acad Dermatol Venereol 2009;
23: 736–737.
How to manage a livedo racemosa in an adult
7. Sangle SR, D’Cruz DP. Livedo reticularis: an enigma. Isr Med
Assoc J 2015; 17: 104–107.
Necrotic Livedo
Infiltrated Livedo
Non necrotic,
8.
Georgesco G, Lorette G. Cutis marmorata telangiectatica
non infiltrated,
congenita. Presse Med 2010; 39: 495–498.
« pure » Livedo
9. Ehrmann S. Ein neues Gefaβsymptom bei Lues. Wien Med
Full thickness biopsy
Wochenschr 1907; 57: 777–782.
10. Parsi K,Partsch H, Rabe E, Ramelet AA. Reticulate eruptions.
Full thickness
Thrombosis
Emboli
Vasculitis
biopsy
Part 2: Historical perspectives, morphology, terminology and
classification. Australas J Dermatol 2011; 52: 237–244.
Complete physical examination:
11. Kluger N, Sirvente J, Rigau V, Guillot B. Extensive thrombotic
Sneddon
neurological signs, cognitive signs, HTA,
purpura revealing multiple myeloma associated – type I cry-
valvulopathy…
oglobulinemia. Br J Haematol 2011; 154: 1.
WBC, platelets, hemostasis, APL, protein C,
12. Kluger N, Fabre S, Durand L, Guillot B. Spontaneous cutaneous
APL +
APL –
protein S, AT III, protein Z, homocysteinemia,
cholesterol embolism. Presse Med 2010; 39: 738–739.
Antiplatelets?
AVK
dysfibrinogenia,….
13. Saric M, Kronzon I. Cholesterol embolization syndrome. Curr
Other measures
Opin Cardiol 2011; 26: 472–479.
Weight loss, lipid-lowering
Cardiac echography
therapies, blood pressure control,
14.
Criado PR, Rivitti EA, Sotto MN, de Carvalho JF. Livedoid
diabetes management
In case of neurological symptoms or
Avoidance of : Smoking, oestrogen
vasculopathy as a coagulation disorder. Autoimmun Rev
contraceptive
valvulopathy, cerebral MRI
2011; 10: 353–360.
Fig. 6. Algorithm for the management of livedo in adults. APL: circulating
15. Francès C, Papo T, Wechsler B, Laporte JL, Biousse V, Piette
antiphospholipid; AT III: Anti-thrombin III; AVK: anti-vitamin K treatment;
JC. Sneddon syndrome with or without antiphospholipid
HTA: health technology assessments; MRI: magnetic resonance imaging; WBC:
antibodies. A comparative study in 46 patients. Medicine
white blood cells.
(Baltimore) 1999; 78: 209–219.
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