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Anais Brasileiros de Dermatologia

Print version ISSN 0365-0596
On-line version ISSN 1806-4841

An. Bras. Dermatol. vol.78 no.6 Rio de Janeiro Nov./Dec. 2003 



Livedoid vasculopathy or vasculitis?*



Maurício ZaniniI; Cláudio WulkanII; Danielle BertinoIII; Lúcia ItoIV

IIIResident in Dermatologist
IVAssistant Professor in Dermatology





Livedoid vasculitis or atrophie blanche is not a true vasculitis. It is believed to be a primary disturbance in fibrinolysis that establishes an occlusive vasculopathy. As it is not a vasculitis, use of the term 'livedoid vasculopathy' is preferable. Moreover, this physiopathological definition reflects new therapeutic perspectives.

Key words: atrophy; pathology; vasculitis; blood vessels.




Small vessel cutaneous vasculitis consists of a heterogeneous group of affections. It may be primarily cutaneous or part of a systemic disturbance. It includes three main entities, namely: leukocytoclastic vasculitis, septic vasculitis and livedoid vasculitis. The latter is the least common form as well as the least understood.1 This vasculitis group mainly affects post-capillary small vessels. It has a condition common to cutaneous vasculitis, which is characteristically manifested as purple and palpable.2

Livedoid vasculitis, an affection first described by Milian in 1929 as atrophie blanche, segmental and in plaques, is a chronic disease that may be resolved within a few years or persist over one's entire lifetime with remissions and aggravations. It is an uncommon affection predominantly afflicting females.1,3

The diagnosis is clinical. The condition begins with macules and purple papules having a contusiform feature progressing into very painful ulcers. Hemorrhagic blisters may accompany the transition of macules into ulcers. Later, stellar, atrophic and white-marble colored cicatricial plaques emerge. Lesions predominate on the lower limbs, particularly on the lower third part of the legs and the ankles. These lesions tend to become aggravated during summertime. This is also why the lesions are called vasculitis with summer ulceration. It is worth pointed out that this affection has no relation to reticular livedo.1,3,4

In the classic histopathologic description of livedoid vasculitis, a fibrinoid deposition may be observed in the vascular wall in association with intraluminal thrombosis and mixed inflammatory infiltrate. The initial inflammatory infiltrate is predominantly neutrophilic, while the lymphocytes are more marked in the late phase.1

Vasculitis differs from obstructive vasculopathy insofar as it determines ischemic-thrombotic alterations resulting from a primary immunologic-inflammatory process. In obstructive vasculopathy, immunologic and/or inflammatory alterations are secondary.1,2

The concept established many years ago according to which livedoid vasculitis would be a true vasculitis is no longer accepted by many authors. It is believed to be a primary fibrinolytic disturbance in the endothelium of the affected vessels, which determines a state of local hypercoagulability. The first histopathologic alteration to be observed in livedoid vasculitis is the deposit of fibrinous material in the blood vessel wall and lumen. The either total or significant absence of inflammatory infiltrate or leukocytoclasia counters the idea of vasculitis. The immunologic complex, basically found in late-onset lesions, consists of a secondary event. As such, livedoid vasculitis is an occlusive vasculopathy resulting from a thrombotic process of the small or medium caliber dermal vessels.3,4,5 Lefebvre et al. define livedoid vasculitis as a thrombotic disease affecting the superficial and deep dermal vessels. It is physiologically characterized by hyalinization of the vascular wall, endothelial proliferation, fibrin deposits and, finally, by the formation of intraluminal thromboses.6

Livedoid vasculopathy may be a primary focal or secondary disturbance to a coagulation disturbance, such as, for example, protein C deficiency and antiphospholipid syndrome.4,5

Therefore, based on the data exposed in this paper, the authors recommend using the term 'livedoid vasculopathy'-and no longer, 'livedoid vasculitis'. An acceptable alternative would have been Milian's previously baptized term, 'white atrophy' (atrophie blanche). On the other hand, not only do these facts imply the wish to establish a more adequate term by which to refer to the affection; they also show new investigative and therapeutic implications based on novel physiopathological concepts.



1. Busam KJ, Barksdale SK, Barnhill RL. Vasculitis and related disorders. In: Barnhill RL. Textbook of dermatopathology. New York, McGraw-Hill co., 1998: 196.        [ Links ]

2. Gibson LE, Daniel Su WP. Cutaneous vasculitis. Rheum Dis Clin North Am 1990; 16:309-324.        [ Links ]

3. Papi M, Didona B, De Pita O et al. Livedo vasculopathy versus small vessel cutaneous vasculitis: cytokine and platelet P-selectin studies. Arch Dermatol 1998; 134(4): 447-52.        [ Links ]

4. Acland KM, Darvay A, Wakelin SH. Russell-Jones R Livedoid vasculitis: a manifestation of the antiphospholipid syndrome? Br J Dermatol 1999; 140(1): 131-5.        [ Links ]

5. Boyvat A, Kundakci N, Babikir MO, Gurgey E. Livedoid vasculopathy associated with heterozygous protein C deficiency. Br J Dermatol 2000; 143(4): 840-2.        [ Links ]

6. Lefebvre P, Motte S, Wautrecht JC, Cornez N, Delplace J, Dereume JP. Livedo vasculitis: report of a case. J Mal Vasc 1996; 21(1): 50-3.        [ Links ]



Correspondence to
Maurício Zanini
Rua Vicente de Carvalho, 198
Santo André SP 09060-590
Tel.: (11) 4992-7724

Received in April, 30th of 2002
Approved by the Consultive Council and accepted for publication in April, 04th of 2003



* Work done at Prof. Dr. Luiz Henrique Camargo Paschoal Dermatology Service, Faculty of Medicine, Fundação do ABC Santo André, Sao Paulo state, Brazil

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