Análise da freqüência de trombofilia em pacientes com atrofia branca de Milian

Abstract

FUNDAMENTOS - Atrofia branca de Milian ou vasculopatia livedóide é entidade clinicopatológica rara, cuja patogênese não é completamente compreendida. OBJETIVOS - Avaliar casos de atrofia branca de Milian para verificar a prevalência de diversas trombofilias. MATERIAL E MÉTODOS - Quatorze pacientes foram submetidos a exames laboratoriais incluindo pesquisa de fator V (Leiden), protrombina mutante, dosagem de antitrombina, proteína S e C, pesquisa de anticorpos anticardiolipina e anticoagulante lúpico, dosagem de homocisteína e pesquisa da mutação da metilenotetraidrofolatoredutase. RESULTADOS - Dos nove doentes cujos critérios de inclusão foram preenchidos para análise da freqüência de trombofilia, foram encontrados quatro com fatores relacionados à trombofilia: deficiência da antitrombina (um caso), deficiência da proteína S (um caso), mutação da metilenotetraidrofolatoredutase com hiperhomocisteinemia (um caso) e presença de anticorpo anticardiolipina (um caso). CONCLUSÃO - Apesar de este estudo não apresentar casuística que possibilite a comparação com a população geral, os dados sugerem a presença de eventos geradores de trombofilia nesses doentes, contribuindo para adoção sistemática de um protocolo de investigação de trombofilia nos doentes portadores de vasculopatia livedóide no Brasil.

Vasculopatia livedoide: uma doença cutânea intrigante

“…9 Although there is no consensus, most authors who study this disease currently consider this A B C D FIGURE 1: Livedoid Vasculopathy -lesions spread on the legs, especially on the ankles and feet. Punctate or lenticular ulcerations (A, B, C and D) coexist with larger ulcerations due to confluence of the former, surrounded by an erythematous and purpuric halo, sometimes covered with melicerous crusts (A, B and C) and sometimes with hematic crusts and necrotic slough (eschar) (D).…”

“…9,[12][13][14][15][16] Changes in the blood flow not only include venous insufficiency, but also diseases that lead to hyperviscosity syndrome such as chronic myelogenous leukemia, cryoglobulinemia and heavy chain disease. 9,17 Hypercoagulable states are rare situations and still partially unknown. They are currently classified as hereditary -which include genetic defects of the coagulation cascade and of fibrinolysis -and acquired.…”

“…21 Among the several reported associations between LV and hypercoagulable states are the following: protein C deficiency, factor V (Leiden) mutation, prothrombin gene mutation, hyperhomocysteinemia, increased levels of lipoprotein(a) and antithrombin deficiency. 8,9,14,20,[30][31][32][33][34][35][36] Consequently, given the heterogeneity of the etiopathogenic factors clinically involved in LV, we propose to classify it according to its etiopathogenesis into primary (idiopathic) or secondary to a known event or condition, with the latter being subdivided into four groups: (i) endothelial damage, (ii) changes in blood flow, (iii) states related to thrombophilias, and (iv) mixed etiopathogenesis.…”

“…17 However, we disagree with this point of view due to the large differential diagnosis involved in the disease as well as to the fact that biopsy only caused difficult-to-heal ulcers among our patients on few occasions. 8,9,58 The initial challenge in the histopathological diagnosis of LV is to obtain an adequate skin sample, which represents from the epidermis to the dermo- 4: System of coagulation, natural anticoagulation and fibrinolysis. Coagulation: after vessel wall damage, the coagulation system produces a hemostatic plug through an interaction between the sub-endothelium, platelets and plasma coagulation factors.…”

“…It especially affects the age group between 15 and 50 years old (mean 32 years) and females at a proportion of 2.4 to 3 women for every man. [6][7][8][9] LV was originally described as a clinical manifestation of vasculitis. However, at present, the main physiopathogenic mechanism considered is a vasoocclusive phenomenon due to intraluminal thrombosis of dermal venules 1 .…”