Lymphocytic Thrombophilic Arteritis

Macular Lymphocytic Arteritis: Three Cases Questioning Its Classification as Primary Lymphocytic Vasculitis

“…Histopathological findings support that by injuring endothelial cells, lymphocytes are the cause and not the consequence of the fibrin deposits in the vessel walls [16]. Antiphospholipid and antinuclear antibodies are found positive in 30% of reported cases [1,2,3,8,13]. However, none of these cases exhibited systemic connective tissue disease or met the criteria for antiphospholipid syndrome diagnosis.…”

“…In the literature, we found 22 cases of MLA [1,2,3,4,5,6,8,9,10,11,12,13,14] (PubMed search for ‘macular arteritis', ‘MLA' and ‘LTA', limits January 2003 to February 2013). This disease mostly affects women (median age: 34 years) often with North African origins.…”

“…A reticulated pattern (with a typical net-like pattern) [1,6,8] or more rarely a true segmental livedo racemosa (with irregular, broken, segmented shapes) may also be observed [2,9]. The term LTA proposed by Lee et al [2] highlights the characteristic histopathological features of the disease, namely the infiltration of medium-sized arteriole walls with lymphocytes coupled with hyalinized fibrin rings in the vessels walls. The dense lymphocytic infiltrate is observed in all cases, whereas neutrophils are absent or rare.…”

“…Macular arteritis [1], lymphocytic thrombophilic arteritis (LTA) [2] and macular lymphocytic arteritis (MLA) [3] (all these terms describe in fact the same disease) constitute a rare and recently described form of cutaneous lymphocytic vasculitis with unique clinicopathological features. It is characterized clinically by erythematous or hyperpigmented, reticulated patches and macules arising preferentially on the lower limbs and histopathologically by a cutaneous lymphocytic medium-sized vessel arteritis.…”

“…The nosology of MLA is still being debated. According to Lee et al [2], it should not be classified into any of the known categories of vasculitis but rather should be individualized as a distinct entity. Conversely, for some other authors [4,5,6,7], MLA could represent an indolent form of cutaneous polyarteritis nodosa (C-PAN).…”