Arterite linfocítica trombofílica: uma nova entidade no espectro das vasculites

Abstract

Lymphocytic Thrombophilic Arteritis is a vasculitis that affects the vessels of the dermis and hypodermis. It is rare and not well-documented, characterized by an indolent nature and restricted to the skin. Clinically, it is marked by macular hyperpigmentation with or without ulceration, predominantly on the lower limbs, and is mainly associated with racemosa livedo. Histologically, it presents with an inflammatory infiltrate predominantly composed of lymphocytes, with associated fibrinoid necrosis. For the preparation of this review, eight articles were selected from the PubMed database, from the first description as macular arteritis by Fein in 2003 up to 2020. The scarcity of publications on the subject can be explained by the fact that, for a long time, it was histologically recognized as a cutaneous polyarteritis nodosa in its subacute or reparative phase. However, the clinical and histological differences between the two conditions lead us to believe they are distinct entities and should be managed as such to avoid unnecessary treatments. Nevertheless, more robust, multicentric studies with longer follow-up periods are needed to better understand this condition, which is still difficult to recognize in clinical practice.