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Author: Sarajane Ting, General Practitioner, Tasmania, Australia. DermNet NZ Editor in Chief: Adjunct A/Prof Amanda Oakley, Dermatologist, Hamilton, New Zealand. Copy edited by Gus Mitchell. February 2020.
Lymphocytic thrombophilic arteritis is characterised by a primary lymphocytic vasculitis. It presents with livedo racemosa or macular hyperpigmentation. It has distinct histological findings affecting small to medium-sized arteries in the deep dermis and superficial subcutis .
It is also known as macular lymphocytic arteritis.
Lymphocytic thrombophilic arteritis tends to affect young female patients, with a median age of 39 years .
The exact cause of lymphocytic thrombophilic arteritis is unknown. It has been proposed that the underlying lymphocytic endovasculitis drives a localised thrombophilia, represented by the intraluminal fibrin ring seen on histology, without any evidence of destruction of the vessel wall .
The clinical features of lymphocytic thrombophilic arteritis include:
The diagnosis of lymphocytic thrombophilic arteritis is based on a combination of typical clinical features of persistent asymptomatic livedo racemosa or macular hyperpigmentation supported by distinct histological findings.
Signs or symptoms of systemic vasculitis are also absent (see Cutaneous vasculitis) .
Lymphocytic thrombophilic arteritis can be confused with several other vascular occlusive conditions.
Lymphocytic thrombophilic arteritis tends to show an indolent course with persistent asymptomatic livedo racemosa or macular hyperpigmentation . As most lesions are asymptomatic, treatment is not always required .
Treatment options that have shown a beneficial response include:
Given the rarity of lymphocytic thrombophilic arteritis and inconsistent treatment responses, there is no current consensus on treatment recommendations.
Lymphocytic thrombophilic arteritis tends to follow a chronic indolent course with no progression to systemic vasculitis [1,2]. Ongoing follow-up is advisable . The long-term prognosis remains unclear.
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