Lymphocytic thrombophilic arteritis

What is it lymphocytic thrombophilic arteritis?

Lymphocytic thrombophilic arteritis is characterized by primary lymphocytic vasculitis. It comes with livedo racemosa or macular hyperpigmentation. It has different histological findings affecting small to medium arteries at depth dermis and superficial subcutis [1].

Also known as macular lymphocytic arteritis.

Who gets lymphocytic thrombophilic arteritis?

Lymphocytic thrombophilic arteritis tends to affect young patients, with a mean age of 39 years. [two].

What causes lymphocytic thrombophilic arteritis?

The exact cause of lymphocytic thrombophilic arteritis is unknown. It has been proposed that the underlying lymphocytic endovasculitis drives a located thrombophilia, represented by intraluminal fibrin ring seen in histology, without any evidence of destruction of the vessel wall [1].

• Some cases have been associated with autoimmune antibodies or heterozygosity for prothrombotic mutations, suggesting that autoimmune and thrombophilic factors may contribute.
• Antiphospholipid antibodies have been detected in some cases, but low titers and lack of systemic characteristics argue against antiphospholipids syndrome contributing significantly to their Pathogenesis [1,3].
• Low to moderate levels of antinuclear antibodies (ANA) have been reported, but there was no evidence of systemic disease. connective tissue disease in these cases [1,4].

What are the clinical features of lymphocytic thrombophilic arteritis?

The clinical features of lymphocytic thrombophilic arteritis include:

• Persistent livedo racemosa or macular hyperpigmentation that generally affects the lower extremities; the upper extremities may be less affected.
• Palpable subcutaneous indurations
• Some reports of ulceration [5,6]
• Lack of itching or pain
• Absence of purplescars and atrophie blanche.

How is lymphocytic thrombophilic arteritis diagnosed?

Diagnosis of lymphocytic thrombophilic arteritis is based on a combination of typical clinical features of persistence. asymptomatic livedo racemosa or macular hyperpigmentation supported by different histological findings.

• A dense inflammatory infiltrate It is found in the wall of the muscular vessel, affecting the small and medium arteries of the deep dermis or superficial subcutis.
• The inflammatory infiltrate consists predominantly of mononuclear cells, mainly lymphocytes and something histiocytes.
• Neutrophils and eosinophils they are scarce or absent.
• A concentric hyalinized The fibrin ring involves the entire periphery of the lumen of the affected vessels.

Signs or symptoms of systemic vasculitis are also absent (see Cutaneous vasculitis) [2].

Which is the differential diagnosis for lymphocytic thrombophilic arteritis?

Lymphocytic thrombophilic arteritis can be confused with several others vascular occlusive terms

Cutaneous polyarteritis nodosa

• Unlike cutaneous polyarteritis nodosa, painful purpura is absent and ulceration is rare in lymphocytic thrombophilic arteritis. [one].
• The livedo pattern in cutaneous polyarteritis nodosa is more localized with a stellar burst pattern [7].
• Early stage cutaneous polyarteritis nodosa sample neutrophilic infiltration involving the muscular layer of medium and small arteries, with a greater participation of mononuclear cells in the later stages [8].

Livedoid vasculopathy

• Although livedo racemosa may be present, livedoid vasculopathy typically presents in the early stages with episodic purpura and painful ulceration involving the lower extremities, followed by white atrophy.
• There is luminal fibrin statement in livedoid vascular disease, but the inflammatory infiltrate is usually scarce [1,9].

Antiphospholipid syndrome

• Livedo racemosa may also be present in antiphospholipid syndrome.

• There is thrombosis without significant inflammation on the wall of the glass
• Antiphospholipid syndrome is characterized by recurrent thrombosis, miscarriages and thrombocytopenia [10].

Sneddon syndrome

• The pattern of livedo racemosa in Sneddon syndrome is broader compared to lymphocytic thrombophilic arteritis [3].
• The livedo is more proximal in Sneddon syndrome, which involves the trunk and the back; the extremities are rarely involved.
• Diagnosis of Sneddon syndrome requires cerebrovascular intervention [11].

What is the treatment for lymphocytic thrombophilic arteritis?

Lymphocytic thrombophilic arteritis tends to show a indolent course with persistent asymptomatic livedo racemosa or macular hyperpigmentation [1]. Since most injuries are asymptomatic, treatment is not always required [2].

Treatment options that have shown a beneficial response include:

• Aspirin
• Pentoxifylline

• Dapsone

Oral and current Steroids have not been shown to be useful [2].

Given the rarity of lymphocytic thrombophilic arteritis and inconsistent responses to treatment, there is no current consensus on treatment recommendations.

What is the result of lymphocytic thrombophilic arteritis?

Lymphocytic thrombophilic arteritis tends to follow a chronic indolent course without progression to systemic vasculitis [1,2]. Continuous monitoring is recommended. [one]. Long-term forecast not yet clear.