Atrophie Blanche

Etiology

There are many theories regarding the cause of livedoid vasculopathy, but the exact underlying mechanism is poorly understood.[4] It is believed to involve occlusion of the blood vessels in the superficial dermis with subsequent skin ulceration.

Epidemiology

Atrophie blanche is an uncommon disease entity affecting 1% to 5% of the population and occurring primarily in middle-aged female adults with an average age of occurrence of 45-years-old. However, there are rare case reports of children with the disease.[5][6][7]

Pathophysiology

The pathophysiology of atrophie blanche can vary depending on the underlying etiology. It is often idiopathic but may be associated with several other disease processes, including autoimmune conditions and hypercoagulable disorders. The pathogenesis typically involves disturbances in fibrinolysis and/or coagulation, leading to thrombo-occlusion of blood vessels in the superficial dermis.[8] Several prothrombotic factors may share a correlation with atrophie blanche, including aPL antibodies, abnormalities in protein S and C, hyperhomocysteinemia, sticky platelet syndrome, antithrombin III deficiency, and factor V Leiden mutation.

Histopathology

The histologic examination can vary depending on the age of the lesion. A characteristic histopathologic finding is mild perivascular lymphocytic inflammation, extravasated red blood cells, superficial dermal vessels with thrombi, and hyalinized vessel walls. Biopsy of an active ulcer will show infarction of the papillary dermis, whereas biopsy of a healed lesion will show an atrophic epidermis and dermal sclerosis. Immunofluorescence examination is non-specific in early lesions. In later lesions, immunoglobulins, routinely IgM, and C3 are deposited within the vessel walls.[9]

History and Physical

Physical exam findings depend on the disease stage. The patient often initially complains of bilateral, painful, purpuric lesions that progress to punched-out ulcers, with or without surrounding livedo reticularis. These lesions will heal with porcelain-white, stellate scars, with or without surrounding telangiectasias. History typically reveals that the ulceration is recurrent and is often exacerbated in the summer and during pregnancy.[9]

It is essential to rule out other possible causes, so history should focus on eliminating underlying diseases such as peripheral arterial disease, venous stasis, and malignancy. A patient with atrophie blanche-like lesions will lack a history or physical exam finding of punched-out ulcers.[3]

Evaluation

Since the end manifestations of many diseases can produce atrophie blanche-like lesions, excluding these other causes is necessary to diagnosis true atrophie blanche, i.e., livedoid vasculopathy. Many studies will help a clinician to rule in or rule out other disease etiologies. The following is not an exhaustive list:

• Doppler studies and ankle-brachial index are aids to rule-out venous stasis and arterial occlusion[10]
• A skin biopsy to rule out cutaneous vasculitis
• Screening coagulation studies to evaluate for antiphospholipid antibody syndrome
• Lab work should include hemoglobin A1C
• Complete blood count with differential
• Basic metabolic panel
• Antinuclear antibody (ANA)
• Rheumatoid factor and C-reactive protein (CRP) to aid differentiation of diabetes mellitus, autoimmune syndromes, and malignancy

Treatment / Management

Treatment of atrophie blanche in the absence of underlying disease often starts with antiplatelet, anticoagulation, and fibrinolytic therapies, including aspirin, dipyridamole, pentoxifylline, and heparin. Sulfasalazine, danazol, or stanozolol can work as single agents or in addition to other treatments. Other options that have shown success include hyperbaric oxygen and PUVA (psoralen and ultraviolet A radiation). For patients with refractory disease, rivaroxaban, oral steroids, prostanoids, and IVIG (intravenous immunoglobulin) have been used with varying success. Patient education should include recommendations to stop smoking, to avoid irritants and poorly fitting shoes, as well as basic wound care of the lower extremities, i.e., leg elevation, compression therapy, and occlusive wound dressings.[6][11][12](B3)

Differential Diagnosis

Many other diseases may present with a similar end-stage as atrophie blanche. The causes of atrophie blanche-like lesions include cutaneous small-vessel vasculitis, hydroxyurea-related ulcers, sickle cell disease, and antiphospholipid antibody syndrome. These other disorders can be distinguished from atrophie blanche as the history will lack preceding punched-out ulceration.[13]

Prognosis

The prognosis of atrophie blanche varies but it tends to be a chronic, recurring disease.[4]

Complications

Infection, scarring, pain, ulceration, blood loss, stricture formation, and poor quality of life are all potential complications of atrophie blanche.