Atrophie Blanche - Star-Like Scars

Introduction

Atrophie blanche is an uncommon disease that affects one to five percent of the population and occurs primarily in female adults with an average age of 45 years old. Atrophie blanche is a chronic condition that causes recurrent, painful, ulcers of the lower leg, dorsal foot, or ankle. It is seen as a result of healed ulcers. They are characterized by atrophic white, stellate, or star-like scars with peripheral telangiectasias (small, wide blood vessels on the skin).

Atrophie blanche, also called livedoid vasculopathy, is the primary, ulcerated lesion. A physical examination usually depends on the stage of the disease. However, the patients usually complain about painful, bilateral, purpuric lower leg lesions that cause punched-out ulcerations. Atrophie blanche occurs more commonly in females, but it also occurs in males, and certain cases in children have also been reported.

What Is the Cause of Atrophie Blanche?

There are many theories associated with the cause of atrophie blanche. However, the exact mechanism is not completely understood. It usually involves occlusion of the blood vessels in the dermis after skin ulceration. Various other diseases might be present with a similar end-stage as atrophie blanche.

The causes of atrophie blanche-like lesions include

• Cutaneous Small-Vessel Vasculitis - It is a disorder characterized by vasculitis or inflammation of blood vessels involving the small blood vessels in the skin.

• Antiphospholipid Antibody Syndrome - It is a disorder where the immune system abnormally attacks the normal proteins in the blood.

The other disorders can be differentiated from atrophie blanche as the history will lack preceding punched-out ulceration.

What Is the Pathophysiology of Atrophie Blanche?

The pathogenesis usually involves disturbances in fibrinolysis (a process that prevents the naturally occurring blood clots from growing and causing further problems) and coagulation that causes blockage of blood vessels in the superficial dermis.

The pathophysiology of atrophie blanche varies according to the etiology. It is usually idiopathic or might be associated with various other disease processes, like autoimmune conditions and hypercoagulable disorders.

The following prothrombotic factors might be associated with atrophie blanche:

• aPL antibodies.

• Abnormalities in proteins S and C.

• Hyperhomocysteinemia (a condition where the levels of homocysteine are greater than 15 micromol/liter blood).

• Sticky platelet syndrome (an autosomal dominant inherited thrombophilia).

• Antithrombin III deficiency (blood clotting disorder that leads to the formation of abnormal blood clots).

What Are the Clinical Features of Atrophie Blanche?

The clinical features usually depend on the stage of the disease. The patient often complains of painful, bilateral, purpuric lesions that progress to punched-out ulcers, with or without surrounding livedo reticularis (a skin condition that leads to the formation of a netlike pattern of reddish-blue skin discoloration where the legs are often affected and are associated with swollen blood vessels). The lesions usually heal with star-like scars, with or without surrounding telangiectasias. Clinical history indicates that the ulceration is recurrent and exacerbated during pregnancy and in summer. It is important to rule out other causes of the disease. Therefore, the presence of underlying diseases such as venous stasis, peripheral arterial disease, and malignancy must be eliminated. A patient with atrophie blanche-like lesions does not have a history or finding of punched-out ulcers.

How Is Atrophie Blanche Diagnosed?

For an accurate diagnosis, it is essential to exclude the diseases whose end manifestations can produce atrophie blanche-like lesions. Various studies can help a clinician rule in or rule out other disease etiologies. The following can help confirm the diagnosis:

• A skin biopsy can be performed to rule out cutaneous vasculitis.

• Doppler studies and ankle-brachial index help to rule out arterial occlusion and venous stasis.

• Screening coagulation studies help to evaluate antiphospholipid antibody syndrome.

The following laboratory tests can be performed to exclude diabetes mellitus, autoimmune syndromes, and malignancy:

• Hemoglobin A1C.

• Complete blood count.

• Basic metabolic panel (a test that gives essential information about the body's chemical balance and metabolism).

• Antinuclear antibody (ANA).

• C-reactive protein (CRP).

• Rheumatoid factor.

The diagnosis is usually complicated and needs proper research. Even after a complete diagnosis, the treatment is not the same for every patient. Not all patients are given the same treatment, and several approaches might be required to heal the ulcers. Patients must be provided with adequate expectations of the prognosis of the disease.

How Is Artophie Blanche Treated?

In the absence of underlying disease, the treatment of atrophie blanche is initiated with anticoagulation, antiplatelet, and fibrinolytic therapies, like Aspirin, Dipyridamole, Pentoxifylline, and Heparin. Other options that are successful include hyperbaric oxygen and PUVA (psoralen and ultraviolet A radiation). Certain medicines that can be used alone or in combination with other medicines include Sulfasalazine, Danazol, or Stanozolol.

For patients with refractory disease (a condition that does not respond to treatment), the following drugs are used

• Rivaroxaban.

• Oral steroids.

• Prostanoids.

• Intravenous immunoglobulin.

Patients are educated on basic wound care of the lower extremities, like compression therapy, leg elevation, and occlusive wound dressings. Patients must also be advised to stop smoking, and avoid wearing poorly fitting shoes.

Pain management is a problem, and a pharmacist and a pain specialist have to be involved. Opioid pain medications must be avoided to prevent tolerance and addiction. Since many patients develop anxiety and stress, mental health professionals might be consulted.

What Is the Prognosis of Atrophie Blanche?

The prognosis of atrophie blanche can vary in different patients. However, it is usually a chronic, recurring disease. Effective communication is key to a good patient prognosis. An interprofessional approach to atrophie blanche is essential. The condition is usually diagnosed by a healthcare professional. Proper nursing is required with history taking and preparing the patient for examination. Nurses can provide counseling to patients about pharmaceutical therapy.

What Are the Complications?

The complications include:

• Infection.

• Scarring.

• Pain.

• Ulceration.

• Blood loss.

• Stricture formation.

• Poor quality of life.

Conclusion

The cause of a lower extremity wound may be due to a myriad of diagnoses, including arterial or venous disease, infection, trauma, and diabetes mellitus, among other etiologies. Lower extremity ulcers can pose a diagnostic dilemma. The treatment usually requires various medical professionals, like nurses, clinicians, wound care, and family medicine. Some patients need long-term wound care with daily dressing changes and regular debridement.