Introduction:

Necrosis of the extremities poses the problem of the underlying etiology which is not always easy. Indeed, they can be symptomatic of several inflammatory, thrombotic or embolic pathologies [1].

Here, we describe a case of an old woman who was diagnosed antiphospholipid syndrome with unusual manifestations.

Presentation of the case:

A 70-year-old woman was admitted to the Department of Cardiology of the University Hospital Center Mohammed VI of Oujda for management of the a myocardial infracts.

She had 3 pregnancies ended by a cesarean section and has a family history of diabetes and hypertension. She presented 4 years ago a deep vein thrombosis of the right leg and was treated with anticoagulation. She has also an uncontrolled arterial hypertension. Clinical examination showed cyanosis of the extremities with necrosis of the fingers and toes.

A coronarography was done and showed a bitroncular damage. Revascularization was realized. The biological assessment revealed anemia and thrombocytopenia. The detection of antiphospholipid antibodies were positive for anticardiolipin and anti-b2-GPI.

A skin biopsy was performed on the right index finger and revealed ischemic skin necrosis associated with capillary thrombi.

On the basis of medical history, clinical, histological and laboratory features diagnosis of antiphospholipid syndrome was made.

The management consisted of treating hypertension, introducing anticoagulation and correcting other cardiovascular risk factors. Unfortunately, the patient was dead.

Figure 1: Clinical photographs of the dorsal (A) and plantar (B) faces of the hands demonstrating a necrosis of the fingers

Figure 2: Clinical photographs of the dorsal (A) and plantar (B) aspects of the feet showinng the location and extent of the necrosis

Discussion:

The necrosis of extremities can affect people of different ages and the etiologies responsible for it are very varied. Etiological investigation is difficult but should not delay management because the functional prognosis is frequently involved [1].

The most common etiologies are Berger’s disease, systemic scleroderma, atherosclerosis, primary anti-phospholipid syndrome, systemic vasculitis, essential thrombocythemia, paraneoplastic cryoglobulinemia of malignant mesothelioma and essential acrocyanosis.

Antiphospholipid syndrome (APS) is an autoimmune disease characterized by the occurrence of venous and/or arterial thrombosis, and the detection of circulating antiphospholipid antibodies [2]. Single or multiple thrombi in veins, arteries and the microvasculature may give rise a wide range of clinical pictures. While deep vein thrombosis, particularly of the lower limbs, is the most frequently reported clinical manifestation (39%), thrombocytopenia (30%), livedo reticularis (24%), stroke (20%), pulmonary embolism (14%), heart valve lesions (10%), epilepsy (7%), myocardial infarction (6%), leg ulcers (5%) and amaurosisfugax (5%) may also occur and they are classified as “non-criteria features of APS” [2].

The average age of primary APS patients has been reported to be about 35–40 years and the disease is more common in women than in men. [2].

The cutaneous manifestations of APS are livedo, skin ulcerations, digital gangrene and extensive superficial cutaneous necrosis.

Treatment of patients with APS and skin lesions depends on the type of skin manifestations and other manifestations of the SAPL. It is curative and preventive.

The indication of an effective anticoagulation by heparin therapy is unavoidable in the presence of extensive necrosis and/or digital gangrene. If it is not enough to stop the thrombotic process, other therapeutic alternatives can be proposed such as plasma exchanges, corticosteroids, gamma globulins, cyclophosphamides or rituximab [4].

In APS diagnostic criteria, skin lesions are the only clinical component. Cutaneous thrombosis is easily detectable on biopsies from pseudo-vasculitis lesions and extensive necrosis as is the case in our patient, more difficult to detect on biopsies from ulcerated lesions and exceptionally in those taken from the mesh or between the meshes of the livedo which is the most frequent cutaneous manifestation [4].

Conclusion:

Dermatological lesions of antiphospholipid syndrome are frequent and sometimes inaugural. Their clinical expression are diverse, ranging from very discrete manifestations, often unnoticed and undiagnosed, to severe, potentially life-threatening injuries such as extensive skin necrosis or distal gangrene.