Cutaneous Manifestations of Antiphospholipid Antibody Syndrome

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Many different cutaneous lesions or cutaneous-systemic syndromes can be the presenting sign of antiphospholipid antibody syndrome (APS), or can develop during the course of disease. None of these conditions are specific for APS. Livedo reticularis or racemosa is commonly seen in APS, but it is one of the least specific findings. Other diseases are less commonly seen, in either their idiopathic or APS-associated form, but are more suggestive of APS. APS should be considered in patients who may appear to have idiopathic livedo reticularis with cerebrovascular accidents (Sneddon's syndrome), atrophie blanche, livedoid vasculitis, malignant atrophic papulosis, or anetoderma. Finally, retiform (branching, stellate) purpura or necrosis is perhaps the most characteristic cutaneous lesion of many different cutaneous microvascular occlusion syndromes, including APS.

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Livedo Reticularis and Racemosa

Livedo reticularis is the most frequently associated cutaneous manifestation in patients who have APS, observed as the presenting sign in up to 40% of patients and seen in up to 70% of patients who have systemic lupus erythematosus (SLE) and APS [1]. In patients who have SLE, the finding of moderate-to-extensive livedo reticularis probably warrants testing for antiphospholipid antibodies (aPLs). In a recent consensus statement on the updated classification criteria for APS, Miyakis and

Idiopathic Livedo Reticularis with Cerebrovascular Accidents (Sneddon's Syndrome)

In 1965, Sneddon reported the association of livedo reticularis or racemosa with cerebrovascular accidents [6]. Sneddon's syndrome is a rare, but potentially severe, condition typically affecting young or middle-aged women, with the first cerebrovascular accident typically occurring before 45 years of age. Livedo of either type, typically on the trunk and buttocks but also on the extremities and face, may precede the onset of stroke by years. Although the relationship between APS and Sneddon's

Atrophie Blanche and Livedoid Vasculopathy

Atrophie blanche is a term that has been used for several different conditions, leading to some confusion in the literature. Originally described as atrophie blanche en plaque by Milian in 1929, synonyms for this disease include livedo reticularis with summer ulcerations; segmental hyalinizing vasculitis; livedo vasculitis; livedoid vasculitis; hypersensitivity-type vasculitis; and painful purpuric ulcers with reticular pattern of the lower extremities (PURPLE) [9], [10], [11]. The disease is

Malignant Atrophic Papulosis (Degos' Disease)

Malignant atrophic papulosis, also known as Degos' disease, is a rare, vaso-occlusive disorder that predominately affects the skin initially, followed by the gastrointestinal tract and central nervous system. Affected men outnumber women three to one [15]. Visceral lesions may result in fatal bowel and cerebral infarctions. Some cases of only cutaneous involvement have been reported. Mucous membranes, particularly the conjunctiva, may be involved. Skin lesions begin as crops of 2- to 5-mm pale

Catastrophic Antiphospholipid Syndrome

The catastrophic APS, also known as CAPS, is a quickly progressing, often lethal manifestation first defined in 1992; in 2003, it also became known as Asherson's syndrome. Most often encountered in patients who have APS without lupus (49.9%), but also commonly in patients who have SLE and “lupus-like disease” (45%), this condition is characterized by rapid onset, resulting in multiple organ dysfunction; small-vessel occlusive disease with thrombotic microangiopathy; fulminant tissue necrosis,

Papular and Plaque Occlusive Lesions

Cutaneous necrosis is another common manifestation of APS and may be second only to livedo reticularis in frequency. In the Cervera and colleagues [22] series, necrotic skin ulcerations were observed in 5.5% of patients who had APS and were the presenting sign in close to 4%. Lesions in Fig. 3 are typical of this presentation. The most commonly involved sites are the upper and lower extremities and helices of ears, cheeks, trunk, and forehead [22]; lesions may be widespread. The onset is often

Anetoderma

Primary anetoderma is a rare skin disease of unknown cause characterized by loss of elastic fibers in the skin. Also known as macular atrophy, it was first described by Jadassohn in 1892. Lesions typically appear on the upper trunk and proximal extremities, presenting as multiple round, well-circumscribed, finely wrinkled patches or papules of slack skin that appear sunken, atrophied, or flaccid and demonstrate inward herniation or loss of substance on palpation (Fig. 4, Fig. 5).

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