Skin ulcerations

Skin ulcerations have been reported since 1963 (Bowie et al., 1963) in association with lupus

Lupus And Skin Lesions

Figure 2. Typical pigmented and atrophic scars of livedoid vasculitis-like ulcers with purpuric necrotic lesions in primary APS.

anticoagulant (LAC). A strong association between leg ulcers and aCL has been reported in SLE, the prevalence of aCL reaching 87% in such patients (Alarcon-Segovia et al., 1989). They were observed in 5.5% of the 1000 European patients with APS, as a presenting manifestation in 3.9% (Cervera et al., 2002). In our series of 100 patients with primary APS and 100 patients of SLE-related APS, ulcers were detected in 8% of cases; they were the presenting feature of APS in 4% of cases without difference of prevalence in both groups (Frances et al., 2005). However, they were not reported in some series (Vianna et al., 1994). In a series of 115 consecutive dermatology patients hospitalized for leg ulcers, aCL could be detected in 43% of cases (Marechal et al., 2000).

Different types of skin ulcerations may be encountered.

Post-phlebitic ulcers were observed in patients with recurrent phlebitis of the leg after many years of follow-up in 4.5% of 200 APS patients (Frances et al., 2005). Usually, a post-phlebitic syndrome was also present characterized by edema and cutaneous erythema of the lower limbs accompanied by inflammatory changes.

In contrast, ulcerations resulting from circumscribed skin necrosis are frequently a presenting feature of APS, occurring in 3.5% of cases (Frances et al., 2005). They consist of painful small (0.5-3 cm in diameter) ulcers with oval, star-like, or irregular borders surrounded by a purplish-brown halo and recurrent purpura (Fig. 2). They develop around the

Figure 2. Typical pigmented and atrophic scars of livedoid vasculitis-like ulcers with purpuric necrotic lesions in primary APS.

ankles, on the feet and in some cases on the calves. They are often preceded by necrotizing purpura and associated with LR of the legs. After healing, the ulcers leave white atrophic scars with a dark pig-mented halo. In a series of 21 consecutive patients with ''atrophie blanche"-like ulcers or livedoid vasculitis, aPL antibodies were detected in four patients (19%) of whom one had SLE-related APS and three primary APS (Tran et al., 2001). Other prothrom-botic factors may also be present (Combemale et al., 2002).

About 10 cases of large ulcers resembling pyoder-ma gangrenosum have been reported in the literature in association with primary or SLE-related APS (Schmid et al., 1998; Chacek et al., 1998).

Contrary to pyoderma gangrenosum, these ulcers did not present undermined borders and were only found on the legs. We have not encountered these types of ulcerations.

3.3. Digital gangrene

Digital gangrene (Fig. 3) was present in 3.3%-7.5% of APS cases and the presenting feature in 2.5% of one series (Cervera et al., 2002; Frances et al.,

Gangrene Finger Neonate
Figure 3. Digital gangrene.

2005). Gangrene may be preceded by distal erythema (Asherson et al., 1986), cyanotic macules (Grob et al., 1989) or pseudo-cellulitis (Jindal et al., 1983). Associated vascular risk factors such as smoking, oral contraceptives, or hypertension were present in some cases (Asherson et al., 1989; Jindal et al., 1983). Angiography visualized occlusion, or sometimes stenosis of large or medium-sized vessels.

3.4. Multiple subungual splinter hemorrhages

Subungual splinter hemorrhages appear as tiny linear longitudinally oriented, reddish-brown to black, distal subungual lesions that fail to blanch under pressure. They may result from nail dystrophy. When the nail shape is normal, subungual splinter hemorrhages were initially recognized as an important sign of subacute bacterial endocarditis. In fact they can be observed in various other conditions, such as Trichinella spiralis infection or hyper-eosinophilic syndrome, and even in healthy people. When multiple hemorrhages are seen on different fingers, the existence of an underlying disease is probable (Fig. 4). In 1966, they were reported in SLE prior to research on the antiphospholipid antibodies (Fraga and Mintz, 1966). Multiple splinter hemorrhages may be observed in APS regardless of the presence of SLE (Asherson, 1990; Ames

Hemorrhage Nail Beds
Figure 4. Multiple subungual splinter hemorrhages concomitant to pulmonary embolism in APS.

et al., 1992; Frances et al., 1994; Mujic et al., 1995). Within the APS, their sudden onset on multiple fingers is frequently concomitant to other worrying thrombotic events, therefore leading to a probable underestimation, as illustrated by the 0.7% prevalence observed in 1000 APS patients (Cervera et al., 2002). A prevalence of 4-5% is likely as in other series (Mujic et al., 1995; Frances et al., 2005). Although their pathogenesis is not fully elucidated due to the rarity of histological examinations performed on this peculiar area, a thrombotic or an embolic process is possible.

3.5. Superficial venous thrombosis

Superficial venous thromboses were present in 11.7% of the 1000 European patients with APS (Cervera et al., 2002), and in 5% of another series of 200 APS patients (Frances et al., 2005). They have been included in the classification criteria for definite APS (Table 2) (Wilson et al., 2001). The diagnosis of superficial thrombophlebitis usually is clinically evident, but may sometimes require echo-Doppler examination or skin biopsy. In APS, they are mostly localized on the limbs. It should be kept in mind that repeated episodes of superficial thrombophlebitis, mainly affecting the trunk, may also reveal an occult cancer, at times accompanied by aPL production (Bessis et al., 1995).

3.6. Thrombocytopenic purpura

Purpura due to a platelet deficiency usually occurs with a count below 20 x 109/l. Bleeding occurs into the skin with crops of petechiae localized on the trunk or on the limbs. Mucosa bleeding is frequently associated. It is a rare manifestation of APS, not reported in many series (Cervera et al., 2002; Vianna et al., 1994), observed in 3.5% of cases in our experience (Frances et al., 2005). It may be the presenting feature of APS (1.5%).

3.7. Pseudovasculitis manifestations

These pseudovasculitic lesions may mimic cutaneous vasculitis and may be misdiagnosed if skin biopsies are not performed, especially in patients with SLE. They are the presenting manifestations in about 3% of cases and present during the course of the disease in 3-4% (Cervera et al., 2002; Frances et al., 2005). Different skin lesions have been reported i.e. purpura (Fig. 5a), small ery-thematous or cyanotic lesions on hands and feet, papules or nodules (Fig. 5b) of the limbs, ears, neck or thighs (Farrant et al., 1989; Grob et al., 1991; Asherson et al., 1992; Renfro et al., 1992; Ishikawa et al., 1999; Frances et al., 2005). The purpura is frequently necrotic and it may precede the livedoid vasculitis-like ulcers by several years. (Grob and Bonerandi, 1989; Frances et al., 2005).

Lesions similar to those of malignant atrophic papulosis may also be included in pseudovasculitis manifestations. They consist of multiple pink, gray, or yellow papules that become umbilicated and develop a porcelain-white central scar with a telangiectatic border (Fig. 5c). They have been reported in SLE-related APS and primary APS (Dublin and Stawiski, 1974; Black and Hudson, 1976; Doutre et al., 1987; Burton, 1988). Exceptionally, aPL were detected in patients with malignant atrophic papulosis (Farrell et al., 1988;

Table 2

Preliminary criteria for the classification of antiphospholipid syndrome according to Wilson et al. 1998

Clinical criteria Vascular thrombosis

One or more clinical episodes of arterial, venous, or small vessel thrombosis, in any tissue or organ. Thrombosis must be confirmed by imaging or Doppler studies or histopathology, with the exception of superficial venous thrombosis. For histopathologic confirmation, thrombosis should be present without significant evidence of inflammation in the vessel wall.

Pregnancy morbidity

One or more unexplained deaths of a morphologically normal fetus at or beyond the 10th week of gestation, with normal fetal morphology documented by ultrasound or by direct examination of the fetus, or

One or more premature births of a morphologically normal neonate at or before the 34th week of gestation because of severe pre-eclampsia or eclampsia, or severe placental insufficiency, or

Three more unexplained consecutive spontaneous abortions before the 10th week of gestation with maternal anatomic, or hormonal abnormalities and paternal and maternal chromosomal causes excluded.

Laboratory criteria

Anticardiolipin antibody of IgG and/or IgM isotype in blood, present in medium or high titre, on two or more occasions, at least 6 weeks apart, measured by a standard enzyme linked immunosorbent assay for 132-glycoprotein 1-dependent anticardiolipin antibodies.

Lupus anticoagulant present in plasma on two or more occasions at least 6 weeks apart, detected according to the guide lines of the International Society on Thrombosis and Hemostasis.

Definite APS is considered to be present if at least one of the clinical and one of the laboratory criteria are met.

Aps Manifestation

Assier et al., 1995). In our mind, APS and malignant atrophic papulosis should be regarded as distinct conditions (Assier et al., 1995).

3.8. Extensive cutaneous necrosis

Clinical features of widespread superficial cutaneous necrosis within APS (Fig. 6) are similar to those observed in other thrombophilic states such as protein C or protein S deficiencies, monoclonal cryoglobulinemia or cryofibrinogenemia. In some cases, these biological abnormalities, present in association with aPL, may contribute to the throm-botic process (Moreb and Kitchens, 1989; Dessein et al., 1989). APS may be primary or associated with other disorders such as SLE, rheumatoid arthritis, mycosis fungoides, or human immunodeficiency virus infection (Dodd et al., 1985; Frances et al., 1989; O' Neill et al., 1990; Creamer et al., 2000b). Widespread cutaneous necrosis occurs in about 2% of APS patients (Cervera et al., 2002; Frances et al., 2005). The onset is often acute with extensive painful purpura followed by a black necrotic plaque with an active purpuric border and bullous lesions. The necrosis is usually localized on the limbs, head (cheeks, nose, and ears), or buttocks.

3.9. Primary anetoderma

Anetoderma is a rare disorder characterized clinically by a circumscribed area of slack skin with macular depressions or outpouchings of skin. Skin lesions are numerous (>10), localized on the half upper part of chest and arms (Fig. 7). Histologically there is a loss of dermal elastic tissue (Disdier et al., 1994). Anetoderma may be primary or secondary to various dermatoses. When primary, it is frequently observed in patients with autoimmune diseases and especially related to antiphospholipid antibodies (Frances and Piette, 1997; Romani et al., 2000). A review of literature disclosed more than 20 such patients with aPL (Romani et al., 2000). Among nine patients with primary anetoderma referred to

Necrotic Skin Conditions
Figure 6. Widespread superficial skin necrosis in SLE-related APS.

our Connective Tissue Disorders Clinic, aPL was detected in eight, of whom four had APS (Sparsa et al., 2003).

3.10. Melanoderma

Adrenal failure secondary to hemorrhagic infarction of the adrenal glands is usually a critical manifestation of APS. However, in about 10% of patients, it may manifest itself as melanoderma of recent onset (Asherson and Hughes, 1989). This rare manifestation was not noticed in the large series of the literature.

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