A Peripheral Vascular Syndrome Overlapping with Systemic Lupus Erythematosus

Johansson, Eija; Niemi, Kirsti‐Maria; Mustakallio, K. K.

doi:10.1159/000250976

Cited by 54 publications

(7 citation statements)

References 8 publications

(9 reference statements)

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“…Peripheral vascular syndrome Johansson et al (1977) described five patients with circulating anticoagulants who demonstrated a vascular syndrome consisting of recurrent deep venous thrombosis and necrotizing purpura with painful superficial ulcers around the ankles. Skin biopsies revealed a massive proliferation of haemorrhagic dermal capillaries without any significant inflammatory reaction.…”

Section: Livedomentioning

confidence: 99%

Anticardiolipin antibodies—clinical associations

Asherson

Harris

1986

Postgraduate Medical Journal

134

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Section: Livedomentioning

confidence: 99%

Anticardiolipin antibodies—clinical associations

Asherson

Harris

1986

Postgraduate Medical Journal

134

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“…However, no thrombotic pathology is found in livedos and certain legs ulcerations [I. [9][10][11][12][13][14][15]. The only superficial extended cutaneous necrosis associated with LA previously reported in the literature differed from our observations in that it was localized on the face, other lupus associated anomalies were lacking, it was sensitive to corticotherapy at a dose of 40 mg/day and fibrin monomers were present indicating disseminated intravascular coagulation 116],…”

Section: Commentsmentioning

confidence: 64%

Cutaneous Necrosis Associated with the Lupus Anticoagulant

et al. 1989

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Extensive cutaneous necrosis of the limbs with sudden onset was observed in 3 women suffering from benign systemic lupus erythematosus. All 3 had a circulating anticoagulant and a positive venereal diseases reference laboratory test without anticardiolipin antibodies.They were successfully treated with pulse methylprednisolone therapy and plasmapheresis. The necrosis-lupus anticoagulant relationship is discussed due to the absence of another possible etiology for cutaneous necrosis and the latter’s histological aspect, i.e. thromboses of the dermal and hypodermal vessels without vasculitis. This cutaneous necrosis resembles that observed in congenital protein C deficiencies and favors the hypothesis of interaction between the lupus anticoagulant and the protein C-S complex.

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“…One of the patients fulfilled the criteria of PAPS. This young man (case 2) with livedoid vasculitis type leg ulcers was included in our previous report then named as 'peripheral vascular syndrome' [7]. The other 2 patients with APS had SLE; in 1 of them diag nosed before the onset of anetoderma.…”

Section: Discussionmentioning

confidence: 94%

Antiphospholipid Antibodies and Anetoderma: Are They Associated?

Stephansson

Niemi

1995

Dermatology

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Background: Macular atrophy or anetoderma is a rare skin disease of unknown pathogenesis, characterised by wrinkled or flaccid skin. Objective: The finding of anetoderma in 5 patients followed up because of false-positive seroreactions of syphilis prompted us to study the occurrence of antiphospholipid (aPL) antibodies in anetoderma. Methods: 14 unselected patients with primary anetoderma (PA) were collected from hospital records and clinical, immunological and histological findings were compared in the two patient groups. Results: Of the 5 patients, 3 fulfilled the criteria for antiphospholipid syndrome. In two cases, it was secondary to systemic lupus erythematosus (SLE). Of the 14 PA patients 1 had aPL antibodies and 4 had borrelia antibodies. Two patients had thyroid antibodies; 1 of them developed SLE. In several biopsy specimens, microthromboses were seen in both patient groups. Conclusion: On the basis of this study and our previous findings, it seems that anetoderma is more often associated with aPL-positive SLE or lupus-like disease than with aPL-negative disease. Immunological mechanisms play an important role in both primary and secondary anetoderma. The meaning of false-positive serological tests for syphilis or borrelia and aPL antibodies is not clear, but they may be reacting to some still unidentified antigen. Probably, various systemic as well as local inflammatory and non-inflammatory processes, e.g. microthromboses, can trigger anetoderma via still unknown pathomechanisms.

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A Peripheral Vascular Syndrome Overlapping with Systemic Lupus Erythematosus

Cited by 54 publications

References 8 publications

Anticardiolipin antibodies—clinical associations

Anticardiolipin antibodies—clinical associations

Cutaneous Necrosis Associated with the Lupus Anticoagulant

Antiphospholipid Antibodies and Anetoderma: Are They Associated?

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