Immunologic abnormalities associated with primary anetoderma

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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“…This association was previously reported by Hodak et al [15]. However, our patient later developed SLE.…”

Section: Discussionsupporting

confidence: 90%

“…Hodak et al [15] used the activated partial thromboplastin time and VDRL test and found that one of their 6 patients with PA was LA positive.…”

Section: Discussionmentioning

confidence: 99%

Antiphospholipid Antibodies and Anetoderma: Are They Associated?

Stephansson

Niemi

1995

Dermatology

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“…18 Anetoderma has also been reported in association with alopecia areata, vitiligo, and hypothyroidism, conditions seen with increased incidence in DS. 19 …”

Section: Anetodermamentioning

confidence: 99%

Dermatological Manifestations of Down's Syndrome

Barankin

Guenther

2001

Journal of Cutaneous Medicine and Surgery: Incorporating Medica

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Background: Down's syndrome (DS) is an extensively researched congenital condition characterized by mental retardation and distinct physical features. The cutaneous manifestations of DS are numerous, yet they seldom receive appropriate attention.Objective: To review the dermatological conditions associated with DS.Methods: A review of the medical literature.Results: DS is associated with an increased incidence of numerous dermatological conditions, some of which may be related to an immunological deficiency.Conclusion: Along with the importance of understanding the physical and psychosocial aspects of DS, an appreciation of the dermatological manifestations of this condition is integral to comprehensive medical care.

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“…In addition, anetoderma is frequently associated with many autoimmune disorders (e.g. lupus erythematosus, Addison’s disease, hypothyroidism, vitiligo, Graves-Basedow disease, autoimmune hemolysis, alopecia areata [3, 6]) and with different autoantibodies, of which the most common are antinuclear antibodies [6]. Our patient also had two other autoantibodies: anti-PCNA and antiphospholipid (lupus anticoagulant and anticardiolipin antibodies).…”

Section: Discussionmentioning

confidence: 81%

“…The most frequent findings are linear or granular deposits of immunoglobulins and/or complement along the dermoepidermal junction of involved skin, just as we observed in our patient. Sometimes immunoreactants can also be found in blood vessel walls and papillary dermis [6, 7]. In addition, anetoderma is frequently associated with many autoimmune disorders (e.g.…”

Section: Discussionmentioning

confidence: 99%

Anetoderma in a Systemic Lupus erythematosus Patient with Anti-PCNA and Antiphospholipid Antibodies

et al. 2001

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Anetoderma is a rare elastolytic disorder included within the group of cutaneous atrophies. Its pathogenesis is not yet clearly established, but immunological mechanisms could play an important role in dermal elastolysis. It has been associated with different autoantibodies and autoimmune disorders. We present a case of anetoderma in a systemic lupus erythematosus patient with anti-proliferating-cell-nuclear-antigen and antiphospholipid antibodies, highlighting the peculiarities of such an association.

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Immunologic abnormalities associated with primary anetoderma

Cited by 24 publications

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Antiphospholipid Antibodies and Anetoderma: Are They Associated?

Antiphospholipid Antibodies and Anetoderma: Are They Associated?

Dermatological Manifestations of Down's Syndrome

Anetoderma in a Systemic Lupus erythematosus Patient with Anti-PCNA and Antiphospholipid Antibodies

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