Systemic lupus erythematosus-associated anetoderma and anti-phospholipid antibodies
Department of Dermatology, University Clinic of Navarra, Pamplona, Navarra, Spain. Clinical and Experimental Dermatology
(Impact Factor: 1.09).
02/2003; 28(1):39-42. DOI: 10.1046/j.1365-2230.2003.01139.x
Anetoderma is characterized by a loss of normal elastic tissue that presents clinically as localized areas of wrinkled or flaccid skin. We describe the case of a 30-year-old woman with systemic lupus erythematosus-associated anetoderma and positive anti-phospholipid antibodies. We discuss the possible role of these antibodies in the pathogenesis of anetoderma, and, when detected, the need to check for an associated anti-phospholipid syndrome in such patients.
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ABSTRACT: Anetoderma is a rare cutaneous disease characterized by a loss of normal elastic tissue that is presented clinically as localized areas of wrinkled or flaccid skin. This form may be associated with several immunological abnormalities, most notably lupus erythematosus and antiphospholipid antibodies with or without clinical manifestations of the antiphospholipid syndrome. A retrospective study was conducted with the aim of summarizing the clinical characteristics, course and laboratory findings in three women with anetoderma-associated lupus erythematosus panniculitis, an unusual variant of cutaneous lupus erythematosus. The 3 patients (of the 12 patients with lupus erythematosus panniculitis seen by us since 1990) were all at a young age at onset of panniculitis (median, 22 years). None of the patients developed severe systemic involvement up to 9 years (median, 5 years) from onset of the disease. The most noteworthy laboratory finding was the presence of antiphospholipid antibodies. Anetodermic lupus erythematosus panniculitis may be regarded as an uncommon variant of cutaneous lupus erythematosus mainly affecting young females and showing a favourable clinical course, although the patients should be followed and screened for the emergence of antiphospholipid syndrome. Antiphospholipid antibodies could play a role in the elastolytic process, leading to anetoderma.
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ABSTRACT: Elastic fibers in the extracellular matrix are integral components of dermal connective tissue. The resilience and elasticity required for normal structure and function of the skin are attributable to the network of elastic tissue. Advances in our understanding of elastic tissue physiology provide a foundation for studying the pathogenesis of elastic tissue disorders. Many acquired disorders are nevertheless poorly understood owing to the paucity of reported cases. Several acquired disorders in which loss of dermal elastic tissue produces prominent clinical and histopathologic features have recently been described, including middermal elastolysis, papular elastorrhexis, and pseudoxanthoma-like papillary dermal elastolysis, which must be differentiated from more well-known disorders such as anetoderma, acquired cutis laxa, and acrokeratoelastoidosis. Learning objective At the conclusion of this learning activity, participants should have an understanding of the similarities and differences between acquired disorders of elastic tissue that are characterized by a loss of elastic tissue.
Available from: Jesús Tercedor Sánchez
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