Increased Fibroblast Elastase Activity in Acquired Cutis laxa

Abstract

Background: Acquired cutis laxa is a rare disease characterized by sagging skin, premature wrinkling and reduced skin elasticity. Observation: We report a 21-year-old woman, who presented with acquired cutis laxa on the face and the ear lobes. Urticarial papules had preceded for 6 years. There was no systemic involvement. Skin specimens were obtained from lax skin and urticarial papules, and from healthy controls. Histology showed only few perivascular lymphocytes in lax ear skin and a dense inflammatory infiltrate in urticarial skin. In both biopsies elastic fibres were decreased as demonstrated by computerized morphometric analyses. Elastase activities of fibroblasts in culture were evaluated. There was a 2- to 3-fold increase in elastase activity in urticarial skin fibroblasts, contrasting with a normal elastase activity in lax ear skin. Conclusion: Our findings suggest that the inflammatory cells could play a significant role in the destruction of elastic fibres.

Case of acquired cutis laxa with preceding urticarial eruption treated by diphenyl sulfone

“…We suggest that abnormality of elastic fibers may be associated with infiltration of neutrophils. Bouloc et al 4. reported 2–3‐times more fibroblast elastase activity in an urticarial eruption in culture, than in the control and lax skin.…”

“…Thus, acquired CL might have been triggered by urticarial eruption. In three cases,2, 3, 4 a biopsy was performed on an urticarial lesion, and revealed the presence of neutrophils, while the elastic fibers were thin and fragmented. In our case, neutrophils adhered to elastic fibers.…”

Acquired disorders of elastic tissue: Part II. decreased elastic tissue

“…Acquired cutis laxa may present without evidence of an antecedent dermatosis but has been reported in association with urticaria, eczema, and erythema multiforme. 56,58,59,68,69 Lesions are commonly described as pendulous or redundant coarsely wrinkled areas of skin (Fig 3, A and B). The onset of disease may be insidious, developing over the course of several years.…”

Generalized Cutis Laxa Associated with Heavy Chain Deposition Disease

“…3,4,6,7,9,14,23,28,29 The preelastic fibers, oxytalan and elaunin, are undetectable, and the microfibrillar component of elastic fibers is reduced. 30,31 Electron microscopy confirms the degenerative changes in the elastin fibers. 18,28,32 Findings in other affected organ systems demonstrate a similar loss of elastic tissue.…”

“…1,7 In cases preceded by inflammatory skin lesions, the inflammatory cells may mediate an autoimmune attack on the elastin fibers. 7,30 In cases associated with multiple myeloma, cutis laxa may represent an immune-mediated complication, with immunoglobulins being deposited on elastic fibers and inciting an inflammatory response. 14,16 Deposition of immunoglobulin on elastic fibers may result in complement fixation or binding to macrophages or neutrophils, resulting in elastolysis.…”