Cutis laxa acquisita – novel insights into impaired elastic fiber regeneration

Abstract

Cutis laxa (CL) is a rare disorder affecting elastic fiber formation¹. The disease is disfiguring and involvement of big vessels in lung and heart often leads to severe complications. CL occurs as an inherited and acquired form, cutis laxa acquisita (CLA). While in the inherited form most mutations have been identified in genes for elastic fiber components like elastin ², fibulin-4 ³, fibulin-5 ⁴, or LTBP-4 ⁵ the pathogenesis of CLA remains poorly understood. Clinically, there often is an association between CLA and paraproteinemia ¹. Deposition of immunoglobulins and complement has been proposed to result in an immune-mediated destruction of elastic fibers ⁶.

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