A Brief Literature Update on Mid-dermal Elastolysis with an Emphasis on Pathogenetic and Therapeutic Aspects.

We provide a brief review on mid-dermal elastolysis (MDE) and summarize clinical data of 105 patients with MDE who were reported in the literature since the disease was first described in 1977. In doing so, emphasis is placed on pathomechanisms and therapeutic aspects. MDE is a rare, acquired skin disease histopathologically characterized by selective loss of elastic fibers in the mid-dermis. Lesions are commonly observed on the trunk and proximal extremities. These include well-circumscribed patches of fine wrinkles, perifollicular papular protrusions, and persistent reticular erythema. With respect to pathomechanisms, current data suggest that different cell types (e.g., macrophages, fibroblasts) might chronically overexpress matrix metalloproteinases resulting in an enhanced elastolytic activity. Together with decreased expression of the tissue inhibitors of metalloproteinases, this is thought to result in zonal degradation and loss of elastic tissue in the mid-dermis. However, the exact mechanisms leading to the enhanced elastolytic activity in MDE remain elusive. A multifactorial pathogenesis is likely, including genetic predisposition, chronic inflammation, and (auto)immune processes. Moreover, the capacity for elastic fiber renewal appears to be diminished in patients with MDE, limiting regenerative potential and informing possible treatment strategies, for example, by stimulating elastic fiber synthesis. Although the course of MDE is usually benign and asymptomatic, it can cause severe cosmetic problems. Hence, new therapeutic approaches that block increased elastolytic activity and enhance regeneration of elastic tissue observed in MDE patients are required.