Mid-dermal elastolysis: A female-centric disease; case report and updated review of the literature

Abstract

BackgroundMid-dermal elastolysis (MDE) is a rare, yet well-defined clinical and histopathologic entity manifested by fine wrinkling of the skin and mid-dermal loss of elastic fibers. This disease predominantly affects young to middle-aged Caucasian females and although it has no reported systemic features, it is psychologically bothersome and can be of great cosmetic concern.MethodsWe report a case of a healthy 45 year-old female with widespread mid-dermal elastolysis. A literature search using the search terms “mid-dermal elastolysis,” “mid dermal elastolysis,” “middermal elastolysis,” and “elastophagocytosis” was conducted on Pubmed, using articles published from January 2008 until November 2014 to accompany Gambichler’s comprehensive 1977 to 2009 review of mid-dermal elastolysis. The references of relevant papers were reviewed and further cases included as appropriate.ResultsWe review the clinical features and histological, ultrastructural, and immunohistochemical findings of MDE, as well as differential diagnoses. There are 13 new publications of MDE since 2008. The novel findings since Gambichler’s review are discussed and pathomechanisms revisited. Interestingly, given the striking female predominance of MDE, there is no known hormonal role in its etiology.

Mycophenolate mofetil to treat mid‐dermal elastolysis

“…The exact pathogenesis of MDE is unknown, but reported triggers include pregnancy, oral contraceptive pills, ultraviolet exposure, and smoking . There have also been reported associations with Graves disease, Hashimoto's thyroiditis, systemic lupus erythematosus, and rheumatoid arthritis . There was no identifiable trigger or association in this case.…”

Cutaneous disorders characterized by elastolysis or loss of elastic tissue

“…Associations with various disorders have been described but a causal relationship is doubtful. While no familial clusters have been observed, there is a significantly higher incidence among – mostly middle‐aged – women . Clinically, MDE is characterized by well‐demarcated, variably‐sized areas of fine wrinkling, some of which show papular protrusions; the trunk and proximal extremities are primarily affected (Figure a, b).…”

“…Sun protection is recommended, although the pathogenetic role of UV radiation is not yet fully understood. An omega‐3 fatty acid, eicosapentaenoic acid, which is supposed to prevent MMP activation, has been shown to be somewhat beneficial .…”

Elastolysen und Hauterkrankungen mit Verlust der elastischen Fasern

“…Die Pathogenese ist nicht geklärt, ein autoimmunologischer Prozess wird diskutiert , eine Assoziation mit einer Reihe unterschiedlicher Erkrankungen ist beschrieben, ein kausaler Zusammenhang ist aber fraglich. Eine familiäre Häufung wird nicht beobachtet, wohl aber eine deutlich höhere Inzidenz bei Frauen in meist mittlerem Lebensalter. Klinisch zeigen sich mit Betonung des Rumpfes und der proximalen Extremitäten meist makulöse Areale unterschiedlicher Größe mit zum Teil leicht vorgewölbter, gering gefältelter Oberfläche (Abbildung a, b).…”

“…Bei letztlich nicht geklärter Rolle des UV‐Lichtes in der Pathogenese wird Lichtschutz empfohlen. Einen gewissen Effekt wird der Eicosapentaensäure als Vertreter der Omega‐3‐Fettsäuren zugeschrieben, welche eine Aktivierung der MMP verhindern soll .…”