Treatment of skin lesions related to pseudoxanthoma elasticum in plastic surgery

Pseudoxanthoma elasticum (PXE) is a hereditary disorder of connective tissue characterized by progressive calcification and fragmentation of elastic fibers, which primarily affects the skin, retinal and arterial walls. Skin damage takes the form of yellow papules that can merge to create a cutaneous fold. This is accompanied by an excess of skin on the different sides of the neck and in the largest folds. These changes to the skin have a significant aesthetic, functional and psychological impact, especially among women.

We evaluated the treatment options in all patients with PXE of our University-Hospital. This group contains people who have been hospitalized for the assessment of their disease and applicants for surgical correction. The goal of the surgery was not the total removal of the lesions but instead a decrease in their size and a tightening of the skin.

In total, 250 patients were seen between 2007 and 2022. Surgical treatment was advised for 29 women and 1 man. The main interventions were based on standard techniques such as cervico-facial facelifts, brachioplasties and cruroplasties. The results obtained during postoperative follow-up consultations were rated satisfactory to very good, both aesthetically and functionally. There were no postoperative complications recorded: neither bruising nor scarring issues. Patients with PXE heal as normal.

Surgical treatment for excess skin observed during PXE is poorly described. Yet, these excesses can be removed when they become troublesome by making use of and adapting the traditional methods of plastic surgery for tightening of the skin.