ABSTRACT
BACKGROUND: 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. PATIENTS AND METHODS: 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. RESULTS: 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. CONCLUSION: 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.
ABSTRACT
BACKGROUND: The Face is the most frequent localization for cutaneous carcinoma. The nose accounts for about 30% of these tumors. Nose tissue loss repair has to pursue 3 types of objectives: carcinologic, aesthetic and functional. The aim of this article is to identify a decision tree to guide the choice of surgical reconstruction technique based on localization and size of the defect. PATIENTS AND METHODS: We performed a retrospective analysis in Angers' CHU from 2013 to 2019 including 229 patients referred for cutaneous tumors excision in need of reconstruction. We analyzed the type of reconstruction, size of the tissue loss and localization of the defect in terms of nose aesthetic subunits. RESULTS: Among the 229 patients included, the most frequent localization was nose tip (32%). 44% of patients were reconstructed with skin grafts or composite graft, 56% with flaps (48% local flaps and 4% association of both methods). Limited central resections of nose tip were reconstructed with skingraft. The Rybka flap and bilobed flap were the preferred choice for lateral reconstruction of nose tip. Largest tip defects were reconstructed using Rieger flap or forehead flap. The dorsum was often reconstructed with local flap: glabellar on the upper part, Rieger on the lower part. Lateral side was perfectly reconstructed with island flap. Nose wings needed framework: composite graft was the judicious choice in case of limited tissue loss whereas forehead flap with framework or Schmid-Meyer flap were chosen for larger defects. CONCLUSION: Our past experience in nasal reconstruction has provided us with an original decision tree to guide surgeons in choosing the right reconstruction technique according to the size and localization of the defect.