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By:  Robert J. Hoehe, M.D.

Dr. Neldner sent me my first PXE patients in the 1970s. The patients were requesting plastic surgery to remove some of the wrinkles and redundant skin folds from their necks, axillae (armpits), and groin areas. Several basic questions had to be answered before surgery could be considered. The first was obvious – could plastic surgery improve the appearance of PXE skin? What were the risks? Does PXE-affected skin heal well? Do individuals with PXE bleed more easily or scar worse than normal?

Most of these questions were soon answered. PXE-affected skin does heal well, with no additional risk factors from the underlying PXE, and they do not bleed any more easily than normal. The resulting surgical scars are generally comparable to those in patients without PXE. 

I learned early on that the neck is the site most commonly requested for plastic surgery because it is obviously the most cosmetically visible affected area. Another important lesson was that simple excision of affected skin from the neck could easily remove much of the objectionable skin, but would leave a linear scar around the neck that could be more cosmetically apparent than the original PXE-affected skin. So I developed a new technique called a “neck lift,” which is based on surgical methods similar to a face lift, but performed on a lateral neck. An incision is made behind the ears in the hairy scalp. The affected skin is freed up and pulled up and back and excised behind the ears and above the hairline. The wound is then closed in the scalp hairy area where the scar cannot be seen. This technique has produced excellent results such that the newly tightened skin on the lateral neck shows immediate improvement because new wrinkles do not seem to form readily in the treated area.

The axilla (armpit) is the second most requested area for surgical removal of the folded, baggy skin that is prone to develop in this area. There are additional problems here because even in individuals without PXE axillary skin is more likely to scar with healing. I tried various Z-type incisions and can obtain good improvement in the axilla, but with a somewhat longer healing time and more tendencies to scar formation. Nonetheless, most patients who have had axillary surgery for PXE are eventually happy with the results.

The groin is the third most common area for possible redundant skin folds due to PXE. Therefore, it is a candidate for plastic surgery. This area actually turns out to be the easiest to treat surgically because it is possible to simply excise a linear strip of affected skin from the inguinal crease area and directly close the surgical defect with a linear closure that is hidden in the normal inguinal crease between the lower abdomen and the thigh.

Skin grafts are possible in PXE skin and will heal well, though with considerable scarring around the graft site. This, however, is true for skin grafts on anyone. For this reason, skin grafts are usually reserved for such problems as badly burned areas or large surgical defects resulting from excisions of large tumors from the skin.

By:  Robert J. Hoehe, M.D. FACS 6:4 (1998)