If you don't remember your password, you can reset it by entering your email address and clicking the Reset Password button. You will then receive an email that contains a secure link for resetting your password
If the address matches a valid account an email will be sent to __email__ with instructions for resetting your password
A 30-year-old woman with known pseudoxanthoma elasticum was referred for surgical correction of extensive loose neck skin. She underwent a standard rhytidectomy, which produced minimal improvement. Revision surgery using a vertical elliptical skin excision, incorporating a Z-plasty was undertaken 3 months later and resulted in a satisfactory outcome.
Pseudoxanthoma elasticum (PXE) is a heterogeneous disorder of connective-tissue elastin, in which all body systems may be involved, although the skin, eyes and arterial blood vessels are predominantly affected. Redundant skin around the neck, axillae, trunk and limbs is often the first manifestation of the disease.
Since there is no known successful medical treatment, plastic-surgical correction is one of the few therapeutic options available. Reports on the surgical management of PXE are rare,
and there is no reported case of either revision surgery or midline skin excision with Z-plasty for the correction of the neck deformity.
1. Case report
A 30-year-old woman with PXE was referred by her dermatologist, with a 6 year history of progressive laxity of her neck skin. She had become very embarrassed by the appearance of her neck. She was under the care of a cardiologist for mild mitral-valve prolapse, but had no clinical symptoms or signs of other organ involvement. Clinical examination confirmed extensive neck-skin laxity with a typical ‘hound dog’ appearance.
The patient underwent rhytidectomy with SMAS (superficial musculoaponeurotic system) dissection through a facelift incision. At 4 weeks postoperatively, a disappointing result with residual loose skin in the neck was noted, and the patient requested further surgical improvement (Fig. 1) .
A second operation was performed 3 months after the primary surgery, involving midline excision of excess skin on the neck; the defect was closed by incorporating Z-plasty (skin and platysma) flaps (Fig. 2) . A satisfactory result was obtained, and the patient remains pleased 1 year later (Fig. 3) .
Figure 2(A) The elliptical skin excision (shaded) and the Z-plasty markings, and (B) the final appearance (cf. Fig. 3).
Figure 3Appearance 6 months after revision surgery using the Z-plasty technique. (A) The redundant skin has been eliminated, leaving an acceptable scar. (B) Lateral view, showing a marked improvement in comparison with the preoperative appearance.
PXE is a degenerative dermal elastosis presenting as premature skin laxity with protean clinical manifestations and is inherited in an autosomal dominant (subdivided into types I and II) or recessive (also subdivided into types I and II) pattern.
and is linked to a variety of other surgical and medical illnesses. Cutaneous, vascular and ocular problems become apparent from the second decade and may eventually cause serious disability. The pathology is primarily an abiotrophy of elastin with calcium deposition in aggregated elastin fibres leading to progressive fragmentation, dystrophic calcification and skin fragility with loss of elasticity. Occlusion of blood vessels and bleeding is the manifestation in other organs.
A literature search revealed only seven previous reports of plastic-surgical procedures on patients with PXE. Pickrell et al demonstrated the surgical benefits to the anterior neck for patients with PXE by using a low collar incision, mobilisation of the skin flaps superiorly and inferiorly, excision of the excess skin and suturing of the overlapping flaps.
Crikeliar excised the skin in two stages using three separate incisions, including a long incision parallel to the sternomastoid on each side of the neck, which resulted in multiple long scars.
Complications include slow wound healing and calcium-plaque extrusion through the healing scar.
Mr D. J. Crockett first described the vertical Z-plasty technique to the senior author, who has used it in a few cases of lax neck skin excision in male patients. Biggs advocated a T–Z-plasty (similar to this technique, with transverse scar components at the proximal and distal ends) for cosmetic excision of limited primary or secondary redundant neck skin,
both recommended the technique for selected male patients. In our female patient, since rhytidectomy produced inadequate improvement, we used this rare approach to achieve a satisfactory result.
In conclusion, rhytidectomy can be effective in the management of lax skin in PXE, but, if the results are unsatisfactory, vertical elliptical excision incorporating a Z-plasty can prove effective. We believe that this surgical technique merits consideration as a primary or secondary procedure that can improve the physical appearance in these patients.
References
Kaplan E.N
Henjyoji E.Y
Pseudoxanthoma elasticum: a dermal elastosis with surgical implications.
00123-1&id=gr1.jpg){kind=link}
00123-1&id=gr2.gif){kind=link}
00123-1&id=gr3.jpg){kind=link}