PURPOSE

Xeroderma pigmentosum is an autosomal recessive disease characterized by an endonuclease enzyme defect and resulting in marked vulnerability of the skin to ultraviolet radiation. Although conservative surgical resection the primary therapy for skin tumors in patients with xeroderma pigmentosum, severe cases have been treated by excision of large portions of facial surface and grafting with uninvolved skin. The purpose of this paper is to report a xeroderma pigmentosum case with multiple malignant melanoma on her face and radical excicion of total facial skin followed by facial resurfacing with monoblock full- thickness skin graft achieved by abdominoplasty material.

CASE REPORT

A thirty-five-year-old woman was referred to our clinic with a multiple pigmented lesion on her face. She had three brothers in good health and her family hisory revealed neither xeroderma pigmentosum nor any other skin disease.

The patient was 5 years old when freckling pigmentations first appeared on her face. The first pigmented lesion on her face appeared two years ago and she had had no treatment or had not been seen by a doctor until her admission to our clinic. Sun exposed areas of her body showed multiple freckling pigmentations. Clinical diagnosis was xeroderma pigmentosum.

Physical examination of the patient revealed multiple pigmented lesions located on bilateral malar region, dorsum of the nose, glabella, frontal region, right half of the chin changing dimensions 0.5x0.5x1 cm to a maximum of 3.5x3x2 cm (Figure 1)

Figure 1

We performed multiple excisional biopsies and pathology revealed that multiple invasive malignant melanoma. After pathological diagnosis excision of almost whole facial skin and resurfacing with a monoblock full thickness skin graft was planned given ubiguity of the invasive tumors the age of the patient.

There was no sign of systemic metastases at physical examination and laboratory data. The upper and lower eyelids , eyebrows, alar rim and vermillon were spared because of challenges in reconstruction of these areas. Except these regions the whole facial skin was excised under general anesthesia (Figure 2)

Figure 2

At the same time we performed an abdominoplasty according to the pattern of the face prepared preoperatively and we harvested monoblock skin graft from this abdominoplasty excision material. The graft was adapted to the excised area of the face (Figure 3,4,5).

Figure 3

Figure 4

Figure 5

The donor area was primary repaired. Pressure bandages were applied to face and a nasogastric catheter was applied for the immobilization of perioral region but patient denied nasogastric catheter and we could not use this.

On the first control examination during the fifth postoperative day graft on the face was good but after 1 weeks, there was perioral necrosis (Figure 6).

Figure 6

We performed debridment and full-thickness skin graft harvested form right inguinal, left and right end of the abdominoplasty incision (Figure 7)

Figure 7

Postoperrative there was no graft problem and achieved acceptable result. The patient has been followed for three months and there has been no recurrence so far (Figure 8)

Figure 8

CONCLUSİON

Face reconstruction is a challenging procedure that there are many techniques including allotransplantation, expanded skin flaps, split thickness skin grafting. Allotransplantation has some difficulties that immunsupresion, total loss of transplanted face,  technique details. Expanded flaps are limited usage for total face reconstruction. Split thickness skin grafting has a poor aesthetic results.

For xeroderma pigmentosum patients who developed a large number of invasive tumors, complete resurfacing of exposed skin with a full thickness skin grafting provides acceptable aesthetic and reconstructive result. Monoblock full thickness skin graft achieved by abdominoplasty material is a good donor choice for suitable cases.

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