Xeroderma Pigmentosum : resurfacing versus dermabrasion K. Agrawal,
A. J. Veliath,
S. Mishra
and K. N. Panda
Departments of Plastic Surgery and Pathology, Jawaharlal Institute of Postgraduate Medical Education and Research. Pondicherry, India SUMMARY.
Three patients
of Xeroderma
Pigmentosum
(XP) have been managed
for recurrent tumours.
Improvement in pigment pattern was observed at both the donor as well as the recipient sites. An attempt has been made to prevent further occurrence of tumours over the exposed parts of the body by resurfacing and also by dermabrasion. A comparative study of the two procedures is being carried out and the preliminary results are discussed. Deep dermabrasion appears to be preferable as a prophylactic procedure.
Case 3
Xeroderma Pigmentosum (XP) is a relatively rare autosomal recessive disorder (Robbins, 1974) in which exposure to the ultra-violet rays of the sun results in ectodermal and mesodermal tumours (Gleason, 1970). In spite of the discovery of the genetically determined DNA defect in XP (Cleaver, 1968) there is no cure and the outlook for patients with this disease remains bleak. Until now the treatment modalities have been prophylaxis by avoidance of exposure, surgical excision of premalignant and malignant tumours and resurfacing with skin grafts, and dermabrasion (Epstein et al., 1972). The rarity of XP renders controlled trials of any treatment modality impracticable (Ashall et al., 1987). However, we have attempted to carry out controlled trials of dermabrasion and resurfacing on two halves of the faces of two patients.
A 22-year-old female had, after previous treatments, multiple hyperkeratotic lesions over the face (Fig. 5). Excision of the lesions and simultaneously deep dermabrasion of the right half of the face was carried out prophylactically. Since then the patient’s face is tumour free at 18 months (Fig. 6).
Results Where skin grafts have been used, the donor sites of all these grafts have remained clear and nonxerodermatous in contrast to the adjacent skin, which is still darkly speckled in all cases (Figs 7, 8). The grafted areas in all our cases also remained relatively smooth and satisfactory on follow-up. We have followed up grafts in our cases from 21 months7 years. All the graft sites have behaved in a similar fashion and are tumour free. The prophylactically dermabraded areas with follow up of 18 months-2 years in these three cases have also remained tumour free. The dermabraded skin remained speckled and xerodermatous when superficial dermabrasion was performed (Figs 2,4). When dermabrasion was done at a deeper level the skin became non-xerodermatous, and has remained so in a followup of 18 months (Fig. 6). Since in two patients the graft has been applied on one side of the face and dermabrasion has been carried out on the other side, we have been able to compare the two areas in determining their effectiveness in the prevention of occurrence of tumour in XP patients.
Case 1 A 12-year-old boy had had multiple skin cancers and tongue cancer treated surgically by us since age 5 (Fig. 1). He presented with multiple lesions on the right side of the face and part of the scalp. The left side of the face was relatively tumour free. Excision of skin on the right half of the face and the scalp lesions and split skin grafting was carried out. Prophylactic dermabrasion was performed over the left half of the face and uninvolved scalp. Nose reconstruction was done with an oblique forehead flap. The grafted as well as dermabraded areas have been free from tumour for 2 years (Fig. 2). Case 2 A IO-year-old female, the younger sister of case 1, had had skin cancers and tongue cancer treated surgically on numerous occasions. She presented with multiple squamous cell carcinoma and hyperkeratotic lesions over the right side of the face (Fig. 3). Excision of the skin on the right side of the face was performed and it was resurfaced with a split skin graft taken from the thigh. Simultaneously, prophylactic dermabrasion of the left half of the face was done. Since then the dermabraded as well as the grafted sites have been tumour free for over 21 months (Fig. 4).
Discussion Moore and Iverson (1954) were the first to report a 2year recurrence free follow-up after radical excision of large areas of skin followed by resurfacing with skin harvested from the thighs and legs, which is less exposed to solar radiation and hence less damaged. In 311
British
Fig. 1 1. Recurrence of tumours over right side of face (left Figure I-Case of the left half of the face. of the right half and dermabrasion
of Plastic
Sureerv
Fig. 2
: previously enucleated). eye
Figure 2-Case
Fig. 3
1. Two years after resurfacing
Fig. 4
2. Preoperatively, with multiple tumours over the rilght side of the face. Figure 4-Case Figure &Case right side and dermabrasion of the left side of the face.
our ntaximum follow-up period of 7 years in case 1 and 5 yea.rs in case 2, the grafted areas remained ttimour free alnd relatively plain when compared to the original skin. Gleason (1970) has reported on a 5-year followup wrhere, although the grafted skin became hyperpigm ented like the original skin, it remained tumour free. McJuly et al. (1980) have recommended scalp as a dono r site likely to provide disease free grafts. How-
Journal
2. 21 months
after resurfacing
of the
ever, two of our cases (cases 1 and 2) had multiple scalp lesions which needed repeated excision and split skin grafting, making it unsuitable as a donor site (Agrawal, 1992). Dermatome shaving and dermabrasion as a prophylactic manoeuvre in XP have been considered useful (Epstein et al., 1972). The benefit accrues from reepithelialisation by the cells derived from those lying deep in the skin adnexae which have received less
Xeroderma Pigmentosum : Resurfacing versus Dermabrasion
313
Fig. 5 Figure S--Case 3. Recurrence
of turnours
Fig. 6 over the right cheek. Figure &Case
Fig. 7 Figure 7 -Donor
areas of case
I : 1-7 years follow-up. Figure 8-Donor
exposure to UV-rays. The cosmetic result has been claimed to be better with dermabrasion than that achieved by split skin grafting. Our observations agree with this. However, we also observed that when superficial dermabrasion was carried out in the first two cases, the dermabraded areas remained xerodermatous and speckled like the adjacent areas, while the split skin graft donor sites remained relatively smooth even after 7 years of follow-up. The normal appearance of donor sites after dermatome shaving has been reported after a follow-up of 2 years only
3. 18 months
after dermabrasion
of the right half of the face.
Fig. 8 areas of case 2: 21 months-5 years follow-up.
(Epstein et al., 1972). Hence a deeper dermabrasion has been carried out in case 3 to reproduce the effect seen at the split skin graft donor site and we have observed that the skin has remained non-xerodermatous after a period of 18 months. We therefore recommend deeper dermabrasion for prophylaxis. As regards the cosmetic superiority and lack of recurrence in dermabraded areas, a longer follow-up is required before the supremacy of one procedure over the other can actually be judged. One must allow that resurfacing is relatively difficult as well as more
British Journal of Plastic Surgery
314 deforming, while dermabrasion is technically a simpler procedure. We are in agreement with Ashall et al. (1987) that not all patients require a radical surgical approach, which should be reserved for those who develop a large number of invasive tumours. Finally, the importance of conservative methods such as avoidance of exposure to sunlight should be emphasised, despite modern innovative surgical techniques.
Cleaver, J. E. (1968). Defective repair replication of DNA in Xeroderma Pigmentosum. Nature, 218, 562. Epstein, E. H., Burk, P. G., Cohen, I. C. and Deckers, P. (1972). Dermatome shaving in the treatment of Xeroderma Pigmentosum. Archives of Dermalology, 105, 589. Gleason, M. C. (1970). Xeroderma Pigmentosum: five year arrest after total resurfacing of the face. Plastic and Reconsrructive Surgery, 46, 577. Moore, C. and Iverson, P. C. (1954). Xeroderma Pigmentosum. Cancer, I, 377. Mouly, R., Dufourmentel, C., Banzet, P. and Papadopoulos, 0. (1980). Xeroderma Pigmentosum. Annals de Chirurgie Pfastique, 25, 117. Robbins, J. H., Kraemer, K. H., Lutzner, M. A., Festoff, B. W. and Coon, H. G. (1974). Xeroderma Pigmentosum. Annals of Internal Medicine, 80, 221.
Acknowledgements The authors would like to thank the Department of Medical Illustration Division for preparing the photographs. We thank the Medical Records Division of our Institute for permitting us to use the records. We are grateful to the Department of Pharmacology for allowing us to use the computer and printing facilities. We sincerely thank Dr (Mrs) Apama Agrawal, MD, for her unstinted help in compiling the work and preparing the script.
References Agrawal, K. (1992). Xeroderma
Pigmentosum.
(letter). British
Journal of Plastic Surgery, 45, 72.
Ashall, G., Quaba, A. A. and Hackett, M. E. J. (1987). Facial resurfacing in Xeroderma Pigmentosum : are we spoiling the ship for a ha’p’orth of tar? British Journal of Plastic Surgery, 40,610.
The Authors K. Agrawal, MS, MCh, Associate Professor and Head, Department
of Plastic Surgery A. J. Vellath, MD, Director Professor and Head, Department of Pathology S. Mishra, MS, MCh, Senior Medical Officer, Department of Plastic Surgery K. N. Panda, MS, Senior Medical Officer, Department of Plastic Surgery Jawaharlal Institute of Postgraduate search, Pondicherry 605 006, India.
Medical Education and Re-
Requests for reprints to Dr K. Agrawal. Paper received 23 October 1991. Accepted 12 December 1991.
A. J. Veliath,
S. Mishra
and K. N. Panda
Departments of Plastic Surgery and Pathology, Jawaharlal Institute of Postgraduate Medical Education and Research. Pondicherry, India SUMMARY.
Three patients
of Xeroderma
Pigmentosum
(XP) have been managed
for recurrent tumours.
Improvement in pigment pattern was observed at both the donor as well as the recipient sites. An attempt has been made to prevent further occurrence of tumours over the exposed parts of the body by resurfacing and also by dermabrasion. A comparative study of the two procedures is being carried out and the preliminary results are discussed. Deep dermabrasion appears to be preferable as a prophylactic procedure.
Case 3
Xeroderma Pigmentosum (XP) is a relatively rare autosomal recessive disorder (Robbins, 1974) in which exposure to the ultra-violet rays of the sun results in ectodermal and mesodermal tumours (Gleason, 1970). In spite of the discovery of the genetically determined DNA defect in XP (Cleaver, 1968) there is no cure and the outlook for patients with this disease remains bleak. Until now the treatment modalities have been prophylaxis by avoidance of exposure, surgical excision of premalignant and malignant tumours and resurfacing with skin grafts, and dermabrasion (Epstein et al., 1972). The rarity of XP renders controlled trials of any treatment modality impracticable (Ashall et al., 1987). However, we have attempted to carry out controlled trials of dermabrasion and resurfacing on two halves of the faces of two patients.
A 22-year-old female had, after previous treatments, multiple hyperkeratotic lesions over the face (Fig. 5). Excision of the lesions and simultaneously deep dermabrasion of the right half of the face was carried out prophylactically. Since then the patient’s face is tumour free at 18 months (Fig. 6).
Results Where skin grafts have been used, the donor sites of all these grafts have remained clear and nonxerodermatous in contrast to the adjacent skin, which is still darkly speckled in all cases (Figs 7, 8). The grafted areas in all our cases also remained relatively smooth and satisfactory on follow-up. We have followed up grafts in our cases from 21 months7 years. All the graft sites have behaved in a similar fashion and are tumour free. The prophylactically dermabraded areas with follow up of 18 months-2 years in these three cases have also remained tumour free. The dermabraded skin remained speckled and xerodermatous when superficial dermabrasion was performed (Figs 2,4). When dermabrasion was done at a deeper level the skin became non-xerodermatous, and has remained so in a followup of 18 months (Fig. 6). Since in two patients the graft has been applied on one side of the face and dermabrasion has been carried out on the other side, we have been able to compare the two areas in determining their effectiveness in the prevention of occurrence of tumour in XP patients.
Case 1 A 12-year-old boy had had multiple skin cancers and tongue cancer treated surgically by us since age 5 (Fig. 1). He presented with multiple lesions on the right side of the face and part of the scalp. The left side of the face was relatively tumour free. Excision of skin on the right half of the face and the scalp lesions and split skin grafting was carried out. Prophylactic dermabrasion was performed over the left half of the face and uninvolved scalp. Nose reconstruction was done with an oblique forehead flap. The grafted as well as dermabraded areas have been free from tumour for 2 years (Fig. 2). Case 2 A IO-year-old female, the younger sister of case 1, had had skin cancers and tongue cancer treated surgically on numerous occasions. She presented with multiple squamous cell carcinoma and hyperkeratotic lesions over the right side of the face (Fig. 3). Excision of the skin on the right side of the face was performed and it was resurfaced with a split skin graft taken from the thigh. Simultaneously, prophylactic dermabrasion of the left half of the face was done. Since then the dermabraded as well as the grafted sites have been tumour free for over 21 months (Fig. 4).
Discussion Moore and Iverson (1954) were the first to report a 2year recurrence free follow-up after radical excision of large areas of skin followed by resurfacing with skin harvested from the thighs and legs, which is less exposed to solar radiation and hence less damaged. In 311
British
Fig. 1 1. Recurrence of tumours over right side of face (left Figure I-Case of the left half of the face. of the right half and dermabrasion
of Plastic
Sureerv
Fig. 2
: previously enucleated). eye
Figure 2-Case
Fig. 3
1. Two years after resurfacing
Fig. 4
2. Preoperatively, with multiple tumours over the rilght side of the face. Figure 4-Case Figure &Case right side and dermabrasion of the left side of the face.
our ntaximum follow-up period of 7 years in case 1 and 5 yea.rs in case 2, the grafted areas remained ttimour free alnd relatively plain when compared to the original skin. Gleason (1970) has reported on a 5-year followup wrhere, although the grafted skin became hyperpigm ented like the original skin, it remained tumour free. McJuly et al. (1980) have recommended scalp as a dono r site likely to provide disease free grafts. How-
Journal
2. 21 months
after resurfacing
of the
ever, two of our cases (cases 1 and 2) had multiple scalp lesions which needed repeated excision and split skin grafting, making it unsuitable as a donor site (Agrawal, 1992). Dermatome shaving and dermabrasion as a prophylactic manoeuvre in XP have been considered useful (Epstein et al., 1972). The benefit accrues from reepithelialisation by the cells derived from those lying deep in the skin adnexae which have received less
Xeroderma Pigmentosum : Resurfacing versus Dermabrasion
313
Fig. 5 Figure S--Case 3. Recurrence
of turnours
Fig. 6 over the right cheek. Figure &Case
Fig. 7 Figure 7 -Donor
areas of case
I : 1-7 years follow-up. Figure 8-Donor
exposure to UV-rays. The cosmetic result has been claimed to be better with dermabrasion than that achieved by split skin grafting. Our observations agree with this. However, we also observed that when superficial dermabrasion was carried out in the first two cases, the dermabraded areas remained xerodermatous and speckled like the adjacent areas, while the split skin graft donor sites remained relatively smooth even after 7 years of follow-up. The normal appearance of donor sites after dermatome shaving has been reported after a follow-up of 2 years only
3. 18 months
after dermabrasion
of the right half of the face.
Fig. 8 areas of case 2: 21 months-5 years follow-up.
(Epstein et al., 1972). Hence a deeper dermabrasion has been carried out in case 3 to reproduce the effect seen at the split skin graft donor site and we have observed that the skin has remained non-xerodermatous after a period of 18 months. We therefore recommend deeper dermabrasion for prophylaxis. As regards the cosmetic superiority and lack of recurrence in dermabraded areas, a longer follow-up is required before the supremacy of one procedure over the other can actually be judged. One must allow that resurfacing is relatively difficult as well as more
British Journal of Plastic Surgery
314 deforming, while dermabrasion is technically a simpler procedure. We are in agreement with Ashall et al. (1987) that not all patients require a radical surgical approach, which should be reserved for those who develop a large number of invasive tumours. Finally, the importance of conservative methods such as avoidance of exposure to sunlight should be emphasised, despite modern innovative surgical techniques.
Cleaver, J. E. (1968). Defective repair replication of DNA in Xeroderma Pigmentosum. Nature, 218, 562. Epstein, E. H., Burk, P. G., Cohen, I. C. and Deckers, P. (1972). Dermatome shaving in the treatment of Xeroderma Pigmentosum. Archives of Dermalology, 105, 589. Gleason, M. C. (1970). Xeroderma Pigmentosum: five year arrest after total resurfacing of the face. Plastic and Reconsrructive Surgery, 46, 577. Moore, C. and Iverson, P. C. (1954). Xeroderma Pigmentosum. Cancer, I, 377. Mouly, R., Dufourmentel, C., Banzet, P. and Papadopoulos, 0. (1980). Xeroderma Pigmentosum. Annals de Chirurgie Pfastique, 25, 117. Robbins, J. H., Kraemer, K. H., Lutzner, M. A., Festoff, B. W. and Coon, H. G. (1974). Xeroderma Pigmentosum. Annals of Internal Medicine, 80, 221.
Acknowledgements The authors would like to thank the Department of Medical Illustration Division for preparing the photographs. We thank the Medical Records Division of our Institute for permitting us to use the records. We are grateful to the Department of Pharmacology for allowing us to use the computer and printing facilities. We sincerely thank Dr (Mrs) Apama Agrawal, MD, for her unstinted help in compiling the work and preparing the script.
References Agrawal, K. (1992). Xeroderma
Pigmentosum.
(letter). British
Journal of Plastic Surgery, 45, 72.
Ashall, G., Quaba, A. A. and Hackett, M. E. J. (1987). Facial resurfacing in Xeroderma Pigmentosum : are we spoiling the ship for a ha’p’orth of tar? British Journal of Plastic Surgery, 40,610.
The Authors K. Agrawal, MS, MCh, Associate Professor and Head, Department
of Plastic Surgery A. J. Vellath, MD, Director Professor and Head, Department of Pathology S. Mishra, MS, MCh, Senior Medical Officer, Department of Plastic Surgery K. N. Panda, MS, Senior Medical Officer, Department of Plastic Surgery Jawaharlal Institute of Postgraduate search, Pondicherry 605 006, India.
Medical Education and Re-
Requests for reprints to Dr K. Agrawal. Paper received 23 October 1991. Accepted 12 December 1991.
