Extensive facial angiofibromas in tuberous sclerosis treated with carbon dioxide laserbrasion

Introduction

Facial angiofibromas are one of the major cutaneous manifestations of systemic sclerosis occurring in about 90% of these patients. The lesions cause significant cosmetic disfigurement leading to emotional distress. The treatment of these lesions with various therapeutic modalities has not been very satisfactory[1],[2],[3] because of the multiplicity of the lesions and the presence of the lesions at anatomically difficult sites like nasolabial folds, chin, upper lip, nose and periorbital areas, particularly so in mentally retarded uncooperative patients. We treated four such patients with CO2 laserbrasion with good cosmetically acceptable results.

Materials and Methods

In this study 4 female patients between 12-22 years in age having extensive tuberous sclerosis-associated angiofibromas involving the face were taken up.In all the patients, a detailed clinical history including the duration of the lesion(s), evolution, symptoms if any, and the previous therapeutic modalities used, if any, were recorded. A thorough clinical examination of the lesions including the examination of the old injury scar, BCG scar etc, to look for the keloids/hypertrophic scars was performed. The diagnosis in each patient was made purely on the basis of clinical features. The patients having history of hypertrophic scars/ keloids and bleeding disorders were excluded from the study. The surgical laser system used was model C-40 unit, an indigenous carbon dioxide laser apparatus developed by Centre for Advanced Technology, Department of Atomic Energy, Indore, India. It employs Carbon dioxide, Nitrogen and Helium (1:2.5:12) gases to produce 10600 nm wavelength laser energy. The lesions were vaporized in two sittings in 2 patients and in three sittings in the other 2 patients at an interval of 2 weeks each using the power setting of 12-16 watts delivered in continuous defocussed mode. The charred surface was cleaned with 3% hydrogen peroxide or sterile saline after laserbrasion. Topical antibiotic (framycetin sulphate 1%) cream was used in all the patients till the ulcers healed and subsequently they used sunscreen lotion. The patients were evaluated at the end of 1st and 2nd week and then every month for 6 months to assess the overall therapeutic response and side effects like colour changes, itching, pain, infection, scarring, atrophy and recurrence etc. if any. The final assessment was done at the end of 6 months.

Results

The present study had 4 patients, all females, between 12-22 years in age (mean age 16.8 years) having extensive angiofibromas involving the central part of the face, nose, nasolabial folds, cheeks, upper lip, periorbital areas [Figure - 1], chin forehead for 2-10 years. The lesions were asymptomatic and gradually increasing in number in all the patients. Tuberous sclerosis associated shagreen patch and ash-leaf macules were present in all the patients however periungual angiofibromas were there in one patient only. One patient had mild to moderate degree of mental retardation. After laserbrasion the ulcers healed in 2 weeks while the erythema persisted for about 4 weeks in all the patients. The lesions improved by 80% [Figure - 2] in 2 patients while the improvement was about 50% in the other 2 patients at the end of 6 months of treatment. The recurrence of lesions as a few small papules were noticed in 2 patients. Two patients had post treatment hyperpigmentation which was treated with 5% topical hydroquinone lotion and sunscreens. There was no scarring or any other side effect in any of the patients.

Discussion

Angiofibromas of systemic sclerosis cause disfiguring facial deformity. Various therapeutic modalities used in the past like electrocoagulation, cryosurgery, shave excision, and a combination of shave excision and dermabrasion have not given entirely satisfactory results.[1],[2],[3],[4] Electrocoagulation of individual lesions is a time consuming, difficult and cumbersome process. The recurrence rate is also high after the procedure. Diver et al reported a patient of angiofibroma treated with cryosurgery with good improvement which lasted for over 18 months. Shave excision of the lesions gives good immediate results in some patients however the recurrences are common with this procedure.[3] Shave excision and dermabrasion has been tried in some patients with extensive lesions. However because of the presence of the lesions on nose, nasolabial folds, chin, upper lip and periorbital areas etc., it has been difficult to carry out the procedure and the problems of post operative wound management, scarring and recurrences were there. Therefore it has not been very successful in these patients.

CO2 laser has been effectively used for facial angiofibromas by various workers with long lasting improvement and without scarring.[5],[6],[7],[8] It has given superior cosmetic results compared to other therapeutic modalities. The recurrences have been minimal with C02 laser even on long term follow-up. According to Bel lack et al C02 laser is the treatment of choice for these patients.[8] In this study we treated 4 patients of extensive facial angiofibromas on an outpatient basis f n C02 laser in continuous mode using an irradiance of 320 watts/cm2 to 63,000 watts/cm2 by holding the hand piece of the laser at varying distance from the target tissue. All the 4 patients had 50-80% improvement in their lesions which was cosmetically well acceptable. Perhaps better results would have been obtained with more treatment exposures and better patient compliance. Recurrences as small papules were seen in some treated areas in 2 patients. Our results are similar to the ones reported by other workers in fair skin patients though our patients had skin type IV and V. Erythema of the treated sites lasting up to 4 weeks and hypopigmentation were seen in all our patients. Two patients had post laser hyperpigmentation which is perhaps uncommon in fair skin patients. Both these patients were treated with topical 5% hydroquinone lotion and sunscreens.