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On-line version ISSN 1806-4841
An. Bras. Dermatol. vol.85 no.4 Rio de Janeiro July/Aug. 2010
Daniela Rezende NevesI; Daniel Gontijo RamosII; Geraldo Magela MagalhãesIII; Rogério da Costa RodriguesIV; Joana Barbosa Alves de SouzaV
IDoctor of the "Santa Casa" Hospital of Belo Horizonte, on going specialization course run by the Faculty of Medical Sciences of Minas Gerais/Post-Graduation
and Research Center- Belo Horizonte(MG), Brazil
IIPreceptor of Surgical Dermatology of the "Santa Casa "Hospital of Belo Horizonte - Belo Horizonte (MG), Brazil
IIIPreceptor of Surgical Dermatology and Cosmiatry of the "Santa Casa" Hospital of Belo Horizonte - Belo Horizonte (MG), Brazil
IVDermatologist, member of the Brazilian Society of Dermatology - Belo Horizonte - (MG), Brazil
VDermatologist, member of the Brazilian Society of Dermatology - Belo Horizonte - (MG), Brazil
Photodynamic therapy is an effective alternative for the treatment of non-melanoma skin cancer, selectively destroying the neoplastic cells through the use of photosensitizer substances that are irradiated with a source of light of adequate wave length. Nevoid Basal Cell Carcinoma Syndrome is a genodermatosis characterized by multiples basal cell carcinomas occurring at an early age, compelling patients to various surgeries, some of them disfiguring. The aim of this article is to show the excellent result of the treatment of multiple basal cell carcinomas on the scalp of a patient suffering from Nevoid Basal Cell Carcinoma Syndrome.
Keywords: Basal cell nevus syndrome; Carcinoma, basal cell; Photochemotherapy
A variety of malignan and pre-malignant cutaneous conditions, including basal cell carcinoma (BCC), actinic keratoses (AK) and Bowen disease have been efectively treated with photodynamic therapy (FDT). 1,2
The 5-aminolevulinic acid (ALA) and its derivative methylamineolevulinato (MAL) are topic photosensitizers used in FDT. When employed, they are selectively absorbed by the neoplastic cells and converted into a photoactive porphyria: the protoporphyrin IX.1 After the illumination of the sensitized tissue with a source of light of adequate wave length, the photoactive porphyrins are stimulated and produce great amount of energy. This energy is transferred to molecules of oxigen, resulting in the formation of reactive species of oxigen, specially of singlet oxigen that, for being cytotoxic, cause the destruction of the sick tissues. Protoporphyrin IX is metabolised in heme, as it becomes photodynamically inactive within 24 to 48 hours.4
The most common adverse effects are phototoxical reactions,described as burning sensation during the treatment and erythema, persisiting for more than 7 days. Pigmentary alterations are rare and, when they occur, they have short duration. Other reactions are usually uncommon.5
The use of topical anesthetics before the irradiation should not be done once the acid pH of anesthetics can inactivate chemically the photosensitizers.6 I n general, FDT is well tolerated and, in some cases, pain can be relieved by using oral painkillers, given an hour before the procedure. Although the sirurgy continues to be the gold rule for the treatment of BCC, FDT is an efficient alternative for those patients with inumerous lesions, with large lesions or when the surgical treatment will cause great esthetic damage. Another indication is for patients without clinical conditions to undergo surgical procedures. (elderly people, pacemaker, coagulation disorders).7
The Nevoid Basal Cell Carcinoma Syndrome. (NBCs) or Gorlin-Goltz Syndrome is a rare genodermatosis, of dominant autosomatical inheritance, with variable penetrance. Mutations in the PTCHED gene have been considered as influencing its pathogenesis. It is characterized by the appearing of multiple CBC in precocious age and many stigmas of abnormal development. 4
FDT is a therapeutic option which offers excellent or good esthetic results,1 that is particularly important when BCC is concerned as it is a neoplasia more frequently found in relatively young patients, such as, the carriers of the Nevoid Basal Cell Syndrome.
S.R, aged 50, female, with various lesions on the scalp, reported the begining of the condition when she was 15 years old, with inumerous surgical procedures since then.
When examining the patient we observed an erythematic ulcerated lesion, measuring 6,0 x 5,0 cm, and three minor lesions measuring, on average, 1,5 x 1,0 cm on the right temporal region and ten erythematic lesion, the colour of a pearl, of an average size of 1,0 x 1,0 cm on the left temporal region.The patient also presented palmo-plantar pits, ocular hypertelorism, polydactylism and syndactyly of the third and fourth chirodactyls. Radiologycal studies did not show odontogenic cysts nor intracranial calcifications. Incisional byopsies on the lesions were carried out, from which the hystopathologic diagnosis revealed solid BCC infiltrating the dermis (Picture 1) confirming our clinical hypothesis of Nevoid Basal Cell Syndrome.
The patient was directed to the service of plastic surgery for therapeutic evaluation. As a consequence of the great number of lesions on the scalp two approaches were sugested: exeresis of the lesions with healing by second intention or use of skin expanders with posterior resection of the lesions and reconstruction by primary closure. Due to the aggressiveness of these surgical procedures of high morbidity we proposed the FDT with MAL and red light of narrow band for the treatment of lesions on the scalp.
The first FDT session was carried out on 20th August, 2007. The patient was submitted to a trichotomy on the whole scalp, with curretage of the lesions. (Picture 2). Following, a layer of medication was put, with a tickness,of 1 mm and in 5 to 10 mm around the normal skin.8 It was used a tube for lesions on the scalp. A dressing with PVC plastic and aluminum paper was put on the lesions to avoid exposition of the medication to the light. It was given a sublingual pill of ketorolac of thrometamine 30 mg (ToragesicR), a non-hormonal anti-inflamatory, 30 minutes before the illumination.Three hours later we removed the MAL layer and the area was irradiated with a LED, using red light (Aktlite), with an average wave lenght of 670 nm and total fluency of 37J/cm2,during 7 minutes and 50 seconds. The patient tolerated very well the treatment without being necessary the interruption of the treament in any moment of the irradiation.
The patient returned to the service after one week without any complaints. Exam showed that she presented erythema and adherent crust on the scalp, without infectious signs.
On September, 10th, 2007, it was carried out a second session. With a curet the crusts were removed and the procedure of the first session was carried out in the same way.
A week later, the patient returned to the service presenting meliceris crusts in the treated areas (Picture 3). It was initiated the treatment with a topical antibiotic, mupirocin cream, 2%, twice a day during 10 days, with improvement of the condition. New biopsies to control the cure were carried out 9 months after the treatment with FDT (Picture 4) The patient remains in attendance at the service and up to now new lesions have not been identified, neither on the areas of alopecia nor on the treated areas (Picture 5).
There are various reasons for the treatment of BCC. The surgical treatment is a therapeutic option relatively secure, following a previous planning, adequated to the histopathological type, size and location of the BCC.9 The reconstruction using skin expanders is useful, in large or multiple lesions in areas such as: scalp and cervical region. One of the disadvantages of this procedure is the performance of the treatment in two surgical events and the great physical and psychological discomfort during the expansion of the skin which is slow and gradual. In general, it is indicated the use of expanders when it is recquired the best esthetic result without being available any other reconstruction alternative with comparable results. The impossibility of a histhopathological control and the residual hyperpigmentation are some of the disadvantages of cryotherapy. Among the disadvantages of radiotherapy we could mention: the impossibility of ensuring (through microscopic exam) the inclusion of all the lesion (within the treatment fields) and the difficulty of future execution of surgical procedures on the irradiated areas (in the face of the formation of fibrotic tissue).
Dijkstra et al showed successful treatment of basal cell carcinoma with FDT, using ALA and red light (400-450 mm) in the population he studied, including three patients with NBCS. The possibility of treating large areas in a unique procedure allows carriers of the syndrome to treat not only the clinical basocellular carcinomas but also the subclinical ones without any scars.10
THe FDT showed to be the most adequate option of treatment for our patient due to the number, site and size of the lesions. The two sessions were well tolerated having as the only intercurrence a secondary bacterial infection after the second session. Although pigmented BCC is not an indication for FDT treatment, we observed an important decrese on the tumor which allowed its removal with primary closure. The esthetic result was excellent and there was no prejudice to the regrowth of hair on the scalp, result particularly interesting, thanks to the sex of the patient (Picture 5).
The FDT is an alternative therapeutics for extense and non melanoma malignant lesions. 11 Other methods such as radiotherapy, cryotherapy or traditional surgery might bring, apart from undesirable scars, cicatricial alopecia.12
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12. Lui H, Hobbs L, Tope WD, Lee PK, Elmets C, Provost N, et al. Photodynamic therapy of multiple nonmelanoma skin cancers with verteporfin and red light-emitting diodes: two-year results evaluating tumor response and cosmetic outcomes. Arch Dermatol. 2004;140:26-32. [ Links ]
Mailing address: Received on 29.12.2008. * Work carried out in the Ambulatory of Dermatology of the "Santa Casa" Hospital of Belo Horizonte- - Belo Horizonte (MG), Brazil.
Daniela Rezende Neves
Rua Rio Grande do Sul 1030 / apt. 2002 - Santo Agostinho
30170 111 Belo Horizonte - MG, Brazil
Tel.: 31 9427 77-44 31 329249-05
Approved by the Advisory Board and accepted for publication on 29.01.2010
Conflict of interest: None
Financial funding: None
Received on 29.12.2008.
* Work carried out in the Ambulatory of Dermatology of the "Santa Casa" Hospital of Belo Horizonte- - Belo Horizonte (MG), Brazil.