Perianal Bowen's disease treated with liquid nitrogen cryosurgery

Abstracts

Doença de Bowen designa carcinoma in situ cuja evolução é lenta e progressiva, geralmente assintomática. Na região perianal essa lesão é pouco freqüente, ocorrendo mais em indivíduos jovens. Quando acomete áreas que não sofreram exposição solar, outros fatores podem estar associados, como HPV, doenças inflamatórias ou neoplasias de cólon. A doença pode ser tratada com cirurgia convencional, criocirurgia ou terapia fotodinâmica. O objetivo é relatar um raro caso clínico da doença de Bowen, em paciente do sexo feminino, jovem, negra, que apresentava uma extensa lesão na região perianal com positividade para HPV. A paciente estava em tratamento para retocolite ulcerativa com suspeita de ter, associadamente, doença de Crohn. Foi tratada com criocirurgia e evoluiu com boa cicatrização do local, sem apresentar sinais de recidiva 34 meses após o tratamento.

Criocirurgia; Doença de Bowen

CASE REPORT

Perianal Bowen's disease treated with liquid nitrogen cryosurgery^*

Aparecida Machado de Moraes^I; Sílvia Helena Rodrigues Leite^II; Maria Letícia Cintra^III; Eliane Ramires Terrazas^IV; Elemir Macedo de Souza^V

^IPh.D., Assistant Professor, Dermatology Department - FCM-Unicamp

^IIResident of the Dermatology Department - FCM- Unicamp

^IIIPh.D., Assistant Professor, Pathological Anatomy Department - FCM- Unicamp

^IVFormer-intern of the Dermatology Department - FCM- Unicamp

^VAdjunct Professor, Dermatology Department - FCM- Unicamp

^{Correspondence}

SUMMARY

Bowen's disease is generally an asymptomatic in situ carcinoma that develops slowly. It rarely occurs in the genital region and mainly affects young persons. Skin lesions occurring in areas not exposed to sunlight may be associated with other factors, like HPV, inflammatory disease or colon neoplasia. The disease may be treated by conventional surgery, cryosurgery or photodynamic therapy. The aim is to present the clinical characteristics of a rare case of Bowen's disease in a young, black female, who had a large lesion in the perianal region, which tested positive for HPV. The woman had been undergoing treatment for intestinal ulcerative rectocolitis or Crohn's disease. She was treated with cryosurgery and there was no sign of recurrence during the ensuing 34 months.

Key words: Bowen's disease; Cryosurgery.

INTRODUCTION

Bowen's Disease (DB) refers to an in situ carcinoma that may appear as plaque lesions whose borders are sharply demarcated, irregular, salient, erythematous-squamous and even verruca, hypo-or hyperpygmented and, eventually, exulcerated.

Its evolution is slow and progressive, generally asymptomatic, with the possible occurrence of local pain, irritation, pruritus and bleeding.¹

Histopathology shows disorganization of the dermis with loss of cellular polarity from the base to the cornea, hyperplasia with fusion of interpapillary crystals, hyperparakeratosis with atypical and malignant diskeratotic cells, and presence of mytosis, without exceeding the primary basal membrane.²

In the perianal region, this abnormality is strongly associated with inflammatory diseases of the colon, like ulcerative rectocolitis,³ Crohn's disease ⁴ and recto carcinoma,⁵ supposedly due to the local irritation produced by the diseases.^3,4 Human Papillomavirus (HPV) has also been made responsible for the appearance of this tumor in the perianal region.^1,6

The disease must be distinguished from Paget's extramammary disease, basocellular carcinoma, seborreica keratosis, dermatosis papulosis nigra, chronic simple lichen, scleroatrophic lichen, malignant melanoma, condylomata acuminatum, eczema and dis colon rectum.²

Therapeutic resources are being studied in order to obtain treatment that reduces complications and prevents recurrence. Currently proposed therapies are surgical excision,⁷ cryosurgery,⁸ photodynamic⁹ and laser therapies.¹⁰

CASE REPORT

A 33-year-old black female patient sought dermatological care for a dark and pruriginous lesion that had appeared in the perianal region eight years earlier.

Her personal medical history brought out the fact that the patient had been undergoing treatment for nonspecific ulcerative rectocolitis, suspected to be an associated case of Crohn's disease.

The dermatological test showed the lesion as a rounded and hyperchromic plaque with some irregularly clearer areas, while other areas presented erythema. The center had a scleroatrophic aspect with more pigmented points at the edge of the area. It was located exactly at the intergluteal fold, and measured about 10 cm in diameter.

The patient was submitted to biopsies in the central area of the lesion and in the periphery. The histopathologic result suggested viral perianal condyloma, associated with a high degree displasia. An immuno-histochemical test was performed, which was positive for the HPV pool.

These findings were compatible with the diagnosis of BD, associated or induced by HPV.

Given the site, extension and associated systemic diseases, cryosurgical treatment with liquid nitrogen was proposed.

The patient was submitted once again to a biopsy at the margins and at one centimeter distance from them in order to evaluate its possible occurrence in apparently normal skin in addition to its clinical manifestation.

The plaque was divided into quadrants. Liquid nitrogen was applied in two cycles to each of the delimited quadrants by a technique employing a jet spray, consisting of 40 seconds of freezing and then completed by an average 2-minute-and-20-second interval. After the procedure, cephalexine was administered orally as well as analgesics for seven days (Figure 1 and 2).

A day after cryosurgery, the patient referred to local pain. The region presented an approximately four-centimeter tense bullosa lesion that was shed on the third day. A week later, the region treated presented a central exulceration with the onset of a peripheral process of scarring.

The histopathological examination performed during the pre-operation procedure demonstrated active disease only in the areas corresponding to the clinical manifestation and with no epithelial histopathologic alterations further than one centimeter beyond the margin. The action of the cryogen was more effective in the lesion, with a discreet effect on the normal skin at the safety margin.

In the one-month follow-up there was a two-centimeter exulcerated area, without any necrosis or phlogistic signs. At the two-month mark, a small central exulcerated area remained. The rest showed excellent wound healing with original repigmentation.

The region was reevaluated six and nine months later. Upon the latter evaluation, it showed partial repigmentation of the lesion. There was no sign of recurrence within the 34- month follow-up (Figure 3).

DISCUSSION AND CONCLUSION

BD generally affects individuals older than 50 years of age, and occurs especially in sun-exposed areas. In the perianal region, this disease is less frequent and affects younger individuals in the 30-40 age group, in accordance with the case described.⁴ Most frequently, the lesion appears as a plaque whose borders are sharply demarcated, possibly irregular, salient, and even verruca and hypopigmented. In the patient described in this paper, the lesion was hyperpigmented, suggesting a reflex of racial pigmentation.¹¹ As the course of BD is slow, progressive and asymptomatic, lesions in the perianal region are frequently diagnosed after the fact. This is why they appear over a large area, as in this patient's case.

This disease, like most cutaneous tumors, is strongly related to sun exposure.¹¹ When occurring in the perianal region, however, other factors have been associated. Currently, the causes most related to the appearance of BD in the anogenital area are related to inflammatory colon diseases, like ulcerative rectocolitis and Crohn's disease and, mainly, to the HPV agent (Human Pappiloma Virus). By means of immuno-histochemical tests, HPV has been found at a high frequency in the lesions, and isolated even in cases of extragenital BD. HPV 16, 18, 34 and 48 subtypes are possibly more related to the development of the disease in the perianal region.¹¹ It is interesting to mention that HPV is more associated to BD in young, black-skinned individuals, who generally present verruca or hyperkeratotic lesions.¹¹ The patient studied in this paper was a carrier of intestinal disease, though not clinically or histopathologically defined as to whether it was ulcerative rectocolitis or Crohn's disease, or an association of both. While there were no hyperkeratosic lesions, the lesion did appear papullous. Histopathologic and immuno-histochemical tests strongly suggest association with HPV.

Histopathologic study is fundamental for excluding the possibility of invasive carcinomas and also for immuno-histological analysis, the purpose of which is to associate the lesion to some such virus.² The viral association suggests the possibility of multifocal disease and consequent recurrences. The medical procedure follow-up must also include rectosigmoidoscopy and colonoscopy every two to three years, due to the risk of tumors appearing in the intestinal tract.⁵

Various therapies have been proposed for treating BD. The most cited are conventional excision surgery, laser, photodynamic therapy and cryosurgery. In lesions located in the perianal region, the choice of treatment should be made carefully owing to the high risk of complications that may occur. Conventional excision surgery is most suggested in the literature, because it ressects the whole lesion and has a low recurrence rate. On the other hand, in the perianal region, scarring (fibrosis) may produce sphincter incontinence and canal stenosis, and the site plasty must be performed in the same surgical operation.^1,2Considering the multifocal, intra-epithelial BD carcinoma thus, recurrence may occur on the surface or lateral to the area treated, despite a complete in-depth surgical excision. In the case described, surgery was not proposed because a large reconstructive graft would be necessary for functional as well as anatomic reconstruction of the area, and also because the nature of the site would certainly not facilitate a favored result.

Among the modalities employed, laser therapy is not the first choice owing to the low cure rate.¹²

The recently introduced photodynamic therapy, based in the use of photosensitive substances and subsequent laser radiation, has been described for in situ tumors, however it presents side effects, like pain and blister formation, and still does not offer sufficient oncological control.¹³

For this patient, liquid nitrogen cryosurgery was proposed in spray form, an option that benefited the large lesion extension (10 cm in diameter), which allowed treatment in quadrants and made access easier, precisely in the intergluteal fold. Cryosurgery is an easy treatment to carry out. It may be performed in an outpatient's clinic, under local anesthetic and with a low morbidity rate.^8,13,14 There are few otherwise controllable complications, like pain, blister forming and ulcerations.^8,13,14

The freezing of the affected area at predetermined time periods guarantees in-depth treatment, as was performed in this study - two 40-second freezing cycles safely reached the entire thickness of the dermis and epidermis. Lateral treatment was done by freezing a 1-cm safety margin. It is interesting to observe the main cryogenic effect on the tumoral lesion, because the development of blisters and necrosis occurs fundamentally in the tumor. In the safety margin, the clinical and histopathologically normal area, there were no blisters and there was light epidermic peeling. Moreover, the elimination of the tumor necrosis occurred later, starting approximately on the tenth day of post-operation.

Cryosurgery has been described as a method having a low recurrence rate, which shows the possibility of a more accessible retreatment for cutaneous tumors.^8,14 It is important to emphasize that scarring evolves well with this therapy, including skin repigmentation.^8,14 In the case in question, after 34 months of treatment the patient showed excellent scarring with no signs of retraction, with skin repigmentation and, especially, without any signs of recurrence, despite the uncertainty of whether cryosurgery is the best treatment for this disease.

REFERENCES

Received in December, 2nd of 1999.

Approved by the Consultive Council and accepted for publication in April, 17th of 2002.

* Work done at "Dermatology Department of the Faculdade de Ciências Médicas at Universidade Estadual de Campinas - FCM/ UNICAMP".

• 1. Sarmiento JM, Wolff BG, Burgart LJ, Frizelle FA, Ilstrup DM. Perianal Bowen's disease; Associated tumors, Human Papillomavirus, Surgery and others controversies. Dis Colon Rectum 1997;40 (8):912-918.
• 2. Beck DE, Fazio VW, Jagelman DG, Lavery IC. Perianal Bowen´s disease. Dis Colon Rectum 1988;31(6):419-22.
• 3. Balázs M. Bowenoid change in perianal condyloma acuminatum associated with ulcerative colitis. Hepato-Gastroenterol 1991;38:311-3.
• 4. Beck DE, Harford FJ, Roettger RH. Perianal Bowen´s disease associated with Crohn´s colitis; report of a case. Dis Colon Rectum 1989;32(3):252-5.
• 5. Beck DE, Fazio VW. Premalignant lesions of the anal margin. South Med J 1989;82(4):470-4.
• 6. Harris AJ, Purdie K, Leigh IM, Proby C, Burge S. A novel Human Papillomavirus identified in epidermodysplasia verruciformes. Br J Dermatol 1997;136:587-91.
• 7. Sagher U, Krausz MM, Peled IJ. V-Y Plasty for perianal reconstruction after resection of tumor. Surg Gynecol Obstet; 1992;175 (1):31-2.
• 8. Graham GF, Hill C. Advances in cryosurgery during the past decade. Cutis 1993;52:365-72.
• 9. Petrelli NJ, Cebollero, JA, Bigas MR Mang. T. Photodinamic therapy in the management of neoplasms of the perianal skin. Arch. Surg 1992;127:1436-1438.
• 10. Boynton KK, Bjorkman DJ. Argon laser therapy for perianal Bowen's disease: A case report. Lasers Surg Med 1991;11(4): 385-7.
• 11. Papageorgiou PP, Koumarianou AA, Chu AC. Pigmented Bowen's disease. Br J Dermatol 1998;138(3):515-8.
• 12. Marchesa P, Fazio VW, Oliart S, Goldblum JR, Lavery IC. Perianal Bowen's disease: A clinicopathologic study of 47 patients. Dis Colon Rectum 1997;40(11):1286-93.
• 13. Morton CA, Whitehusrt C, Moseley H, Mccoll JH, Moore JV, Mackie RM. Comparison of photodinamic therapy with cryotherapy in the treatment of Bowen's disease. Br J Dermatol 1996; 135:766-771.
• 14. Kuflik EG. Cryosurgery updated. J Am Ac Dermatol 1994;31 (6):925-44.

Publication Dates

• Publication in this collection
  12 May 2006
• Date of issue
  Oct 2002

History

• Received
  02 Dec 1999
• Accepted
  17 Apr 2002