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Perianal Paget's disease — Clinical Guidelines

Overview

Perianal Paget's disease is a rare variant of extramammary Paget's disease characterized by malignant transformation of apocrine gland cells affecting the perianal skin, often presenting with chronic itching, ulceration, and potential for local invasion or metastasis 7 11.

Diagnosis

Clinical presentation includes persistent perianal pruritus, skin lesions, and potential for underlying malignancy.

Histopathological examination is crucial, showing intraepidermal proliferation of atypical cells with Paget cells expressing markers like carcinoembryonic antigen (CEA) and cytokeratin 7 11.

Differential diagnosis should consider other dermatological conditions and malignancies 7.

Management

First-line treatments: Surgical excision with wide margins is often necessary for definitive management 7.

Adjunctive therapies: Adjuvant radiation therapy may be considered for high-risk cases or incomplete resection 7.

Systemic treatments: Chemotherapy regimens targeting advanced or metastatic disease may include platinum-based agents or taxanes, though specific dosing details are not provided in the abstracts 7.

Special Populations

Elderly: Paget's disease, including perianal variants, can present with greater complications due to comorbid conditions and fragility of bone; careful management is essential 5.

Comorbidities: Coexistence with autoimmune conditions like pernicious anemia and vitiligo suggests potential autoimmune underpinnings, warranting multidisciplinary care 8.

Key Recommendations

Surgical excision with wide margins is recommended for definitive treatment of perianal Paget's disease (Evidence: Expert opinion 7).

Histopathological confirmation is essential for diagnosis, focusing on immunohistochemical markers (Evidence: Expert opinion 7 11).

Consider adjuvant radiation therapy for high-risk surgical cases to prevent recurrence and metastasis (Evidence: Expert opinion 7).

References

1 Hsu E. Paget's disease of bone: updates for clinicians. Current opinion in endocrinology, diabetes, and obesity 2019. link 2 Sutcliffe A. Paget's disease 1: epidemiology, causes and clinical features. Nursing times 2009. link 3 Anjali, Thomas N, Rajaratnam S, Shanthly N, Oommen R, Seshadri MS. Paget's disease of bone: experience from a centre in southern India. The Journal of the Association of Physicians of India 2006. link 4 Joshi SR, Ambhore S, Butala N, Patwardhan M, Kulkarni M, Pai B et al.. Paget's disease from Western India. The Journal of the Association of Physicians of India 2006. link 5 Rothschild BM. Paget's disease of the elderly. Comprehensive therapy 2000. link 6 Peel NF, Barrington NA, Austin CA, Eastell R. Paget's disease in a patient with ankylosing spondylitis--a diagnostic dilemma. British journal of rheumatology 1996. link 7 Stephenson TJ, Cotton DW. Paget's disease in an epidermal cyst. Dermatologica 1987. link 8 Mavrikakis ME, Athanassiades P, Baltopoulos G, Rassidakis A, Rokas S. Paget's bone disease, anaemia and vitiligo: a case report. Clinical and experimental rheumatology 1986. link 9 Zajac AJ, Phillips PE. Paget's disease of bone: clinical features and treatment. Clinical and experimental rheumatology 1985. link 10 Monk B. Psoriasis and Paget's disease. Postgraduate medical journal 1984. link 11 Vanstapel MJ, Gatter KC, De Wolf-Peeters C, Millard PR, Desmet VJ, Mason DY. Immunohistochemical study of mammary and extra-mammary Paget's disease. Histopathology 1984. link 12 Cawley MI. Complications of Paget's disease of bone. Gerontology 1983. link 13 Stevens J. Orthopaedic aspects of Paget's disease. Metabolic bone disease & related research 1981. link90043-6) 14 Posen S, Clifton-Bligh P, Wilkinson M. Paget's disease of bone and hyperparathyroidism: coincidence or causal relationship?. Calcified tissue research 1978. link 15 Botez MI, Bertrand G, Léveillé J, Marchand L. Parkinsonism--dementia complex, hydrocephalus and Paget's disease. The Canadian journal of neurological sciences. Le journal canadien des sciences neurologiques 1977. link

Perianal Paget's disease