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Perianal Paget's disease--report of 4 cases
1Department of Colon & Rectal Surgery, Chang Gung Memorial Hospital, Kaohsiung, Taiwan, R.O.C.
Abstract:
Over the past nine years (from 1981 to 1989), four patients with perianal Paget's disease were treated. All were male with an average age of 58.5 years. Clinically, perianal Paget's disease manifests as a slowly enlarging eczematous, and sharply demarcated perianal skin rash that may be oozing or itching. In the characteristic pathology finding, Paget's cells appear as large, rounded signet-ring cells with abundant mucin stain positive cytoplasm in the basal layer of the acanthotic epidermis. All but one, who suffered from primary sweat gland carcinoma, had underlying rectal adenocarcinoma. The first two cases expired soon after a delayed diagnosis of terminal underlying malignancy. Only in the later two cases was there a preoperative suspicion of perianal Paget's disease. There is often a delay in diagnosis due to clinical ignorance. Patients with persisting perianal skin rash should be biopsied frequently. If perianal Paget's disease is diagnosed, the underlying malignancy should be surveyed and managed thoroughly.
Insights
Perianal Paget's disease, a rare skin condition, often indicates underlying rectal cancer. Early biopsy of persistent perianal rashes is crucial for timely diagnosis and management of this malignancy.
Area of Science:
- Dermatology
- Gastroenterology
- Oncology
Background:
- Perianal Paget's disease is a rare intraepithelial malignancy affecting the perianal skin.
- It often presents as a chronic, eczematous rash, frequently misdiagnosed.
- This condition is strongly associated with underlying gastrointestinal malignancies, particularly rectal adenocarcinoma.
Observation:
- A review of four male patients (average age 58.5) treated between 1981-1989 revealed common clinical features.
- Manifestations include slowly enlarging, sharply demarcated, oozing, or itching perianal skin rashes.
- Histopathology shows characteristic large, rounded Paget cells in the epidermis.
Findings:
- Three out of four patients had associated rectal adenocarcinoma; one had sweat gland carcinoma.
- Delayed diagnosis was common, leading to poor outcomes in early cases.
- Preoperative suspicion improved diagnosis in later cases.
Implications:
- Clinical ignorance contributes to diagnostic delays for perianal Paget's disease.
- Frequent biopsies are recommended for persistent perianal skin rashes.
- Prompt diagnosis and thorough management of associated malignancies are essential for patient survival.