Paget's disease extramammary C44.L

Author: Prof. Dr. med. Peter Altmeyer

All authors of this article

Last updated on: 29.10.2020

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Synonym(s)

Adenocarcinoma apocrinocellulare epidermotropicum; Extramammary Paget`s disease; Extramammary Paget's disease; Paget's carcinoma

History
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Crocker, 1889

Definition
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Rare form of Paget' s disease in body regions with apocrine sweat glands, e.g. anogenital region, armpits, navel region.

Manifestation
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Women are affected 3-4 times more frequently than men (Caucasian populations).

In Japan, the extramammary Paget's disease is mainly observed in males.

The average age in larger studies was 70 years (61-78 years).

The mean time from onset of the initial symptoms to diagnosis was 24 months.

Localization
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Anogenital area (most frequent localization); perineum; axillae; groin;

Clinical features
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Typical is a (solitary) sharply defined, 1.0-10.0 cm large plaque with tongue-like extensions, possibly with accentuated edges, flat, often erosive or weeping plaque, caused by the intertriginous localization (axillae, groin region, genitoanal region).

Itching (or slight pain) is a leading symptom (Patrizi A 2017).

Equally typical is a diagnostic misinterpretation of the initial focus as anal eczema, perianal psoriasis or inguinal or axillary tinea.

Histology
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In a mostly hyperplastic and parakeratotic epidermis, in older lesions also growing downwards at the skin appendages, there are mostly single and randomly scattered, large, bright cells with large pleomorphic nuclei (Paget cells). This "Swiss cheese pattern" is diagnostically very characteristic (see also pagetoid). In sections, the cells in the epithelium can also condense into nests. The Paget cells are CEA-positive and also stain with low-molecular cytokeratins. Important: the cells are Melan A and S100 negative.

Diagnosis
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Histological clarification, punch biopsy at the centre of the lesion.

Differential diagnosis
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Tinea corporis, tinea inguinalis, eczema; intertrigo; M. Bowen.

Complication(s)
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In 25% of cases adnexal adenocarcinoma. Conveyance of tumor cells per continuitatem! In 15% carcinoma of internal organs (rectum, bladder, prostate, cervix or urethra). Here a metastatic process must be assumed.

Remark: The high association between Paget's disease and associated carcinomas is not confirmed in other studies.

Therapy
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Excision far into the healthy area with a safety margin of 1-2 cm, since Paget's cells are also observed outside the clinically healthy zone. In case of extensive tumours or tumours in difficult localisation, leave the defect open. Edge cut and deep (step) cut control (microscopically controlled surgery)! Cover the defect with split or full skin.

External therapy
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Alternative to the surgical procedure (these measures are only recommended to very experienced therapists):

  • Local applications of 5-fluorouracil (Efudix®)
  • Local applications of Imiquimod (Aldara®)

Radiation therapy
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Radiotherapy may be recommended as an alternative to the surgical procedure.

Aftercare
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Close-meshed follow-up checks are absolutely necessary for all procedures, especially the non-operative ones.

Literature
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  1. Chiba H et al (2000) Two cases of vulval pigmented extramammary Paget's disease: histochemical and immunohistochemical studies. Br J Dermatol 142: 1190-1194
  2. Crocker HR (1888-1889) Paget's disease affecting the scrotum and penis. Trans Pathol Soc Lond 40: 187-191
  3. Ito T et al (2015) Tumor thickness as a prognostic factor in extramammary Paget's disease
    . J Dermatol 42:269-275
  4. Ladak A et al (2014) Unilateral Pigmented Extramammary Paget's Diseaseof
    the Axilla Associated with a Benign Mole: A Case Study and a Review ofLiterature
    . Korean J Catholic 48:292-296
  5. Luk NM et al (2003) Extramammary Paget's disease: outcome of radiotherapy with curative intent. Clin Exp Dermatol 28: 360-363
  6. Molinie V et al (1993) Paget disease of the vulva. 36 cases. Ann Dermatol Venerol 120: 522-527
  7. Moreno-Arias GA et al (2003) Radiotherapy for in situ extramammary Paget's disease of the vulva. J Dermatologist Treat 14: 119-123
  8. O'Connor WJ et al (2003) Comparison of mohs micrographic surgery and wide excision for extramammary Paget's disease. Dermatol Surgery 29: 723-727
  9. Patrizi A (2017) extramammary Paget's disease. J Dtsch Dermatol Ges. 15: 856-859
  10. Rajendran S et al (2014) Extramammary Paget's disease of the perianal region
    : a 20-year experience. ANZ J Surg 12. doi: 10.1111/ans.12814
  11. Yamamoto O et al (2003) Extramammary Paget's disease with superimposed herpes simplex virus infection: immunohistochemical comparison with cases of the two respective diseases. Br J Dermatol 148: 1258-1262

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Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 29.10.2020