AKT · Endocrine/Breast

Paget's disease of the nipple

Intraepidermal spread of underlying breast carcinoma to the nipple

Overview

An eczema-like change of the NIPPLE caused by malignant ductal carcinoma cells spreading into the nipple epidermis — almost always associated with an underlying in-situ or invasive breast cancer. The trap is treating it as eczema. Unlike eczema, it starts on and destroys the NIPPLE (not the areola) and is unilateral and persistent.

Recognise

Unilateral, persistent, eczema-like (red, scaly, crusted, itchy/burning) change involving the NIPPLE, ± ulceration/bleeding and nipple destruction/retraction
Starts on the nipple and spreads to the areola (eczema does the opposite); may have an underlying palpable lump
Almost always an underlying breast carcinoma (DCIS or invasive)

Red flags

A persistent unilateral nipple 'eczema' not responding to topical steroids → refer (cancer pathway), biopsy
Underlying mass on imaging

Differentials & how to tell them apart

Eczema/dermatitis of the nippleusually BILATERAL, involves the AREOLA more than the nipple, itchy, responds to topical steroids, spares the nipple tip

Nipple adenomabenign nodular nipple lesion

Amelanotic melanomapigmented/atypical lesion — biopsy

Paget's disease of the nipple — eczema-like nipple change
Monica Giovannini et al / CC BY-SA 2.0 — Wikimedia Commons

Investigations

Suspected-cancer referral; mammography/ultrasound; nipple/skin biopsy (Paget cells); assess for the underlying carcinoma.

Management

Biopsy + treat the underlying breast carcinoma (NOT topical steroids)

1Refer any persistent unilateral nipple eczema on a suspected-cancer pathway; mammography/ultrasound and biopsy. Treat the underlying carcinoma.Gate: Do NOT keep treating it as eczema — unilateral, nipple-centred, persistent change is Paget's of the nipple (an underlying cancer) until biopsy proves otherwise; eczema is bilateral, areola-centred and steroid-responsive
2Surgery (mastectomy or central excision) ± sentinel node and adjuvant therapy according to the underlying tumour.

Treat the underlying breast cancer — surgery (often mastectomy or central excision) ± adjuvant therapy per the underlying tumour

Key points

Persistent unilateral nipple 'eczema' that starts on the NIPPLE and destroys it = Paget's of the nipple (underlying cancer). Eczema is bilateral, areolar and steroid-responsive. Biopsy, don't keep steroiding.

Monitor & prognosis

As for the underlying breast cancer.

Depends on the underlying carcinoma.

Source: NICE NG101