CE is almost exclusively associated with breast carcinoma (1%-2% of all breast carcinomas), with poorly differentiated ductal carcinomas being most common, and rarely primary tumors of the parotid, tonsils, colon, pancreas, esophagus, stomach, rectum, lung, ovary, uterus, prostate, or bladder, or with melanoma or mesothelioma.
If a primary tumor has been surgically resected, CE is considered a marker of tumor recurrence. Very rarely (2%-5% of all CE cases), it is a presenting sign of underlying carcinoma.
CE usually proceeds clinically with rapid enlargement of the affected area without skin ulceration. As with other cutaneous metastases, the prognosis is extremely poor, with an average life expectancy of 2 years from diagnosis.
C50.919 – Malignant neoplasm of unspecified site of unspecified female breast
32968003 – Inflammatory carcinoma
Differential Diagnosis & Pitfalls
- Radiation dermatitis
- Intralymphatic histiocytosis
- Erythema migrans
- AESOP syndrome
- Carcinoma en cuirasse – Another rare form of cutaneous metastasis. Clinically it presents as an indurated or erythematous, sclerodermoid or keloidal plaque on the breast (in the setting of locally advanced breast cancer) or on the chest wall (following mastectomy for breast cancer). Histologically there are tumor cells in between collagen bundles.
- Angiosarcoma (and other vascular proliferations) that can follow breast irradiation for breast cancer – Vascular-appearing papules from intralymphatic metastases can mimic skin changes in CE.
- Inflammatory breast carcinoma – Cutaneous manifestations of inflammatory breast carcinoma (IBC) may be similar to CE secondary to other primary carcinomas. A diagnosis of IBC is contingent upon meeting all of the following criteria: rapid onset of breast erythema, edema and/or peau d'orange, and/or warm breast, with or without an underlying palpable mass; duration of history no more than 6 months; erythema occupying at least one-third of the breast; and pathologic confirmation of invasive carcinoma. Biopsy may also demonstrate dermal infiltration with tumor cells (as in CE), although this is not necessary for the diagnosis of IBC. Whereas cutaneous manifestations of IBC are considered primary, CE refers to secondary metastases from carcinomas that can include breast carcinomas and cancer primary to other sites in the body. IBC carries a poor prognosis and risk of early recurrence, and patients in whom IBC is suspected should be immediately referred to an oncologist.