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Carcinoma erysipeloides
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Carcinoma erysipeloides

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Contributors: Tyler Hayes MRes, Susan Burgin MD
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Synopsis

Carcinoma erysipeloides (CE; also known as carcinoma erysipelatoides) is a rare form of cutaneous metastasis that presents as an erythematous, well-demarcated patch or plaque that resembles erysipelas. It is most commonly seen unilaterally on the chest wall. It represents lymphatic spread of a primary tumor, usually directly from an affected lymph node, to the cutaneous lymphatics with subsequent lymphatic obstruction. It often appears after chemotherapy, radiotherapy, lymphadenectomy, or tumor excision surgery, all of which are hypothesized to cause shedding of the malignant cells into lymphatics.

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.

Codes

ICD10CM:
C50.919 – Malignant neoplasm of unspecified site of unspecified female breast

SNOMEDCT:
32968003 – Inflammatory carcinoma

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

All may present with fever and leukocytosis, whereas CE does not. If antibiotics are ineffective for an expected infection, CE must be considered.
  • Dermatitis
  • 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.

Best Tests

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Therapy

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References

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Last Updated: 04/23/2019
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Carcinoma erysipeloides
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Carcinoma erysipeloides : Smooth plaque, Unilateral chest
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