Carcinoma erysipeloides as a presenting feature of breast carcinoma: A case report and brief review of literature.

Carcinoma erysipeloides (CE) is an uncommon but distinctive form of cutaneous metastasis that usually manifests clinically as a fixed erythematous patch or plaque resembling cellulitis or erysipelas. The inflamed area may show a distinct raised edge at the periphery and also edema secondary to lymphatic obstruction. Histologically, dense carcinoma cells in the dermal vascular channels and dense perivascular lymphocytic infiltrate may be seen. CE may rarely be the first sign of "silent" tumor of breast. We report a case of a 52-year-old married lady who presented with a three months history of pruritic erythematous patches over the left side of the breast that was resistant to all topical treatments and was diagnosed as erysipeloid breast carcinoma.

INTRODUCTION

Carcinoma erysipeloides (CE) is an uncommon type of cutaneous metastasis in which malignant cells spread to the skin via superficial dermal lymphatic vessels. The diagnosis of underlying breast carcinoma may be delayed in the context of uncommon presentations such as CE. We present a case of carcinoma erysipeloides with underlying breast carcinoma, which was initially being treated as eczematous dermatitis.

CASE REPORT

A 52-year-old married lady presented to our outpatient department with a three months history of erythematous patches over the left side of the breast. The lesion was treated as an eczema by her previous dermatologist, with combinations of moderate to potent topical steroids and topical antifungals, but there was no response with the topical treatment. She had also experienced some weight loss over the last three months.

On examination ill-defined, erythematous macules and plaques were seen over the left breast [Figure 1]. There was no evident oozing, crusting, or scaling. There was no nipple retraction on the involved side. No definite mass was palpable in breast. Left axillary lymph nodes were enlarged. Medical history was negative regarding diabetes mellitus, hypertension, or any other gastrointestinal disturbances. A complete blood count and routine biochemistry analysis showed normal results. Hormonal assay showed low estrogen levels. Luteinizing hormone (LH), follicle stimulating hormone (FSH), and prolactin levels were within normal limits. Ultrasonography and mammography results were not significant. Chest radiography was within normal limits.

Figure 1

Erythematous macules and plaques on the left breast

Fine-needle aspiration cytology (FNAC) was done with the suspicion of inflammatory breast carcinoma. As no definite mass was palpable; the FNAC was done from the each breast quadrant underlying the eczematous area. The FNAC smears were of low cellularity, but showed the presence of few dispersed highly atypical cells having marked nuclear abnormalities compatible with carcinoma cells. Afterward, core needle biopsy from breast was done, which showed occasional intravascular malignant cells in adipose tissue.

Multiple skin punch biopsies were also evaluated from the eczematous areas; all of which showed emboli of carcinoma cells in dermal and subcutaneous blood vessels [Figures 2 to 4]. In addition, dermal extravascular invasion was present focally. Moderate perivascular lympho-mononuclear cell infiltrate was also present around many of such vessels containing tumor emboli [Figure 2]. On immunohistochemistry evaluation, the tumor cells stained with CK7 and CKAE1/AE3 and were negative for estrogen receptors (ER) and progesterone receptors (PR).

Figure 2

Histopathology low power 10× (H and E stain) showing emboli of carcinoma cells in dermal and subcutaneous blood vessels

Figure 4

H and E stain, ×100 showing carcinoma cells in the dermal vascular channel and infiltrating the dermal stromal tissue

Histopathology high power 40× (H and E) stain showing dermal extravascular invasion present focally. Moderate perivascular, lymphomononuclear cell infiltrate is also present around many vessels containing tumor emboli

We referred the patient to a specialized oncology center, where she received sequential treatment with chemotherapy. After that the patient was lost to follow up. We contacted her family later on, and got the news of her death while on chemotherapy treatment.

DISCUSSION

Erysipeloid or inflammatory carcinoma is an uncommon form of cutaneous metastasis. Worldwide, breast carcinoma (BC) comprises 22.9% of all nonmelanoma skin cancers and 16% of all female cancers.[1] Primary breast carcinoma is the most common cause of cutaneous metastases in adult females.[1] CE constitutes about 1% of metastasis from breast cancer and is often considered a marker of tumor recurrence.[2] CE, as the first manifestation of BC, is very rare and accounts for about 2%–5% of all cases.[34]

CE is associated with a poor prognosis and the average life expectancy is 2 years from the time of diagnosis of CE. The most common features of inflammatory breast carcinoma are erythema (51%), and a palpable breast mass (51%). The median duration of onset of inflammatory skin changes before the diagnosis of malignancy is considered to be about 10 weeks.[5] Dissemination usually tends to occur via a direct spread from the affected lymph nodes to the cutaneous lymphatics.[5]

Carcinoma erysipeloides manifests clinically with a fixed erythematous patch or plaque resembling cellulitis or erysipelas, but without fever.[6] The inflamed area may show a distinct raised periphery and edema secondary to lymphatic obstruction.[7] In our case the amount of erythema and induration was probably less due to the previous use of topical steroids.

Malignant cells are predominantly within the dermal lymphatic vessels, with malignant thrombi-induced lymphatic obstructions causing the erysipeloid induration.[8] The pathology of inflammatory carcinoma is distinctive with the deposition of tightly packed malignant cells within the superficial and deep lymphatics without the pathologic evidence of acute inflammation, such as neutrophils, although the lesions look clinically red and inflamed. Dermal lymphatic invasion is considered to be the hallmark of CE.[4] Immunohistochemistry may prove invaluable in establishing the tissue of origin in inflammatory breast carcinoma. Gross cystic disease fluid protein-15 (GCDFP-15) and estrogen receptor protein (ERP) are valuable markers for cutaneous metastatic breast carcinoma, which can be used in combination for better diagnosis.[910]

CE of the breast represents a diagnostic challenge, and delay in diagnosis is common. Erysipelas, cellulitis and dermatitis are the differential diagnoses, which should be considered. Our case also illustrates that CE could be misdiagnosed as dermatitis.

Our case had certain interesting features. CE, resembling a dermatitis was the initial presenting feature of breast carcinoma with no history of a previous mass or nipple discharge. Also, despite the early suspicion of breast carcinoma, normal ultrasonography and mammography reports shed no further light on the diagnosis. Another important point to remember is that the use of topical steroids may attenuate the erythema and induration associated with CE.

CONCLUSION

Our case highlights the need to maintain a high index of suspicion for dermatitis-like lesions over the breast, which are resistant to conventional treatment. Awareness of the distinctive inflammatory manifestations of CE is essential for the dermatologists to establish an early diagnosis for the appropriate treatment and overall survival rate.

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Conflicts of interest

There are no conflicts of interest.