Primary Carcinoma en Cuirasse

To the Editor: There are four well-recognized presentations of breast cancer with cutaneous involvement and these include an inflammatory carcinoma, telangiectatic carcinoma, nodular carcinoma and carcinoma en cuirasse. ‘Carcinoma en cuirasse” or ‘Panzerkrebs, first described by Velpeau in 1838, is a marked sclerodermatous changes of the chest wall. It typically occurs as a thick, leathery ‘breast-plate' of cancer invading the cutaneous lymphatics and usually involves large areas of chest wall, abdomen and groin. The two stages described in the development of carcinoma en cuirasse are an early stage of swelling with pitting erythema followed by an advanced stage of thickening and brawny hardness of the skin.1 Carcinoma en cuirasse may present as a primary untreated breast cancer with extensive skin involvement or more often as a local chest wall recurrence following therapy for breast cancer. Primary carcinoma en cuirasse is a rare presentation of breast cancer with only a few reported cases.1,2 We describe a case of primary carcinoma en cuirasse responding remarkably well to hormonal therapy. A 76-year-old white achondroplegic dwarf presented with a pruritic “rash” over the trunk. She was treated by the local physician with antihistaminics, antibiotics, and diuretics with no improvement of her skin condition. The examination of the “rash” revealed an ulcerated areas of 4 × 4-cm over her right breast and multiple discrete and confluent red purple fleshy nodular skin lesions (Figure 1). There was no Iymphadenopathy or hepatosplenomegaly. A biopsy from a nodule from the back showed extensive infiltration of the skin by duct carcinoma consistent with mammary primary with a positive estrogen receptor status. The patient was started on Tamoxifen 20 mg/day, and improvement in the pruritus occurred within a week. By the end of 12 weeks of the Tamoxifen therapy, all her skin lesions had disappeared, and the chest wall ulcer healing complete, leaving a pigmented area of skin marking the extent of the skin involvement (Figure 2). . Primary carcinoma en cuirasse. . Primary carcinoma en cuirasse. Extensive skin lesions in association with primary malignancy is seen with tumors of the breast, stomach, intestine, pancreas, kidney, bladder, lung, esophagus, and, less frequently, with ovary and uterus.3 Primary carcinoma en cuirasse is usually not associated with spread to internal organs, and symptoms tend to occur from local disease causing pruritus, foul smelling discharge, edema, bleeding, and pain.1 Although many measures are available to treat carcinoma en cuirasse, the principle aim is to improve, if possible by simplest means, the quality of life of patients.4 Prostaglandin-like material from the tumor cause the pruritus and pain in carcinoma en cuirasse which responds to prostaglandin synthetase inhibitors.5 Skin graft,6 local irradiation technique, including orthovoltage X-ray beam, tangential 60Cobalt beam and multiple electron beam fields have been used in treating carcinoma en cuirasse.2 The tumor cells confined to dermal lymphatics are thought to cause the extensive reactive fibrosis seen in the disease, thereby preventing chemotherapeutic agents to attain tumoricidal concentrations locally, rendering them ineffective.7 The extensive cutaneous involvement in the disease have lead investigators to try modifications in irradiation for the control of the disease with variable success. Rotational subtotal skin electron beam therapy (SSEBT) is an application of the radiotherapy and has been employed recently for the treatment of carcinoma en cuirasse with promising results. Four patients with recurrent breast cancer of the carcinoma en cuirasse type, two of which resistant to hormonal therapy and the other two unresponsive to chemotherapy, showed good control of their disease following SSEBT.1,2 Carcinoma en cuirasse of the secondary type is the more common than the primary form of presentation and usually occurs after an initial therapy for the breast cancer such as following mastectomy, chemotherapy, or radiotherapy. Fugimoto and Yamagata8 reported a 52-year-old women with radical mastectomy and postoperative radiotherapy several years in the past, who presented to the dermatology clinic for eczema of chest wall which proved to be a cancer en cuirasse. Similarly, a 70-year-old patient with 24-year history of untreated breast cancer had involvement of both breasts and thorax on presentation along with superficial bleeding from large areas of ulcerated tissue.1 In another report,9 all six of the nine patients with carcinoma of the breast with chest wall metastases treated with fibroblast interferon were resistant to the therapy. Our patient is unusual for the extensive dermal infiltration from breast carcinoma at the time of her initial presentation which was adequately controlled by hormonal manipulation with Tamoxifen therapy. Pain was never a feature during the course of the disease, which may account for the delay in the presentation of our patient. We are unable to find any prior report of such a dramatic control of primary carcinoma en cuirasse of breast with Tamoxifen alone. Although this observation needs further confirmation, primary carcinoma en cuirasse of the breast with extensive cutaneous dissemination of the disease may be adequately controlled by relatively simple measures such as hormonal therapy.