Carcinoma en cuirasse of the breast

ARTICLE

Auteur(s) : Marta Carlesimo¹, Alfredo Rossi², Gabriella De Marco¹, Alessandra Narcisi¹, Claudia Cacchi³, Elena Mari¹, Flavia Persechino¹, Germana Camplone¹

¹UOC Dermatology, II Unit University of Rome “Sapienza” Via di Grottarossa, 1039, 00189 Rome, Italy
²Department of Dermatology, I Unit University of Rome “Sapienza”
³Histopathology, II Unit University of Rome “Sapienza”

Carcinoma en cuirasse is a rare form of metastatic cutaneous carcinoma with a large skin involvement, occurring most commonly as a recurrence of breast cancer, but there are some reports of primary presentation of this malignancy [1, 2]. We report a rare case of an anaplastic lobular breast carcinoma, with an impressive primary cutaneous presentation involving skin of the emitorax, scapulo-omeral region and neck.

In April 2007, a 53-year-old woman presented a 30-day history of an erythematous-papular eruption located on the right side of the neck, without itching or pain. She denied any dermatological diseases and the personal history was unremarkable. On physical examination shiny erythematous papules and sclerotic thick plaques were noted on the right side of the neck, which progressively extended to the right arm (figures 1A, B). In May 2007 the patient was admitted to our Department. General and breast examinations were normal: the nipple, from which there was no discharge, was neither depressed nor retracted and could not be easily invaginated. Lymphoadenopathy in the right armpit and lymphoedema of the homolateral arm were present. Laboratory examinations were within normal limits, except for ESR which was 84 mm/h (0-20). Two punch biopsies of the cutaneous lesions showed an extensive dermal infiltration by anaplastic carcinoma, with epidermotropism and lobular morphology, suggesting a mammary origin (figures 1C, D).

Breast echography and mammography showed no focal lesions, but microscopic examination of a tissue biopsy from the right breast confirmed the presence of anaplastic lobular carcinoma. Immunohistochemical staining of the skin lesion revealed positivity for estrogen and progesterone receptors.

The clinical presentation, histological examination and other examinations led to the diagnosis of a lobular infiltrate carcinoma with epidermotropism. The patient was immediately subjected to chemotherapy with farmorubicin in June 2007, and she died after 30 days.

Breast carcinoma is the most common carcinoma in women, and about 25% of patients develop skin metastases [1]. In the literature there are eight distinct clinicopathological types of cutaneous involvement, including en cuirasse (sclerodermoid), inflammatory (carcinoma erysipelatoides) and teleangectatic metastatic breast carcinoma (carcinoma teleangectaticum) [1].

Carcinoma en cuirasse was first described by Velpeau in 1838, a description chosen because of its resemblance to the metal breastplate of a cuirassier [1]. It has also been called scirrhous carcinoma, pachydermia and “Acarcine eburnee” by various authors [1-4]. In 1886, Hutchinson described carcinoma erysipelatoides, (inflammatory metastatic carcinoma), to identify a clinical picture of red indurated skin with sharp borders in association with skin metastatis [3]. Histologically, this carcinoma shows infiltration of malignant cells between the lymphatics without inflammation [3]. Carcinoma teleangectaticum shows a similar pattern, but with aggregates of both erythrocytes and tumor cells and many dilated blood vessels in the papillary dermis [5].

Though rare, carcinoma en cuirasse has been associated with other adenocarcinomas, such as those of the lung, gastrointestinal tract and kidney [1, 2]. It is characterized by a diffuse morphea-like induration of the skin and begins as scattered, lenticular papulo-nodules, overlying erythematous or red-blue smooth cutaneous surfaces. These papulo-nodules coalesce into a sclerodermoid plaque with no associated inflammatory changes [1]. Histologically, fibrosis is the predominant feature, with some tumour cells exhibiting an “Indian file” pattern, where the tumor cells form small lines between collagen bundles [3].

To our knowledge, this is the first report in which cutaneous lesions represent the first clinical manifestation of breast carcinoma and we want to underline how the skin should be considered evidence of internal diseases. Moreover, we report this case for the impressive skin involvement, which should be correlated to the aggressive findings of this anaplastic tumour. The patient died only two months after our first observation. Clinical and histopathological features were typical of both carcinoma en cuirasse and carcinoma teleangectaticum.

Acknowledgements

Références

2 Mullinax K, Cohen JB. Carcinoma en cuirasse presenting as keloids of the chest. Dermatol Surg 2004; 30: 226-8.

3 Nambi MDR. Carcinoma erysipeloides as a presenting feature of breast carcinoma. Int J Dermatol 1999; 38: 367-8.

4 Siddiqui MA, Zaman MN. Primary carcinoma en cuirasse. J Am Geriatr Soc 1996; 44: 221-2.

5 Schwartz RA. Histopatological aspects of cutaneous metastatic disease. J Am Acad Dermatol 1995; 33: 649-57.

* All the authors contributed equally to this paper.