Enfermedad de Paget pigmentada de la mama simuladora de melanoma cutáneo / Pigmented mammary Paget's disease mimicking cutaneous melanoma

Resumen ANTECEDENTES: La enfermedad de Paget pigmentada de la mama es una variante poco frecuente de este padecimiento cutáneo, una dermatosis que afecta al complejo areola-pezón de manera típicamente unilateral. La importancia del reconocimiento de esta dermatosis pigmentada inespecífica es que forma parte del diagnóstico diferencial del melanoma cutáneo, por manifestarse como una mácula pigmentada irregular, cuyo estudio histopatológico muestra una proliferación de melanocitos en los estratos suprabasales de la epidermis, fagocitosis de melanina y melanófagos en dermis, hallazgos muy similares al melanoma cutáneo, además de la coexistencia de células claras malignas, características de la enfermedad de Paget. La inmunohistoquímica es una técnica auxiliar en la dermatopatología, que forma parte del proceso diagnóstico de los pacientes para lograr el diagnóstico certero que, al correlacionar la clínica y el estudio histopatológico, le permite al dermatólogo tratar a los pacientes con enfermedad de Paget pigmentada de la mama junto con un equipo multidisciplinario para la búsqueda, diagnóstico y, en su caso, tratamiento quirúrgico de las neoplasias subyacentes que suelen relacionarse con esta variante pigmentada. CASO CLÍNICO: Paciente de 35 años con una lesión pigmentada que afectaba el complejo areola-pezón derecho. Con la correlación histopatológica e inmunohistoquímica se estableció el diagnóstico de enfermedad de Paget pigmentada de la mama y posterior al procedimiento ginecológico, se asoció esta dermatosis con un adenocarcinoma microinvasor triple negativo. CONCLUSIONES: La enfermedad de Paget pigmentada de la mama es un diagnóstico complejo que requiere correlación clínico-patológica y estudios de extensión para valorar su asociación con neoplasias subyacentes.

Abstract BACKGROUND: Pigmented Mammary Paget's Disease is a rare variant of this skin condition, an unilateral dermatosis that typically affects the areola-nipple complex. The importance of recognizing this nonspecific pigmented dermatosis resides in its differential diagnosis of cutaneous melanoma, as it presents as an irregular pigmented macula. The histopathological study shows proliferation of melanocytes in the suprabasal layers of epidermis, phagocytosis of melanin and melanophages in dermis. These findings are very similar to cutaneous melanoma, in addition to the presence of malignant clear epitelial cells that are characteristic of Paget's disease. Immunohistochemistry is part of the approach of patients with lesions that shows proliferating melanocytes to rule out other neoplasms. Making an accurate diagnosis by correlating the clinical, histopathological study and immunohistochemistry allows the dermatologist to approach patients with mammary pigmented mammary Paget's disease with a multidisciplinary team for the diagnosis and surgical treatment of the underlying neoplasms that are usually related to this pigmented variant. CASE REPORT: We report the case of a 35 years old woman with a pigmented lesion that affected the right areola-nipple complex. With the histopathological and immunohistochemical correlation, the diagnosis of pigmented mammary Paget's disease was made and after the gynecological approach, this dermatosis was associated with a triple negative microinvasive adenocarcinoma. CONCLUSION: Pigmented mammary Paget's disease is a complicated diagnosis that requires clinicopathological correlation and extension studies to assess its association to underlying neoplasms.

Pigmented Mammary Paget Disease with Reticulated Features: A Rare Variant of Mammary Paget Disease

Pigmented mammary Paget disease (PMPD) is a rare subtype of mammary Paget disease that presents as a hyperpigmented patch or plaque over the areola and nipple. We herein report on an unusual case of PMPD with reticulated features in a 98-year-old female. The histology showed intraepidermal pagetoid cells containing melanin pigments without any underlying intraductal carcinoma.

Pigmented Paget's Disease: A Rare Clinical Manifestation of Recurred Breast Carcinoma / 대한피부과학회지

Pigmented mammary Paget's disease is a rare variant of mammary Paget's disease, and this mimics malignant melanoma both clinically and histopathologically. Two women aged 48 and 44 years old, respectively, presented with 2-cm pigmented patches and 1-cm pigmented erosive crusted papules without discharge or palpable masses, respectively. They had previously undergone breast-conserving surgery following the diagnosis of intraductal carcinoma of the breast. Physical examinations and imaging studies revealed no evidence of the recurrence of breast cancer. However, histopathological and immunohistochemical analyses demonstrated the presence of 1) Paget's cells in the epidermis and these cells contained melanin within their cytoplasm and 2) numerous melanophages in the upper dermis. We diagnosed the two patients with pigmented Paget's disease. Subsequent pathological analyses revealed recurrent intraductal carcinoma. We recommend that skin biopsies should be taken from patients presenting with pigmented lesions of the nipple, who have previously undergone breast-conserving treatment, to test the possibility of cancer recurrence.

A Case of Pigmented Mammary Paget's Disease

Pigmented mammary Paget's disease is a uncommon clinicopathologic variant of mammary Paget's disease, and this mimics malignant melanoma both clinically and histopathologically. Herein, we report on a rare case of pigmented mammary Paget's disease. An 81-year-old woman presented with 2.5x1 cm sized, red and brown, eczematous plaque on her right areola, and she'd had this lesion for 3 years. Histopathology showed large, atypical cells with large nuclei and abundant pale cytoplasm throughout the epidermis. Dispersed melanocytes were noted in the epidermis and some of the Paget's cells contained melanin within their cytoplasm. Immunohistochemical studies demonstrated that the intraepidermal pagetoid cells were positive for cytokeratin 7; in contrast, they were negative for S-100, Periodic-acid Schiff (PAS), Alcian blue at PH 2.5, HMB-45 and carninoembryonic antigen (CEA). We recommend that pigmented mammary Paget's disease should be included in the differential diagnosis of pigmented lesions on the nipple.