ABSTRACT
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.
ABSTRACT
BACKGROUND: Collagen is the most abundant protein in the body and the main structural component of the skin. OBJECTIVE: To provide a review of the histopathology of collagen alterations and to propose a classification with the most important types of collagen anomalies in dermatopathology. The authors describe some of the main morphological clues of collagen anomalies for specific diagnosis of some cutaneous inflammatory and neoplastic conditions. METHODS: The authors review histopathologic collagen anomalies, concerning both morphology and disposition in some inflammatory and neoplastic cutaneous conditions, and they review previous terminology and proposed a classification of the most important types of collagen anomalies that can be seen in dermatopathological practice. RESULTS: Collagen anomalies in skin can be classified into lamellar fibrosis, sclerosis, and "balls" and "rings" of collagen. Lamellar fibrosis presents as long and thin collagen bundles forming a delicate network, which can be disposed in a parallel pattern, onion-bulb-like pattern, and storiform pattern. Sclerosis is characterized by large, thick, and eosinophilic bundles of collagen, which may present as a homogenous-diffuse pattern or as individual thick bundles of collagen with few or abundant number of fibroblasts between them. Finally, the authors propose the terms "balls" and "rings" of collagen. The term "balls" of collagen stands for thick, homogenous, eosinophilic, globular collagen bundles, with no distinguishable individual composing fibers, which include the floating sign and the free-floating sign. The term "rings" of collagen is characterized by sclerotic collagen arranged in a homogenous rimming pattern around vessels without independent fibers in its composition. CONCLUSIONS: Collagen anomalies may be important clues to establish specific clues for specific diagnoses in dermatopathology.