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Se presenta una serie de 6 casos, incluyendo 2 pacientes de una misma familia, se revisa la literatura y se discute su diagnstico diferencial con otras enfermedades locales y sistmicas trascendentes que tambin muestran pigmentaciones maculares cutneas, en labios y mucosa bucal, haciendo nfasis en las caractersticas clnicas como clave para su diagnstico y tratamiento adecuado

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