Nelson Syndrome: A Rare Cause of Generalized Hyperpigmentation of the Skin

Barabash, R.; Moreno-Suárez, F.G.; Rodríguez, L.; Molina, A.M.; Conejo-Mir, J.

doi:10.1016/S1578-2190(10)70582-5

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Abstract

Nelson syndrome is a rare cause of generalized mucocutaneous hyperpigmentation. Its clinical manifestations are due to excessive secretion of adrenocorticotropic hormone from a pituitary adenoma, which develops after bilateral therapeutic adrenalectomy. As this operation has fallen into disuse, Nelson syndrome is now extremely rare and difficult to recognize. We present a very severe case of generalized hyperpigmentation due to Nelson syndrome in a 37-year-old woman.

Keywords:

Nelson syndrome

Hyperpigmentation

Adrenalectomy

Resumen

El síndrome de Nelson (SN) supone una causa infrecuente de hiperpigmentación mucocutánea generalizada, cuyas manifestaciones clínicas se derivan de la secreción excesiva de corticotropina por un adenoma hipofisario secundario a la realización de una suprarrenalectomía bilateral terapéutica. Debido a que esta intervención quirúrgica ha caído en desuso en la actualidad, su presentación es hoy sumamente rara y poco reconocible. Presentamos un caso muy grave de hiperpigmentación generalizada por SN en una paciente de 37 años.

Palabras clave:

Síndrome de Nelson

Hiperpigmentación

Suprarrenalectomía

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