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Vol. 101. Issue 1.
Pages 76-80 (January - February 2010)
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Vol. 101. Issue 1.
Pages 76-80 (January - February 2010)
Case report
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Nelson Syndrome: A Rare Cause of Generalized Hyperpigmentation of the Skin
Aspectos prácticos de la quinacrina como tratamiento del lupus eritematoso cutáneo: serie de casos
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R. Barabash
Corresponding author
romanbarabash@hotmail.com

Corresponding author.
, F.G. Moreno-Suárez, L. Rodríguez, A.M. Molina, J. Conejo-Mir
Servicio de Dermatología, Hospitales Universitarios Virgen del Rocío, Sevilla, Spain
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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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References
[1.]
D.H. Nelson, J.W. Meakin, J.B. Dealy, D.D. Matson, K. Emerson, G.W. Thorn.
ACTH-producing tumor of the pituitary gland.
N Engl J Med, 259 (1958), pp. 161-164
[2.]
M. Hornyak, M.H. Weiss, D.H. Nelson, W.T. Couldwell.
Nelson syndrome: Historical perspectives and current concepts.
Neurosurg Focus, 23 (2007), pp. E12
[3.]
S.K. Nagesser, A.P. Van Seters, J. Kievit, J. Hermans, H.M. Krans, C.J. Van de Velde.
Long-term results of total adrenalectomy for Cushing's disease.
World J Surg, 24 (2000), pp. 108-113
[4.]
M.O. Van Aken, A.M. Pereira, G. Van den Berg, J.A. Romijn, J.D. Veldhuis, F. Roelfsema.
Profound amplification of secretory-burst mass and anomalous regularity of ACTH secretory process in patients with Nelson's syndrome compared with Cushing's disease.
Clin Endocrinol (Oxf), 60 (2004), pp. 765-772
[5.]
Y. Chapuis, J. Pitre, F. Conti, B. Abboud, N. Pras-Jude, J.P. Luton.
Role and operative risk of bilateral adrenalectomy in hypercortisolism.
World J Surg, 20 (1996), pp. 775-780
[6.]
C. Tsigos, D.A. Papanicolaou, G.P. Chrousos.
Advances in the diagnosis and treatment of Cushing's syndrome.
Baillieres Clin Endocrinol Metab, 9 (1995), pp. 315-336
[7.]
P.A. Kelly, G. Samandouras, A.B. Grossman, F. Afshar, G.M. Besser, P.J. Jenkins.
Neurosurgical treatment of Nelson's syndrome.
J Clin Endocrinol Metab, 87 (2002), pp. 5465-5469
[8.]
A. Gil-Cárdenas, M.F. Herrera, A. Díaz-Polanco, J.M. Ríos, J.P. Pantoja.
Nelson's syndrome after bilateral adrenalectomy for Cushing's disease.
Surgery, 141 (2007), pp. 147-152
[9.]
R.E. Mains, B.A. Eipper, N. Ling.
Common precursor to corticotropins and endorphins.
Proc Natl Acad Sci USA, 74 (1977), pp. 3014-3018
[10.]
M.A. Pereira, A. Halpern, L.R. Salgado, B.B. Mendonça, M. Nery, B. Liberman, et al.
A study of patients with Nelson's syndrome.
Clin Endocrinol (Oxf), 49 (1998), pp. 533-539
[11.]
C. Höybye, E. Grenbäck, T. Rähn, M. Degerblad, M. Thorén, A.L. Hulting.
Adrenocorticotropic hormone-producing pituitary tumors: 12- to 22-year follow-up after treatment with stereotactic radiosurgery.
Neurosurgery, 49 (2001), pp. 284-292
[12.]
M. Buchfelder, R. Nistor, R. Fahlbusch, W.J. Huk.
The accuracy of CT and MR evaluation of the sella turcica for detection of adrenocorticotropic hormone-secreting adenomas in Cushing disease.
Am J Neuroradiol, 14 (1993), pp. 1183-1190
[13.]
J. Estrada, M. Boronat, M. Mielgo, R. Magallón, I. Millan, S. Díez, et al.
The long-term outcome of pituitary irradiation after unsuccessful transsphenoidal surgery in Cushing's disease.
N Engl J Med, 336 (1997), pp. 172-177
[14.]
A.A. Kasperlik-Zaluska, W. Zgliczynski, W. Jeske, P. Zdunowski.
ACTH responses to somatostatin, valproic acid and dexamethasone in Nelson's syndrome.
Neuro Endocrinol Lett, 26 (2005), pp. 709-712
[15.]
F. Kelestimur, C. Utas, O. Ozbakir, A. Selcuklu, O. Kandemir, N. Ozcan.
The effects of octreotide in a patient with Nelson's syndrome.
Postgrad Med J, 72 (1996), pp. 53-54
[16.]
A.A. Kasperlik-Zaluska, W. Bonicki, W. Jeske, J. Janik, W. Zgliczynski, Z. Czernicki.
Nelson's syndrome—46 years later: Clinical experience with 37 patients.
Zentralbl Neurochir, 67 (2006), pp. 14-20
[17.]
G. Assié, H. Bahurel, J. Coste, S. Silvera, M. Kujas, M.A. Dugué, et al.
Corticotroph tumor progression after adrenalectomy in Cushing's disease: A reappraisal of Nelson's syndrome.
J Clin Endocrinol Metab, 92 (2007), pp. 172-179
[18.]
K.U. Schallreuter, M.R. Pittelkow, J.M. Wood.
Azelaic acid as a competitive inhibitor of thioredoxin reductase in human melanoma cells.
J Invest Dermatol, 88 (1987), pp. 516
[19.]
M. Nazzaro-Porro, S. Passi, G. Zina, A.S. Breathnach.
Ten years’ experience of treating lentigo maligna with topical azelaic acid.
Acta Derm Venereol (Stockh), 143 (1989), pp. 49-57
[20.]
H. Leibl, G. Stingl, H. Pehamberger, H. Korschan, K. Konrad, K. Wolff.
Inhibition of DNA synthesis of melanoma cells by azelaic acid.
J Invest Dermatol, 85 (1985), pp. 417-422
Copyright © 2010. Academia Española de Dermatología y Venereología and Elsevier España, S.L.
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