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Nelson syndrome(ROES)

MedGen UID:
342174
Concept ID:
C1852159
Disease or Syndrome
Synonyms: Dermal Ridges; DERMAL RIDGES-OFF-THE-END; Ridges-off-the-end syndrome; ROES
 
Monarch Initiative: MONDO:0016035
OMIM®: 125550
Orphanet: ORPHA199244

Definition

A rare, acquired, endocrine disease characterized by the triad of diffuse skin and mucosa hyperpigmentation, markedly elevated serum adrenocorticotropin (ACTH) levels and an enlarging corticotroph adenoma, which manifest following total bilateral adrenalectomy performed for the treatment of Cushing's disease. Additionally, patients may present with headaches, visual field defects, cranial nerve palsy, pituitary apoplexy, diabetes insipidus, panhypopituitarism, and, occasionally, paraovarian or paratesticular tumors. [from ORDO]

Professional guidelines

PubMed

Castinetti F, Brue T, Ragnarsson O
Eur J Endocrinol 2019 May 1;180(5):D9-D18. doi: 10.1530/EJE-19-0092. PMID: 30970325
Barber TM, Adams E, Wass JA
Handb Clin Neurol 2014;124:327-37. doi: 10.1016/B978-0-444-59602-4.00022-8. PMID: 25248597
Banasiak MJ, Malek AR
Neurosurg Focus 2007;23(3):E13. doi: 10.3171/foc.2007.23.3.15. PMID: 17961028

Recent clinical studies

Etiology

Gheorghiu ML
Best Pract Res Clin Endocrinol Metab 2021 Mar;35(2):101514. Epub 2021 Mar 10 doi: 10.1016/j.beem.2021.101514. PMID: 33814300
Castinetti F, Brue T, Ragnarsson O
Eur J Endocrinol 2019 May 1;180(5):D9-D18. doi: 10.1530/EJE-19-0092. PMID: 30970325
Graffeo CS, Perry A, Carlstrom LP, Meyer FB, Atkinson JLD, Erickson D, Nippoldt TB, Young WF, Pollock BE, Van Gompel JJ
J Neurosurg 2017 Dec;127(6):1277-1287. Epub 2017 Jan 13 doi: 10.3171/2016.9.JNS161163. PMID: 28084914
Palermo NE, Ananthakrishnan S
Curr Opin Endocrinol Diabetes Obes 2015 Aug;22(4):313-8. doi: 10.1097/MED.0000000000000175. PMID: 26087343
Banasiak MJ, Malek AR
Neurosurg Focus 2007;23(3):E13. doi: 10.3171/foc.2007.23.3.15. PMID: 17961028

Diagnosis

Fountas A, Karavitaki N
Medicina (Kaunas) 2022 Nov 2;58(11) doi: 10.3390/medicina58111580. PMID: 36363537Free PMC Article
Fountas A, Karavitaki N
Endocrinol Metab Clin North Am 2020 Sep;49(3):413-432. doi: 10.1016/j.ecl.2020.05.004. PMID: 32741480
Palermo NE, Ananthakrishnan S
Curr Opin Endocrinol Diabetes Obes 2015 Aug;22(4):313-8. doi: 10.1097/MED.0000000000000175. PMID: 26087343
Barber TM, Adams E, Ansorge O, Byrne JV, Karavitaki N, Wass JA
Eur J Endocrinol 2010 Oct;163(4):495-507. Epub 2010 Jul 28 doi: 10.1530/EJE-10-0466. PMID: 20668020
Munir A, Newell-Price J
Arq Bras Endocrinol Metabol 2007 Nov;51(8):1392-6. doi: 10.1590/s0004-27302007000800026. PMID: 18209878

Therapy

Fountas A, Karavitaki N
Medicina (Kaunas) 2022 Nov 2;58(11) doi: 10.3390/medicina58111580. PMID: 36363537Free PMC Article
Patel J, Eloy JA, Liu JK
Neurosurg Focus 2015 Feb;38(2):E14. doi: 10.3171/2014.10.FOCUS14681. PMID: 25639316
Barber TM, Adams E, Wass JA
Handb Clin Neurol 2014;124:327-37. doi: 10.1016/B978-0-444-59602-4.00022-8. PMID: 25248597
Barber TM, Adams E, Ansorge O, Byrne JV, Karavitaki N, Wass JA
Eur J Endocrinol 2010 Oct;163(4):495-507. Epub 2010 Jul 28 doi: 10.1530/EJE-10-0466. PMID: 20668020
Munir A, Newell-Price J
Arq Bras Endocrinol Metabol 2007 Nov;51(8):1392-6. doi: 10.1590/s0004-27302007000800026. PMID: 18209878

Prognosis

Fountas A, Karavitaki N
Endocrinol Metab Clin North Am 2020 Sep;49(3):413-432. doi: 10.1016/j.ecl.2020.05.004. PMID: 32741480
Graffeo CS, Perry A, Carlstrom LP, Meyer FB, Atkinson JLD, Erickson D, Nippoldt TB, Young WF, Pollock BE, Van Gompel JJ
J Neurosurg 2017 Dec;127(6):1277-1287. Epub 2017 Jan 13 doi: 10.3171/2016.9.JNS161163. PMID: 28084914
Palermo NE, Ananthakrishnan S
Curr Opin Endocrinol Diabetes Obes 2015 Aug;22(4):313-8. doi: 10.1097/MED.0000000000000175. PMID: 26087343
Barber TM, Adams E, Wass JA
Handb Clin Neurol 2014;124:327-37. doi: 10.1016/B978-0-444-59602-4.00022-8. PMID: 25248597
Barber TM, Adams E, Ansorge O, Byrne JV, Karavitaki N, Wass JA
Eur J Endocrinol 2010 Oct;163(4):495-507. Epub 2010 Jul 28 doi: 10.1530/EJE-10-0466. PMID: 20668020

Clinical prediction guides

Fountas A, Karavitaki N
Medicina (Kaunas) 2022 Nov 2;58(11) doi: 10.3390/medicina58111580. PMID: 36363537Free PMC Article
Fountas A, Karavitaki N
Endocrinol Metab Clin North Am 2020 Sep;49(3):413-432. doi: 10.1016/j.ecl.2020.05.004. PMID: 32741480
Graffeo CS, Perry A, Carlstrom LP, Meyer FB, Atkinson JLD, Erickson D, Nippoldt TB, Young WF, Pollock BE, Van Gompel JJ
J Neurosurg 2017 Dec;127(6):1277-1287. Epub 2017 Jan 13 doi: 10.3171/2016.9.JNS161163. PMID: 28084914
Barber TM, Adams E, Wass JA
Handb Clin Neurol 2014;124:327-37. doi: 10.1016/B978-0-444-59602-4.00022-8. PMID: 25248597
Barber TM, Adams E, Ansorge O, Byrne JV, Karavitaki N, Wass JA
Eur J Endocrinol 2010 Oct;163(4):495-507. Epub 2010 Jul 28 doi: 10.1530/EJE-10-0466. PMID: 20668020

Recent systematic reviews

Jones K, Reis I, Levy MJ, Potdar N
Int J Gynaecol Obstet 2023 Oct;163(1):89-95. Epub 2023 May 2 doi: 10.1002/ijgo.14791. PMID: 37128819
Papakokkinou E, Piasecka M, Carlsen HK, Chantzichristos D, Olsson DS, Dahlqvist P, Petersson M, Berinder K, Bensing S, Höybye C, Engström BE, Burman P, Follin C, Petranek D, Erfurth EM, Wahlberg J, Ekman B, Åkerman AK, Schwarcz E, Johannsson G, Falhammar H, Ragnarsson O
Pituitary 2021 Oct;24(5):797-809. Epub 2021 May 25 doi: 10.1007/s11102-021-01158-z. PMID: 34036460Free PMC Article
Reincke M, Albani A, Assie G, Bancos I, Brue T, Buchfelder M, Chabre O, Ceccato F, Daniele A, Detomas M, Di Dalmazi G, Elenkova A, Findling J, Grossman AB, Gomez-Sanchez CE, Heaney AP, Honegger J, Karavitaki N, Lacroix A, Laws ER, Losa M, Murakami M, Newell-Price J, Pecori Giraldi F, Pérez-Rivas LG, Pivonello R, Rainey WE, Sbiera S, Schopohl J, Stratakis CA, Theodoropoulou M, van Rossum EFC, Valassi E, Zacharieva S, Rubinstein G, Ritzel K
Eur J Endocrinol 2021 Mar;184(3):P1-P16. doi: 10.1530/EJE-20-1088. PMID: 33444221Free PMC Article

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