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Cutaneous lesions associated to multiple endocrine neoplasia syndrome type 1.
J Eur Acad Dermatol Venereol. 2008 Jul; 22(7):835-8.JE

Abstract

BACKGROUND

Multiple endocrine neoplasia type 1 (MEN1) is a genetic disease that predisposes to endocrine tumour development. Some cutaneous lesions (angiofibromas, collagenomas, melanosis guttaca, lipomas, melanomas, 'cafe au lait macules') have been associated to this syndrome. We compare the prevalence of cutaneous lesion in affected patients with their non-carrier relatives.

PATIENTS AND METHOD

We studied 9 patients with MEN1 and 20 non-carrier, first-degree relatives. Genetic screening was realized in all of them. Patients were examined by dermatologist, and biopsy was performed when necessary.

RESULTS

Patients with MEN1 presented hyperparathyroidism (100%), neuroendocrine tumours of pancreas (66%) and pituitary adenomas (44%); their relatives were free of endocrine features of MEN1. The studied cutaneous lesions were more prevalent in affected patients than in non-carriers (55.5% vs. 25%; P = 0.029). Odds ratio of developing cutaneous lesions in MEN1 patients was 6.6 (95% confidence interval, 1.09-40.43). The frequency of angiofibromas was lower (22.2%) than the reported in other studies (43-88%), and we did not find any collagenoma.

CONCLUSIONS

MEN1 is associated to some cutaneous lesions and could be useful for detecting MEN1 carriers in an affected family. Cutaneous lesions should be assessed in MEN1 patients.

Authors+Show Affiliations

Servicio de Endocrinología y Nutrición, Hospital Juan Canalejo, A Coruña, Spain. avcyo@mixmail.comNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

18435740

Citation

Vidal, A, et al. "Cutaneous Lesions Associated to Multiple Endocrine Neoplasia Syndrome Type 1." Journal of the European Academy of Dermatology and Venereology : JEADV, vol. 22, no. 7, 2008, pp. 835-8.
Vidal A, Iglesias MJ, Fernández B, et al. Cutaneous lesions associated to multiple endocrine neoplasia syndrome type 1. J Eur Acad Dermatol Venereol. 2008;22(7):835-8.
Vidal, A., Iglesias, M. J., Fernández, B., Fonseca, E., & Cordido, F. (2008). Cutaneous lesions associated to multiple endocrine neoplasia syndrome type 1. Journal of the European Academy of Dermatology and Venereology : JEADV, 22(7), 835-8. https://doi.org/10.1111/j.1468-3083.2008.02578.x
Vidal A, et al. Cutaneous Lesions Associated to Multiple Endocrine Neoplasia Syndrome Type 1. J Eur Acad Dermatol Venereol. 2008;22(7):835-8. PubMed PMID: 18435740.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Cutaneous lesions associated to multiple endocrine neoplasia syndrome type 1. AU - Vidal,A, AU - Iglesias,M J, AU - Fernández,B, AU - Fonseca,E, AU - Cordido,F, Y1 - 2008/04/24/ PY - 2008/4/26/pubmed PY - 2008/8/30/medline PY - 2008/4/26/entrez SP - 835 EP - 8 JF - Journal of the European Academy of Dermatology and Venereology : JEADV JO - J Eur Acad Dermatol Venereol VL - 22 IS - 7 N2 - BACKGROUND: Multiple endocrine neoplasia type 1 (MEN1) is a genetic disease that predisposes to endocrine tumour development. Some cutaneous lesions (angiofibromas, collagenomas, melanosis guttaca, lipomas, melanomas, 'cafe au lait macules') have been associated to this syndrome. We compare the prevalence of cutaneous lesion in affected patients with their non-carrier relatives. PATIENTS AND METHOD: We studied 9 patients with MEN1 and 20 non-carrier, first-degree relatives. Genetic screening was realized in all of them. Patients were examined by dermatologist, and biopsy was performed when necessary. RESULTS: Patients with MEN1 presented hyperparathyroidism (100%), neuroendocrine tumours of pancreas (66%) and pituitary adenomas (44%); their relatives were free of endocrine features of MEN1. The studied cutaneous lesions were more prevalent in affected patients than in non-carriers (55.5% vs. 25%; P = 0.029). Odds ratio of developing cutaneous lesions in MEN1 patients was 6.6 (95% confidence interval, 1.09-40.43). The frequency of angiofibromas was lower (22.2%) than the reported in other studies (43-88%), and we did not find any collagenoma. CONCLUSIONS: MEN1 is associated to some cutaneous lesions and could be useful for detecting MEN1 carriers in an affected family. Cutaneous lesions should be assessed in MEN1 patients. SN - 1468-3083 UR - https://www.unboundmedicine.com/medline/citation/18435740/Cutaneous_lesions_associated_to_multiple_endocrine_neoplasia_syndrome_type_1_ L2 - https://doi.org/10.1111/j.1468-3083.2008.02578.x DB - PRIME DP - Unbound Medicine ER -