PMID- 18435740
OWN - NLM
STAT- MEDLINE
DCOM- 20080828
LR  - 20091119
IS  - 1468-3083 (Electronic)
IS  - 0926-9959 (Linking)
VI  - 22
IP  - 7
DP  - 2008 Jul
TI  - Cutaneous lesions associated to multiple endocrine neoplasia syndrome type 1.
PG  - 835-8
LID - 10.1111/j.1468-3083.2008.02578.x [doi]
AB  - 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.
FAU - Vidal, A
AU  - Vidal A
AD  - Servicio de Endocrinologia y Nutricion, Hospital Juan Canalejo, A Coruna, Spain. 
      avcyo@mixmail.com
FAU - Iglesias, M J
AU  - Iglesias MJ
FAU - Fernandez, B
AU  - Fernandez B
FAU - Fonseca, E
AU  - Fonseca E
FAU - Cordido, F
AU  - Cordido F
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
DEP - 20080424
PL  - England
TA  - J Eur Acad Dermatol Venereol
JT  - Journal of the European Academy of Dermatology and Venereology : JEADV
JID - 9216037
RN  - 0 (MEN1 protein, human)
RN  - 0 (Proto-Oncogene Proteins)
SB  - IM
MH  - Adenoma/epidemiology/genetics
MH  - Adult
MH  - Angiofibroma/*epidemiology/genetics
MH  - Female
MH  - Genetic Predisposition to Disease/epidemiology
MH  - Genetic Testing
MH  - Humans
MH  - Hyperparathyroidism/epidemiology/genetics
MH  - Lipoma/*epidemiology/genetics
MH  - Male
MH  - Middle Aged
MH  - Multiple Endocrine Neoplasia Type 1/*epidemiology/genetics
MH  - Pancreatic Neoplasms/epidemiology/genetics
MH  - Pituitary Neoplasms/epidemiology/genetics
MH  - Prevalence
MH  - Proto-Oncogene Proteins/*genetics
MH  - Skin Neoplasms/*epidemiology/genetics
EDAT- 2008/04/26 09:00
MHDA- 2008/08/30 09:00
CRDT- 2008/04/26 09:00
PHST- 2008/04/26 09:00 [pubmed]
PHST- 2008/08/30 09:00 [medline]
PHST- 2008/04/26 09:00 [entrez]
AID - JDV2578 [pii]
AID - 10.1111/j.1468-3083.2008.02578.x [doi]
PST - ppublish
SO  - J Eur Acad Dermatol Venereol. 2008 Jul;22(7):835-8. doi: 
      10.1111/j.1468-3083.2008.02578.x. Epub 2008 Apr 24.