PMID- 9236523
OWN - NLM
STAT- MEDLINE
DCOM- 19970828
LR  - 20080317
IS  - 0003-987X (Print)
IS  - 0003-987X (Linking)
VI  - 133
IP  - 7
DP  - 1997 Jul
TI  - Multiple facial angiofibromas and collagenomas in patients with multiple 
      endocrine neoplasia type 1.
PG  - 853-7
AB  - OBJECTIVES: To evaluate patients with multiple endocrine neoplasia type 1 (MEN 1) 
      for cutaneous manifestations. DESIGN: Survey during a 3-year period. SETTING: The 
      National Institutes of Health, a tertiary referral research hospital in Bethesda 
      Md. PATIENTS: A consecutive sample of 32 individuals with previously diagnosed 
      MEN1 who were not preselected for the presence of skin lesions were examined for 
      cutaneous abnormalities. None of the patients or family members were diagnosed as 
      having tuberous sclerosis. INTERVENTIONS: Lesions were identified by clinical 
      appearance, photographed, and confirmed histologically. MAIN OUTCOME MEASURE: To 
      determine the frequency of skin lesions in patients with MEN1. RESULTS: Multiple 
      facial angiofibromas were observed in 28 (88%) of the patients with MEN1, with 16 
      patients (50%) having 5 or more. Angiofibromas were clinically and histologically 
      identical to those in individuals with tuberous sclerosis. Collagenomas were 
      observed in 23 patients (72%). Also observed were cafe au lait macules in 12 
      patients (38%), lipomas in 11 patients (34%), confetti-like hypopigmented macules 
      in 2 patients (6%), and multiple gingival papules in 2 patients (6%). 
      CONCLUSIONS: Multiple angiofibromas, collagenomas, lipomas, confetti-like 
      hypopigmented macules and multiple gingival papules are cutaneous manifestations 
      of MEN1 and should be looked for in both family members of patients with MEN1 and 
      individuals with hyperparathyroidism of other MEN1-associated tumors. Multiple 
      angiofibromas can no longer be considered pathognomonic for tuberous sclerosis. 
      The observation of angiofibromas in individuals without tuberous sclerosis 
      necessitates further biochemical testing for MEN1.
FAU - Darling, T N
AU  - Darling TN
AD  - Dermatology Branch, National Cancer Institute, National Institutes of Health 
      Bethesda, MD, USA.
FAU - Skarulis, M C
AU  - Skarulis MC
FAU - Steinberg, S M
AU  - Steinberg SM
FAU - Marx, S J
AU  - Marx SJ
FAU - Spiegel, A M
AU  - Spiegel AM
FAU - Turner, M
AU  - Turner M
LA  - eng
PT  - Journal Article
PT  - Research Support, U.S. Gov't, P.H.S.
PL  - United States
TA  - Arch Dermatol
JT  - Archives of dermatology
JID - 0372433
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Age Factors
MH  - Aged
MH  - Angiofibroma/genetics/*pathology
MH  - Cafe-au-Lait Spots/pathology
MH  - Collagen Diseases/genetics/*pathology
MH  - Facial Dermatoses/genetics/*pathology
MH  - Facial Neoplasms/genetics/*pathology
MH  - Female
MH  - Gingival Diseases/pathology
MH  - Hamartoma/genetics/*pathology
MH  - Humans
MH  - Hyperparathyroidism/genetics/pathology
MH  - Hypopigmentation/pathology
MH  - Lipoma/pathology
MH  - Male
MH  - Middle Aged
MH  - Multiple Endocrine Neoplasia Type 1/genetics/*pathology
MH  - Neoplasms, Multiple Primary/genetics/*pathology
MH  - Skin Neoplasms/genetics/*pathology
MH  - Tuberous Sclerosis/pathology
EDAT- 1997/07/01 00:00
MHDA- 1997/07/01 00:01
CRDT- 1997/07/01 00:00
PHST- 1997/07/01 00:00 [pubmed]
PHST- 1997/07/01 00:01 [medline]
PHST- 1997/07/01 00:00 [entrez]
PST - ppublish
SO  - Arch Dermatol. 1997 Jul;133(7):853-7.