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.