Birt-Hogg-Dube-syndrome, first described 20 years ago, is an autosomal domi
nant inherited disease, characterized by the occurrence of multiple cutaneo
us hamartomas with adnexal differentiation. An association with intestinal
neoplasms has been mentioned repeatedly. Recently, the cutaneous lesions in
this syndrome have been interpreted as different developmental stages of o
ne single hamartoma with sebaceous differentiation, called mantleoma. Numer
ous dermatohistopathologic studies contribute to the certain diagnosis of B
irt-Hogg-Dube-syndrome. In contrast, little information is available on the
therapy of the multiple skin lesions. A father and his daughter with Birt-
Hogg-Dube-syndrome were treated with the CO2 laser, producing satisfactory
results.