title: [Lymphangioma with epithelial hyperplasia included in a Bardet-Biedl syndrome].
abstract: BACKGROUND
  The Bardet-Biedl syndrome is a rare autosomal recessive disorder, which associates obesity, pigmentary retinopathy, hexadactyly, hypogenitalism, renal dysfunction and mental retardation. Other abnormalities can be observed in the Bardet-Biedl syndrome, but few cutaneous abnormalities have been described.
  
  
  METHODS
  A 41 year-old woman, suffering from a Bardet-Biedl syndrome diagnosed when she was 7 Years old, presented with an atypical pseudo verruca-like, dark red lesion of the interbuttock area that had developed over fifteen Years and had become a handicap. The histological examination revealed a double component: epithelial, papillomatous and acanthosic on the one hand and vascular and lymphatic on the other, suggesting a lymphangioma with epidermal hyperplasia. Magnetic resonance imaging of the sacral area revealed a median subcutaneous lesion, extending deeply to the third coccygial vertebra.
  
  
  CONCLUSIONS
  Such a lymphangioma is unusual. Because it occurred during a rare polymalformative syndrome, we suggest that it may represent a new clinical sign that can be observed during the Bardet-Biedl syndrome.
authors.affiliation: Service de Dermatologie, CHU de Brest.
authors.forename: A
authors.initials: A
authors.lastname: Boulenger-Vazel
authors.forename: J-P
authors.initials: JP
authors.lastname: Leroy
authors.forename: B
authors.initials: B
authors.lastname: Sassolas
authors.forename: I
authors.initials: I
authors.lastname: Kupfer
authors.forename: C
authors.initials: C
authors.lastname: Jacobzone
authors.forename: A-M
authors.initials: AM
authors.lastname: Paule
authors.forename: L
authors.initials: L
authors.lastname: Misery
mesh_terms: D000328:Adult; D020788:Bardet-Biedl Syndrome / Q000150:complications*; D004848:Epithelium / Q000473:pathology; D005260:Female; D006801:Humans; D006965:Hyperplasia; D008202:Lymphangioma / Q000209:etiology* / Q000473:pathology; D008279:Magnetic Resonance Imaging; D012447:Sacrum / Q000473:pathology; D012878:Skin Neoplasms / Q000209:etiology* / Q000473:pathology; D012983:Soft Tissue Neoplasms / Q000209:etiology* / Q000473:pathology