[Lymphangioma with epithelial hyperplasia included in a Bardet-Biedl syndrome].
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.
Boulenger-Vazel
A
A
Service de Dermatologie, CHU de Brest.
Leroy
J-P
JP
Sassolas
B
B
Kupfer
I
I
Jacobzone
C
C
Paule
A-M
AM
Misery
L
L
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