Metaplastic synovial cyst is a recently recognized entity characterize
d by a cystic space lined by polygonal cells and villous structures re
sembling synovium pointing toward the lumen. It usually develops follo
wing trauma. We report a 15-year-old girl with Ehlers-Danlos syndrome
who developed a nodular lesion of her elbow without previous trauma. H
istopathological and immunohistochemical studies yielded results simil
ar to those reported in cases of cutaneous metaplastic synovial cyst.
This case appears to be the first one reported of cutaneous metaplasti
c synovial cyst associated with an Ehlers-Danlos syndrome. Cutaneous f
ragility and anomalous scarring typical of Ehlers-Danlos syndrome may
be related to the development of this pseudocyst. If so, other connect
ive-tissue diseases with increased skin fragility could also be suscep
tible to development of such lesions.