Pachydermodactyly is a benign, superficial fibromatosis frequently obs
erved in young males, We describe the condition in two young girls aff
ected by Ehlers-Danlos syndrome (EDS) and tuberous sclerosis, respecti
vely, We hypothesized that in the patient with tuberous sclerosis, pac
hydermodactyly is a clinical manifestation of the associated systemic
disease, whereas obsessive-compulsive behavior is probably the main ca
use in the girl with EDS.