A 3-year-old Japanese girl presented with disseminated white papules on the
trunk. A biopsied specimen of the lesional skin revealed a focal accumulat
ion of elastic fibers in the mid to deep dermis. No skeletal involvement wa
s detected. This case was considered to be elastic nevus without systemic i
nvolvement. Since an elevated elastin expression in the fibroblasts of typi
cal Buschke-Ollendorff syndrome (BOS) has been reported, elastin and elasti
n-related protein mRNA levels in the cultured patient fibroblasts were dete
rmined. There were no significant differences of steady-state levels of ela
stin, fibrillin 1 and microfibril-associated glycoprotein 1 mRNAs between p
atient and matched control fibroblasts. Elastic nevus without skeletal invo
lvement may be etiologically different from typical BOS.