• congenital smooth muscle hamartoma

Congenital smooth muscle hamartoma (CSMH) with follicular spotted appearance is a rare clinical variant of CMSH in which patients have marked perifollicular papules in the patches. A linear distribution of CSMH is also extremely rare. We report a 16-year-old Korean girl with this uncommon form of CSMH who had linearly arranged, hyperpigmented lesions with follicular papules extending from the right flank to the right lower leg from birth. Pathological findings, including immunohistochemical stains, were consistent with smooth muscle hamartoma. To date, there are only four reports on this rare, follicular form and one report on the linear form of CSMH in the literature. This paper describes the first combined occurrence of follicular spotted lesions and linear arrangement in CSMH.