Atrichia with papular lesions is a rare inherited skin disorder characteriz
ed by congenital atrichia with numerous papules. We describe a 27-year-old
woman with atrichia, who had numerous papules on her scalp, nape, and axill
ae. Histologically, many keratinous cysts were seen in the middermis of a s
kin specimen from the nape. Electron microscopy showed that the developing
keratinocytes in the walls of some cysts were rich in glycogen granules and
had epidermoid keratinization with formation of keratohyaline granules and
that laminated bodies were formed before keratinization. Langerhans cells
were often seen in the walls of the cysts. In addition, a broad glassy vitr
eous layer surrounded the cyst wall. From these findings, it was suggested
that the cystic lesions might have originated from immature or incomplete h
air follicles. In particular, the structure of the cyst wall corresponded w
ell to infundibular and/or isthmal portions of the outer root sheath of the
hair follicle.