Nevus Lipomatosus Cutaneous Superficialis With Perifollicular Fibromas

Nevus lipomatosus cutaneous superficialis (NLCS) is a rare cutaneous hamartoma characterized by groups of mature fat cells in the dermis. The authors report a case of a dark-skinnned, 6-year-old, Brazilian girl with 1 lesion in the right upper chest, extending to the neck, since 1 year of age. The dermatological examination consisted of skin-colored and yellowish follicular papules, isolated and grouped into a papillomatous plaque. The biopsy revealed ectopic mature adipose tissue in the dermis, compatible with NLCS, perifollicular well-circumscribed fibroblast and collagen proliferation, and decreased elastic fibers in the papillary and upper reticular dermis. The finding of perifollicular fibrosis in NLCS is not common, having been first described in 2003. The perifollicular fibrosis is characteristic of other rare hamartoma, the perifollicular fibroma, but is also present in fibrofolliculomas or trichodiscomas, angiofibromas, and fibrotic papules on the face of tuberous sclerosis and postinflammatory fibrosis. This case is very similar to the first described case of NLCS with perifollicular fibrosis, and the authors believe that this is the second case of this entity, but they propose to name it NCLS with perifollicular fibromas, which better describes the nature of this lesion.