Clinically, NLS is usually present at birth but may develop during the first 3 decades of life. Two subtypes, a solitary form and a classic or multiple form, have been reported since this entity was first described.1,2 The lesions in the classic form are usually flesh-colored or yellow, smooth-surfaced papules or nodules, with femoral or gluteal location and in a zonal or segmental arrangement.2,3
NLS has been found to have various clinical features, including a cerebriform shape,4,5 peau d'orange surface, and hairiness.2,3 In addition, NLS has been associated with other types of skin lesion, including folliculosebaceous cystic hamartoma, dermoid cyst,6 and connective tissue nevus.7 To our knowledge, there has been only one previous report of NLS presenting with dilated hair follicles or comedo-like plugs.8 A case of NLS with a folliculosebaceous cystic hamartoma showing cystically dilated hair follicles has been reported.9 However, there was no hyperplasia of the sebaceous gland in presenting cases. Although the exact mechanism underlying the formation of a dilated hair follicle in patients with NLS is not known, follicular keratinocyte may be a part of hamartomatous nevoid skin anomalies in patients with NLS. Dilated hair follicle may represent an epithelial component in NLS.
NLS with dilated hair follicles should be differentiated from nevus comedonicus, a rare developmental abnormality of the pilosebaceous apparatus, which presents as plaques consisting of aggregated dilated follicular orifices in a linear pattern.10 Differentiation between these 2 entities is important because nevus comedonicus can occur in combination with other systemic abnormalities, such as skeletal and neurological disorders.11 Skin biopsy is important in the differentiation between NLS and nevus comedonicus. In contrast to NLS, nevus comedonicus does not present with ectopic adipose tissue in the dermis. However, the proportion of dermal fat in NLS has been found to vary from less than 10% to more than 50% of the dermis.12 NLS with dilated hair follicles and little ectopic adipose tissue may be confused with nevus comedonicus.
In conclusion, we have described 2 patients with unique features of NLS, dilated hair follicles. An exact diagnosis with histopathology is important because NLS has a broad variety in the clinical feature.
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