The main differential diagnoses are acrochordon, seborrheic keratosis, intradermal melanocytic nevi, neurofibromas, verrucae and fibroepithelioma of Pinkus.

The most prevalent variety, known as the classical type, is characterized by a number of flesh-colored or yellowish sessile lesions that have a propensity to combine into smooth or cerebriform plaques that are distributed linearly, zosteriformly, or segmentally.

The solitary form, which has been reported on the arms, knees, ears, axillae, nose, calves, clitoris, and scalp, has no known specific distribution.

[10][11] Although the pathophysiology of NLCS is unknown, ectopic adipocytes may arise from pericytes, similar to embryonic lipogenesis, or from precursor cells from the dermal arteries.

[13][11] Acanthosis, basket weave hyperkeratosis, elevated basal pigmentation, and obliteration with focal rete ridge extension are observed in the epidermis.