Systematized nevus lipomatosus

A 13-year-old female presented with gradually progressive asymptomatic skin-colored to hyperpigmented soft plaques with lobulated surface on the back, neck, and face in Blaschko-linear pattern for the past 5 years [Figure 1a and b]. For the lesions on the back, the clinical differential diagnoses included connective tissue nevus, nevus lipomatosus, and epidermal nevus. For the facial lesions, the differentials considered were connective tissue nevus, nevus lipomatosus, and hemihypertrophy. Histopathology from the lower back lesion revealed mild hyperkeratosis, acanthosis, and aggregates of mature adipocytes interspersed between collagen bundles in the reticular dermis, not connected to the subcutaneous fat [Figure 2], suggestive of nevus lipomatosus cutaneous superficialis. Nevus lipomatosus is a hamartoma of adipocytes that present as painless pedunculated skin-colored to yellowish nodules in a zosteriform distribution with a predilection for the pelvic girdle.^[1] Our case had atypical involvement of the face along with extensive Blaschko-linear involvement akin to the systematized distribution described for epidermal nevi.^[2]

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Figure 1:

(a) Nevus lipomatosus involving the right side of the face as soft, barely elevated skin-colored, ill-defined plaques with sharp midline demarcation, causing facial asymmetry, (b) Nevus lipomatosus involving the right side of the back as hyperpigmented cobblestone plaques, extending to the contralateral side as multiple linear lesions.

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Figure 2:

Histopathology of nevus lipomatosus showing mild hyperkeratosis, papillomatosis, epidermal acanthosis, and the presence of multiple mature adipocytes in reticular dermis with no connection to subcutaneous fat (hematoxylin and eosin, ×40).

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