Nevus lipomatosus superficialis (NLS or NLCS, also known as nevus lipomatosis of Hoffman and Zurhelle[1]) is characterized by soft, yellowish
papules or cerebriform
plaques, usually of the buttock or thigh, less often of the ear or scalp, with a wrinkled rather than
warty surface.[1][2]: 625 It is usually congenital
in origin or appears within the first three decades.[3]
A pedunculated lipofibroma is a solitary variant of nevus lipomatosus superficialis. It usually appears in adult life, and usually on the axilla, knee, ear, arm, scalp and the lower trunk.[3]
Clinically, there are two variations. 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. Lessons tend to focus on the lower trunk, particularly the gluteal, sacrum, and lumbar regions as well as the
pelvic girdle.[4][5]
The second clinical pattern of NLCS is a solitary
papule or
nodule that typically appears later in life. It mimics a
skin tag in appearance and is flesh-colored and domed. The solitary form, which has been reported on the arms, knees, ears,
axillae, nose, calves,
clitoris, and scalp, has no known specific distribution.[5][6][7]
The lesions are asymptomatic in both forms.[8] In rare cases,
ulceration happens, particularly following
ischemia or external damage.[9] Moreover, coexisting
comedo-like changes, leukodermic patches,
café-au-lait macules, and overlaying
hypertrichosis are possible.[10][11]
Causes
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.[12][13]
Diagnosis
The histology of NLCS typically demonstrates the proliferation of ectopic mature
adipocytes in the reticular
dermis, which ranges from 10 to 50% of the lesion.[13][14]Adipocytes can exist alone or in small groups between
collagen bundles, but they most frequently originate surrounding blood arteries or
eccrine glands.[9][15] In certain instances, there is a perivascular infiltration of spindle-shaped and mononuclear cells, as well as an increased density of
collagen fibers and
fibroblasts.[13][11]Acanthosis, basket weave
hyperkeratosis, elevated basal pigmentation, and obliteration with focal rete ridge extension are observed in the
epidermis.[8] Adnexal structures may exhibit perifollicular
fibrosis and be unaffected or diminished in certain instances.[13]
Given the rarity of
malignant degeneration and systemic problems, treatment is only recommended for cosmetic reasons.[10] The best course of treatment is surgical excision because recurrence lesions are uncommon.[11]
^Jones, E. W.; Marks, R.; Pongsehirun, D. (1975). "Naevus superficialis lipomatosus. A clinicopathological report of twenty cases". The British Journal of Dermatology. 93 (2): 121–133.
doi:
10.1111/j.1365-2133.1975.tb06731.x.
ISSN0007-0963.
PMID1235780.
^
abcdBuch, A. C.; Panicker, N. K.; Karve, P. P. (2005). "Solitary nevus lipomatosus cutaneous superficialis". Journal of Postgraduate Medicine. 51 (1): 47–48.
ISSN0022-3859.
PMID15793341.