Nevus lipomatosus superficialis

Signs and symptoms

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.

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.

The lesions are asymptomatic in both forms. In rare cases, ulceration happens, particularly following ischemia or external damage. Moreover, coexisting comedo-like changes, leukodermic patches, café-au-lait macules, and overlaying hypertrichosis are possible.

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.

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. Adipocytes can exist alone or in small groups between collagen bundles, but they most frequently originate surrounding blood arteries or eccrine glands. 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. Acanthosis, basket weave hyperkeratosis, elevated basal pigmentation, and obliteration with focal rete ridge extension are observed in the epidermis. Adnexal structures may exhibit perifollicular fibrosis and be unaffected or diminished in certain instances.

Clinically, NLCS needs to be distinguished from focal dermal hypoplasia (Goltz syndrome), neurofibroma, lymphangioma, hemangioma, sebaceous nevus, and connective tissue nevus. File:Pedunculated lipofibroma, gross pathology.jpg|Pedunculated lipofibroma, gross pathology File:Pedunculated lipofibroma (low magnification).jpg|Micrograph of pedunculated lipofibroma, low magnification, with dermal expansion of fatty tissue. File:Pedunculated lipofibroma (intermediate magnification).jpg|Intermediate magnification, showing mature lipocytes with absence of capsule.

Treatment

Given the rarity of malignant degeneration and systemic problems, treatment is only recommended for cosmetic reasons. The best course of treatment is surgical excision because recurrence lesions are uncommon.

References

References

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