Dear Editor:
Nevus lipomatosus cutaneous superficialis (NLCS) is an infrequent benign hamartoma that was first described by Hoffman and Zurhelle in 1921. It’s characterized by the presence of aggregates of mature adipocytes among the collagen bundles of the dermis. Clinically, NLCS is classified into two types: classic and solitary. Due to its rarity, the diagnosis of NLCS requires a high index of clinical suspicion.1 To our knowledge, few cases of adult-onset NLCS have been reported and there is only one previously reported case of NLCS with clinical resemblance to plane xanthoma.2
A 55-year-old female patient with diabetes presented with asymptomatic skin lesions that had been present on the chin for five months with a sudden onset and progressive course. The patient was diagnosed with nephrotic syndrome eight years prior and was receiving treatment in the form of systemic steroids and angiotensin converting enzyme inhibitor. There was no family history of hyperlipidemia or nephrotic syndrome.
Skin examination showed multiple yellowish, non-tender, smooth, coalescing papules and plaques occupying most of the submandibular and submental areas (Figure 1). General examination revealed generalized edema, puffy eyelids, owed to nephrotic syndrome, and Cushingoid features in the form of moon face, buffalo hump, obese torso and thin, easily bruised skin, all representing the cumulative effect of high-dose steroids taken by the patient. There was no regional lymphadenopathy or organomegaly.
FIGURE 1.
Multiple yellowish, coalescing, smooth papules and plaques involving most of the submandibular and submental areas
Laboratory findings included elevated levels of serum cholesterol (230 mg/dl) and serum creatinine (1.6 mg/dl). Serum albumin and total protein levels were 2.9 g/dl (normal 3.5 to 5 g/dl) and 5.2 g/dl (normal 6 to 8 g/dl), respectively. Urinalysis disclosed 1+ protein. CBC, ESR, serum triglycerides, blood urea and ANA levels were normal. Abdominal ultrasound showed unremarkable findings.
A punch biopsy was taken from one of the yellowish plaques and pathological examination showed lobules of mature adipocytes embedded with collagen fibers in the reticular dermis, with no pathological evidence of xanthoma (Figure 2). Based on these findings, we settled on the diagnosis of NLCS.
FIGURE 2.
A–C) Mature adipocytes are scattered in the dermis, particularly centered around the adnexa and blood vessels with no epidermal changes (A, H&E x100; B, H&E x200; C, H&E x400); D) Characteristic signet ring appearance of fat cells (H&E x400)
NLCS is an uncommon benign hamartomatous cutaneous lesion. The true origin of this rare nevus is obscure and several theories have been proposed to explain its pathogenesis.3 Two clinical types of NLCS are distinguished: the classic (multiple) type and the solitary type. The classic type occurs at birth or during first two decades of life and consists of multiple, soft, pedunculated, skin-colored or yellowish nodules that might coalesce to form plaques. The solitary form usually occurs after the age of 20 years, presents with a single nodular lesion mimicking a skin tag with no particular predilection sites. NLCS is asymptomatic in both types.1
Reported associations include café-au-lait macules, leukodermic spots, overlying hypertrichosis, comedo-like alterations, retractile testis and folliculosebaceous cystic hamartoma.1,3 None of these associations were evident in our patient. However, the clinical appearance is unique and was only reported once before.2 Additionally, the onset of classic NLCS in the elderly along with extension of the lesion across the midline are other rare features observed in our patient.1, 4, 5
NLCS is pathologically characterized by mature adipocytes, irregularly scattered throughout the dermis, particularly centered around the adnexa and blood vessels. Because of this placement, the junction between the dermis and the subcutis is often blurred.
Treatment of NLCS is not indicated except for cosmetic reasons.3 Our patient was offered treatment by carbon dioxide laser, but she refused. Written informed consent was obtained from the patient for publication.
We report this rare case of adult-onset NLCS resembling plane xanthoma with the aim of raising awareness of such morphologically mimicking presentation among dermatologists and highlighting the role of histopathology in confirming the diagnosis.
REFERENCES
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