Giant naevus lipomatosus cutaneous superficialis on the buttock

Naevus lipomatosus cutaneous superficialis (NLCS) is a rare idiopathic benign hamartomatous skin tumour characterized by mature ectopic dermal lipocytes with no associated systemic symptoms. It was first described by Hoffman and Zurhelle in 1921 [1] and is now classified into 2 clinical subtypes: classical (multiple) and solitary. The classical form usually presents in adolescence and consists of grouped, fleshy, skin-coloured to yellow papules and nodules that coalesce into plaques and are often located on the lower trunk, buttocks, or thighs in a segmental distribution. The second variant presents in adulthood as a solitary papule or nodule anywhere on the skin, including unusual sites such as the sole [2], nose [3], and vulva [4]. Here, we report a rare case of classical giant NLCS on the right buttock with successful treatment by surgical excision. To date, reported cases of NLCS with positive treatment outcomes, as in the case presented herein, are rare.
A 19-year-old girl presented with a giant (17 × 25 cm in size), flesh-coloured, soft, nontender, rubbery, sessile, and cerebriform mass on the right buttock, which was attached with multiple comedones on the surface (Figure 1 A). The mass appeared 10 years prior and gradually increased in size. When she visited our hospital, the mass was so large that her appearance and movement had been obviously influenced. There were no symptoms due to the lesions. The patient was otherwise healthy, and there was no relevant family history. No therapies had been attempted previously. Serum triglyceride and cholesterol levels were normal. The lesions were completely removed by excisional surgery and the treatment site was covered with local advanced flaps. Histological examination of a diagnostic biopsy found normal epidermis and mature fat cell deposits in the superficial dermis (Figure 1 B). Based on the clinical and histopathological features, the diagnosis of naevus lipomatosus cutaneous superficialis (NLCS) was made.
NLCS is a rare developmental anomaly characterized by isolated ectopic mature adipose tissue in the dermis [1]. Only a few cases have been reported in the literature worldwide. NLCS is usually present at birth but can arise anytime within the first 2 decades of life, with the main affected locations including the pelvic girdle, gluteal region, back, or abdomen [5]. The pathogenesis of NLCS is still unclear. Hoffman and Zurhelle postulated that fat deposition in the dermis is secondary...


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