Nevus lipomatosus cutaneous superficialis: An unusual presentation

Abstract

Nevus lipomatosus cutaneous superficialis (NLCS) is an uncommon benign hamartomatous condition characterized by the presence of mature ectopic adiopocytes in the dermis. It was first reported by Hoffman and Zurhelle in 1921. Clinically they areit is classified into two forms. The classical form is characterized by groups of multiple, non-tender, soft, pedunculated, cerebriform, yellowish or skin-colored papules, nodules, or plaques. The other form of NLCS clinically manifests as a solitary dome-shaped or sessile papule. The classical NLCS is mostly reported to involve the pelvic or gluteal region. We report here a case of adult-onset classical NLCS on perianal area because of its rarity and unusual location. In addition, our patient also had some rare features of NLCS, such as recurrent in nature, presence of foul-smelling discharge, and comedo-like plugs on the lesions.

Nevus Lipomatosis Cutaneous Superficialis – A clinicopathologic study of the solitary type

“…Though Hoffmann and Zurhelle postulated that the fat deposition in the dermis was secondary to degenerative changes in the connective tissue, 1 we did not observe any degenerative changes in collagen or elastic fibres. Some authors have suggested that the fat cells represented a true nevus which resulted from focal heterotopic development of adipose tissue during embryonic life.…”

“…These lesions, occasionally may have a foul smelling discharge and may show the presence of comedo-like plugs. 1 Histopathological examination of NLCS revealed an atrophic epidermis in all cases and focal spongiosis in one case. Changes like mild to moderate acanthosis, basket weave hyperkeratosis, increased basal cell pigmentation, focal elongation of rete ridges have been described but were not seen in our cases.…”

“…Systemic abnormalities or malignant change have not been reported with NLCS and post surgical recurrence is rare. 1 …”

“…1 The classical form can be present at birth but usually appears in the first two decades of life. It is seen to have a linear, zonal distribution along natural cleavage lines of the skin and has a predilection for the pelvic girdle, sacral and lumbar regions.…”

“…The solitary form is uncommon and presents as a single sessile or dome shaped papule. 1 Though there have been many isolated case reports published of NLCS, a study of case series have been very few and most of them describe the classical variant as the predominant type. 2,3 We highlight the clinicopathological features of 7 cases of the solitary type of NLCS, all of which were clinically misdiagnosed.…”