Nevus Lipomatosus Cutaneous Superficialis - A Rare Hamartoma: Report of Two Cases

Abstract

Case 1: A 40-years-old female presented with asymptomatic growth on lower back which had gradually increased to the present size over a period of 25 years. There were no systemic complaints. Family history of similar lesions was absent. Cutaneous examination revealed multiple soft, skin colored to hyperpigmented, non tender, smooth surfaced, coalescing papules and cerebriform plaques situated on the right lower back [Table/ Fig-1]. There was no ulceration, discharge, associated excessive hair growth, comedo like plugs or cafe-au-lait spots. Biopsy from the papule revealed islands of mature adipose tissue in the dermis [Table /Fig-2]. Based on the clinical and histopathological findings diagnosis of nevus lipomatosus cutaneous superficialis was confirmed. Patient was referred to plastic surgery department for further management.

Multiple yellow nodules in a 13-year-old girl

“…Lesions usually occur at birth or in the first two decades of life without any familial tendency or sex predilection. Clinically, NLCS may present as one of two variants: (i) the classic form, which is composed of multiple, grouped, skin‐coloured, pedunculated nodules, and (ii) the rarer solitary form, which is characterized by a solitary dome‐shaped sessile papule or nodule, and is commonly seen in elderly patients …”

“…Clinically, NLCS may present as one of two variants: (i) the classic form, which is composed of multiple, grouped, skin-coloured, pedunculated nodules, and (ii) the rarer solitary form, which is characterized by a solitary dome-shaped sessile papule or nodule, and is commonly seen in elderly patients. 2 NLCS is usually located on the abdomen, back, buttocks, hips, upper posterior thigh and pelvic area, with a unilateral, linear or zosteriform arrangement. The classic form, which was that described by Hoffman and Zurhelle, is characterized by clusters of nontender, yellow or skin-coloured, soft papules or nodules, with a smooth and wrinkled surface, which occur within the first three decades of life and may reach up to 20-30 cm over time.…”

“…A few theories have been proposed, including the role of fat deposition secondary to some degenerative changes in the connective tissue; a true naevus formation from the developmental displacement of adipose tissue; metaplasia of collagen tissue with transformation into adipose tissue; and growth of the mature adipocytes from mononuclear cells differentiating into lipoblasts in a perivascular area and focal heterotopic development of fat in the dermis. 2 In addition, deletion of 2p24 in NLCS was reported to have a supporting genetic role in the development of NCLS. 4 Clinically important differential diagnoses of NLCS include naevus sebaceous, plexiform neurofibroma, focal dermal hypoplasia, segmental neuroma, connective tissue naevus, angiolipoma and lipomatosis.…”

Nevus Lipomatosus Superficialis on the Left Proximal Arm

“…In another retrospective study, 8 cases were seen in a 14-year-period from 1997 to 2010 at the Department of Dermatology, Charles Nicole Hospital in Tunis, Tunisia [ 5 ]. There is no familial or sex predilection [ 3 ].…”

“…The condition was first described by Hoffman and Zurhelle in 1921 [ 1 ]. Two types of nevus lipomatosus cutaneous superficialis are recognized, namely, the classical multiple type (also known as the Hoffman-Zurhelle type) and the solitary type [ 2 , 3 ]. We describe a 58-year-old woman with a solitary type of nevus lipomatosus superficialis on the left proximal arm.…”