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Table of Contents 
Year : 2017  |  Volume : 62  |  Issue : 4  |  Page : 429-431
Nevus lipomatosus cutaneous superficialis with unusual presentation over the nipple

1 Department of Pathology, SHKM, GMC, Mewat, Haryana, India
2 Department of Pathology, Assam Medical College, Dibrugarh, Assam, India
3 Department of Pathology, BPS Medical College, Khanpur, Haryana, India
4 Department of pathology, SMS Medical College, Jaipur, Rajasthan, India

Date of Web Publication10-Jul-2017

Correspondence Address:
Shilpa Bairwa
Department of Pathology, SHKMs, GMC, Mewat, Haryana
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijd.IJD_384_16

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How to cite this article:
Bairwa S, Sharma M, Sangwaiya A, Singla S, Gupta K, Yadav A. Nevus lipomatosus cutaneous superficialis with unusual presentation over the nipple. Indian J Dermatol 2017;62:429-31

How to cite this URL:
Bairwa S, Sharma M, Sangwaiya A, Singla S, Gupta K, Yadav A. Nevus lipomatosus cutaneous superficialis with unusual presentation over the nipple. Indian J Dermatol [serial online] 2017 [cited 2022 Oct 4];62:429-31. Available from:


Nevus lipomatosus cutaneous superficialis (NLCS) is a rare idiopathic benign hamartomatous cutaneous lesion characterized by the presence of mature ectopic fat in the dermis. Hoffman and Zurhelle were the first to report this rare developmental skin anomaly in 1921.[1] The classical type clinically presents as asymptomatic, groups of multiple, soft, nontender, skin colored to yellow papules, and nodules which are either sessile or pedunculated with a smooth, wrinkled, or cerebriform surface.[2] Herein, we report a case of classical NLCS in a 25-year-old female having two swellings, one over the nipple and other on the sacral region. Nipple involvement is not commonly reported. To the best of our knowledge, classical NLCS of nipple has not been reported till date.

A 25-year-old female presented with two asymptomatic, slow growing papulonodular lesion over the right nipple and left sacral region since birth. Both the lesions started simultaneously as a single, small papule that progressively increased in size over a period and attained the present size. No other skin abnormalities and no history of similar lesions in the family were noted. There was no history of trauma, discharge, and itching. Physical examination of the swellings over the nipple and sacral region revealed multiple, soft, skin-colored to hyperpigmented papules, and nodules measuring 6 cm × 5 cm × 3 cm and 5 cm × 4 cm × 2 cm, respectively, [Figure 1] and [Figure 2]. The surface over the swellings was wrinkled, cerebriform, and nonhairy. Excision biopsy was done. Histopathological examination of both the lesion revealed hyperplasia, hyperkeratosis, papillomatosis of the epidermis, and hyper-pigmentation of the basal layer. Dermis show groups of mature fat cells, distributed throughout as nests and cords [Figure 3]. There is increased density of collagen and skin appendages are not displaced [Figure 4]. On the basis of clinical and histopathological examination, diagnosis of NLCS was made. The patient was referred to the surgery department for surgical excision.
Figure 1: Polypoidal nodular skin-coloured mass arising from nipple

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Figure 2: Multiple, sessile, skin-coloured papules and nodules having a wrinkled, verrucous surface over the left buttock

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Figure 3: Photomicrograph from nipple lesion showing epidermal hyperplasia, hyperkeratosis, papillomatosis, and groups of mature fat cells in dermis (H and E, ×40)

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Figure 4: Photomicrograph from nipple lesion showing increased density of collagen and fat lobules in the dermis (H and E, ×200)

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Nevus lipomatosus as the name implies represents a nevoid anomaly characterized by the presence of mature ectopic fat in the dermis. Classical NLCS has a predilection for pelvic girdles, buttocks, gluteal region, back, and abdomen. The lesion may be present at birth or infancy (nevus angiolipomatosis of Howell) or may emerge in the first two decades.[3] The solitary lesions have no gender and site predilection and may occur at unusual site like scalp and clitoris have been reported.[2],[3]

The etiopathogenesis of NLCS is still unknown. Many theories have been postulated. Hoffman and Zurhelle proposed that it is the degenerative changes in the dermal collagen and elastic tissue which causes the depositon of adipose tissue in the dermis.[3] Other hypothesis includes adipose metaplasia of dermal connective tissue or representing a true nevus resulting from focal heterotropic development during embryonic life. Electron microscopy supports the view that they may originate from the pericytes as immature lipocytes containing numerous small lipid droplets are found in the vicinity of capillaries.[4] Deletion of 2p24 in the NLCS has been reported recently.[5]

Differential diagnosis of NLCS includes nevus sebaceous, skin tag or fibroepithelial polyp, neurofibroma, lymphangioma, lipofibroma, focal dermal hypoplasia (Goltz syndrome), lipomatosis, and Michelin tire baby syndrome.[2],[4] Histopathological examination helps in the differentiation. There is absence of fat cells in the dermis in skin tag. In lipofibroma skin, appendages are absent in the dermis, but a dermal collection of fat cells are present. Goltz syndrome is associated with several ectodermal and mesodermal deformities, and clinically, the patient presents with syndactyly, hypoplasia of nails, teeth, and hairs. Microscopically, there is extreme attenuation of collagen and absence of skin appendages in the dermis. There is no continuity of the fat cells with the subcutis in lipomatosis.[6]

Recurrences and malignant transformations are rare, and therefore, treatment is medically not required. A surgical incision is curative though not necessary done only for cosmetic purposes.[2]

To conclude, the pathologist should be aware of this rare cutaneous hamartomatous lesion of the skin, and early diagnosis is required so to eliminate the extensive reconstruction of the defect and to reduce the postoperative scar formation.

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   References Top

Chaogule A, Rashmi K, Thappa DM. Giant nevus lipomatosus superficialis of the thigh. Indian J Dermatol 2007;52:120-1.  Back to cited text no. 1
Buch AC, Panicker NK, Karve PP. Solitary nevus lipomatosus cutaneous superficialis. J Postgrad Med 2005;51:47-8.  Back to cited text no. 2
[PUBMED]  [Full text]  
Khandpur S, Nagpal SA, Chandra S, Sharma VK, Kaushal S, Safaya R. Giant nevus lipomatosus cutaneous superficialis. Indian J Dermatol Venereol Leprol 2009;75:407-8.  Back to cited text no. 3
[PUBMED]  [Full text]  
Dotz W, Prioleau PG. Nevus lipomatosus cutaneus superficialis. A light and electron microscopic study. Arch Dermatol 1984;120:376-9.  Back to cited text no. 4
Cardot-Leccia N, Italiano A, Monteil MC, Basc E, Perrin C, Pedeutour F. Naevus lipomatosus superficialis: A case report with a 2p24 deletion. Br J Dermatol 2007;156:380-1.  Back to cited text no. 5
Park HJ, Park CJ, Yi JY, Kim TY, Kim CW. Nevus lipomatosus superficialis on the face. Int J Dermatol 1997;36:435-7.  Back to cited text no. 6


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]

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[Pubmed] | [DOI]


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