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   Abstract
   Introduction
   Case Reports
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SHORT COMMUNICATION
Year : 1992  |  Volume : 58  |  Issue : 1  |  Page : 27-29

Naevus lipomatosus cutaneous superficialis : Report of 2 cases


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Correspondence Address:
D M Thappa


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  Abstract 

Two cases of naevus lipomatosus cutaneous superficialis (NLCS) are reported. One of them was a case of multiple form of NLCS while other was having cutaneous lesions resembling solitary form of NLCS.


Keywords: Naevus lipomatosus cutaneous superficialis


How to cite this article:
Thappa D M, Sharma R C, Lal S, Logani K B. Naevus lipomatosus cutaneous superficialis : Report of 2 cases. Indian J Dermatol Venereol Leprol 1992;58:27-9

How to cite this URL:
Thappa D M, Sharma R C, Lal S, Logani K B. Naevus lipomatosus cutaneous superficialis : Report of 2 cases. Indian J Dermatol Venereol Leprol [serial online] 1992 [cited 2014 Sep 2];58:27-9. Available from: http://www.ijdvl.com/text.asp?1992/58/1/27/3736



  Introduction Top


Naevus lipomatosus, cutaneous superficialis (NCLS) is a rare but distinctive hamartoma of adipose tissue. Since first case report by Hoffmann and Zurhelle [1] in 1921, about 130 cases have been reported in the world literature [2] and 6 cases of multiple form of NLCS have been reported in Indian Literature [3],[4],[5] Here we report two cases of NLCS.


  Case Reports Top


Case 1 : R, a 15-year-old female student had asymptomatic nodular lesions over the arms, chest and buttocks of 1 year duration. They gradually increased in number and size. There was no history of preceding trauma. Family history of similar disorder was not forthcoming.

Discrete, skin coloured, smooth, soft, nontender nodular lesions varying in size from 8mm to 12mm, were present on the lateral aspect of both arms [Figure - 1], anterior aspect of chest, and buttocks. The hair, nails and mucosae were normal. No other cutaneous or systemic abnormality was observed.

Histopathological examination of a lesion over upper arm [Figure - 2] showed groups and islands of fat cells embedded among the collagen bundles in the middle and lower dermis, especially around the blood vessels. Epidermis was unremarkable.

Case 2 : AL, a 15 -year-old school girl developed asymptomatic multiple swellings behind the right knee at the age of 7 years. Over a period of time, new lesions appeared which increased in size and spread to involve the inner and outer aspects of the right knee. They remained asymptomatic throughout the course. There was no history of developmental or neurological abnormalities or skin nodules in other members of her family.

Multiple, skin-coloured, soft and nontender papulonodular lesions having wrinkled surface were observed in the right popliteal fossa, extending on to the lateral and medial aspects of the knee [Figure - 3].

They were not fixed to underlying structures. There were no cafe-au-lait macules or axillary freckling. Systemic examination did not reveal any significant finding. Histopathological examination of the lesion from knee revealed findings similar to first case except for hyperkeratosis and islands of fat cells in superficial and mid dermis.


  Comments Top


NLCS is a developmental anomaly, characterised by ectopic adipose tissue in the dermis. The various hypothesis regarding origin of the ectopic fat cells are: deposition of fat secondary to degenerative changes in connective tissue, permeation of subcutaneous tissue or its mesenchyme into the cutis during embryonal development, derivation of pre-adipose tissue from blood vessels, heterotopic histogenesis of adipose tissue and development of adipose tissue from undifferentiated perivascular mesenchymal cells. [6],[7] Electron microscopic studies suggested that fat cells originated from lipoblasts or lipocytes from the perivascular area. [2],[7]

There is neither a sexual predilection nor a familial trend in this disorder. NLCS is classified into a multiple form (Hoffmann­Zurhelle) and a solitary form. [7],[8] Multiple form of NLCS usually manifest at birth or during the first two decades of life. [2],[8] In this type, a group of multiple, soft, nontender, skin coloured or yellowish papules or nodules are found, which often coalesce into plaques. The nodules may be dome-shaped, sessile or pedunculated. Their surface is usually smooth, but they may be wrinkled, verrucoid, or have a peau d'orange appearance. [7] Characteris­tically, they cover a zonal area and usually remain unilateral. The pelvic girdle is most frequently afflicted site, though thorax, abdominal wall, extremities and scalp may by involved. [8] Our case 2 depicted similar lesions over the right popliteal fossa. Solitary form of NLCS usually occurs in third to sixth decade, though it may present at birth also. [2],[9] It is characterised by a solitary, dome-shaped papule or nodule which is skin coloured and soft in consistency [2],[6],[8] and may occur at any site.[ 9] Although similar lesions were observed in our case 1, but they were multiple in number, discrete, irregularly scattered over the arms, anterior aspect of chest and buttocks. Miedzinski [10] reported a similar case having randomly distributed nodular lesions resembling the solitary and gluteal region with skeletal and other naevoid anomalies. However, no such anomalies were observed in our case.

The lesions once formed remain static, although it has been observed that new lobules may continue to develop over the course of many years, [8] as seen in our cases. Occasionally, they may ulcerate, probably from • external trauma. [7] Both clinical types of NLCS show the same histological findings. [2],[8] Usually, there are no associated abnormalities but there are reports of coexistent anomalies in the form of cafe-au-lait spots, scattered leukodermic macules, increased hairiness and comedo like plugs present on the surface of some, patients' lesions. [11] No such, lesions were observed in our cases.

 
  References Top

1.Hoffmann E, Zurhelle E. Uber einen Naevus lipomatodes cutaneus superficialis der linken Glutalegegend. Arch Dermatol Syphilol 1921; 130: 327-33.  Back to cited text no. 1    
2.Chanoki M, Sugamoto I, Suzukie S, et al. Nevus lipomatosus cutaneous superficialis of the scalp. Cutis 1989; 43 : 143-4.  Back to cited text no. 2    
3.Shorff HJ, Moses JM, Gadgil RK, et al. Nevus lipomatosus cutaneous superficialis. Ind J Dermatol Venereol 1971; 37 : 227-30.  Back to cited text no. 3    
4.Siddappa K, Mahipal OA,, Chandrasekhar HR. Nevus lipomatosus cutaneous superficialis (Case Report). Ind J Dermatol Venereol Leprol 1982; 48: 282-6.  Back to cited text no. 4    
5.Gupta K, Gupta AK, Gupta M. Naevus lipomatosus cutaneous superficialis (A reprot of three cases). Ind J Dermatol Venereal Leprol 1986; 52: 35-7.  Back to cited text no. 5    
6.Weitzner S. Solitary nevus lipomatosus cutaneous superficialis of scalp. Arch Dermatol 1968; 97: 540-2.  Back to cited text no. 6  [PUBMED]  
7.Dotz W, Prioleau PG. Nevus lipomatosus cutaneous superficialis (A light and electron microscopic study). Arch Dermatol 1984; 120: 376-9.  Back to cited text no. 7  [PUBMED]  
8.Jones E W, Marks R, Pangsehirun D. Naevus superficialis lipomatosus (A clinicopathological report of twenty cases). Br J Dermatol 1975;93 : 121-33.  Back to cited text no. 8    
9.Sathyanarayana V, Weitzner S. Solitary naevus lipomatosus cutaneous superficialis of the knee. Arch Dermatol 1978;114: 1226-7.  Back to cited text no. 9  [PUBMED]  
10.Meidzinski F. Naevus lipomatoides superficialis. Dermatologica 1963; 126: 223-8.  Back to cited text no. 10    
11.Hendricks WM, Limber GK. Nevus lipomatosus cutaneous superficialis. Cutis 1982; 29 : 183-5.  Back to cited text no. 11  [PUBMED]  


    Figures

[Figure - 1], [Figure - 2], [Figure - 3]

This article has been cited by
1 Nevus lipomatosus superficialis
Yap, F.B.B.
Singapore Medical Journal. 2009; 50(5): e161-e162
[Pubmed]



 

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