|Year : 1992 | Volume
| Issue : 1 | Page : 27-29
Naevus lipomatosus cutaneous superficialis : Report of 2 cases
DM Thappa, RC Sharma, S Lal, KB Logani
D M Thappa
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 2013 May 25];58:27-9. Available from: http://www.ijdvl.com/text.asp?1992/58/1/27/3736
| Introduction|| |
Naevus lipomatosus, cutaneous superficialis (NCLS) is a rare but distinctive hamartoma of adipose tissue. Since first case report by Hoffmann and Zurhelle  in 1921, about 130 cases have been reported in the world literature  and 6 cases of multiple form of NLCS have been reported in Indian Literature ,, Here we report two cases of NLCS.
| Case Reports|| |
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|| |
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. , Electron microscopic studies suggested that fat cells originated from lipoblasts or lipocytes from the perivascular area. ,
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. , Multiple form of NLCS usually manifest at birth or during the first two decades of life. , 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.  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.  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. , It is characterised by a solitary, dome-shaped papule or nodule which is skin coloured and soft in consistency ,, 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  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,  as seen in our cases. Occasionally, they may ulcerate, probably from • external trauma.  Both clinical types of NLCS show the same histological findings. , 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.  No such, lesions were observed in our cases.
| References|| |
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[Figure - 1], [Figure - 2], [Figure - 3]
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