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   Abstract
   Introduction
   Case Report
   Comments
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CASE REPORT
Year : 1994  |  Volume : 60  |  Issue : 1  |  Page : 39-40

Naevus lipomatosus superficialis of Hoffman and Zurhelle


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Correspondence Address:
Sandipan Dhar


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  Abstract 

A 45 year old male presented with a large yellowish irregular surfaced tumour of 5 years duration, situated over lower back. There were comedo like lesions over it and a few nodules on top showed necrosis. A diagnosis of naevus lipomatosus superficialis was made. Histopathology revealed presence of mature adipose tissue interposed with bundles of collagen within the reticular dermis. Late appearance, large size, extension beyond midline, comedolike lesions were some unusual features observed in this rare disorder.


Keywords: Naevus lipomatosus Superficialis, Late onset, Ulceration


How to cite this article:
Dhar S, Kumar B, Kaur I. Naevus lipomatosus superficialis of Hoffman and Zurhelle. Indian J Dermatol Venereol Leprol 1994;60:39-40

How to cite this URL:
Dhar S, Kumar B, Kaur I. Naevus lipomatosus superficialis of Hoffman and Zurhelle. Indian J Dermatol Venereol Leprol [serial online] 1994 [cited 2014 Nov 25];60:39-40. Available from: http://www.ijdvl.com/text.asp?1994/60/1/39/3982



  Introduction Top


Naevus lipomatosus superficialis is a rare idiopathic abnormality characterized by isolated dermal collection of adipose tissue. [1] Clinically there are two types of presentations. The classic form, as described first - by Hoffman and Zurhelle, [2] is characterized by grouped soft fleshy skin coloured or yellow nodules, most commonly found over lower back, buttocks and hips. Usually they are present from birth. The second, less common form is a solitary domed or sessile papule developing mostly in adults and have been reported to occur over several other sites apart from lower back. [3],[4] We report a classical case of naevus lipomatosus superficialis with some unusual features.


  Case Report Top


A 45-year-old male presented with a large yellowish irregular surfaced tumour of 5-years duration. The lesion started as a small yellowish sessile nodule which gradually increased in size. He also reported ulceration in the upper part of the lesion for the last 1 month which was now healing with scarring. The patient was otherwise healthy and had no other complaints. There was no family history of similar kind of disease.

Examination revealed a large circular yellowish tumour of about 15 cm diameter having a cerebriform appearance [Figure - 1]. The lesion was situated over the skin overlying the vertebrae L 2 and L 3 extending beyond the vertebral column on both sides. Individual nodules were soft and independently pedunculated but they could not be separated out from the main lesion. A few nodules on the top of the lesion were showing necrosis [Figure - 1] and it emitted rancid odour.

Comedolike plugs were noticed over many of the nodules.

Histopathological examination of biopsy specimen showed normal epidermis, with thinning of papillary dermis. Within the reticular dermis, mature adipose tissues were interposed with bundles of collagen. Fat cells at places were forming sleeves around and compressing the tortuous blood vessels in papillary and reticular dermis.

A second biopsy from the ulcerated site showed relatively more compressed blood vessels by fatty tissue with sloughing out of epidermis and dermis containing polymorphonuclear lymphocytic infiltrate.


  Comments Top


Naevus lipomatosus superficialis is a rare disorder. [5],[6] So far only about 60 cases have been reported, the largest series is that of 20 cases. [6] Idea of the present communication was to highlight some of the unusual observations like late appearance, large size, extension beyond midline and comedo like plugs seen in our patient.

Though late onset is well known in solitary form, in classic type lesions are either present from birth or form childhood. [2] Onset at fourth decade as in our case is extremely rare and so far only 2 such cases have been reported. [5],[6]

Only rarely the lesions cross the midline [7] as was seen in our patient.

Ulceration is only occasionally seen. [8] It probably results from ischaemia due to compression of dermal capillaries, as in our case rather than external trauma mentioned by other workers.

Regarding its pathogenesis, whether this is due to deposition of adipose tissue secondary to degenerative changes in the dermal collagen and elastic tissue, [2] or it is a true naevus resulting from the focal heterotropic development of adipose tissue' is yet to be settled.

 
  References Top

1.Abel R, Dougherty JW. Nevus lipomatosus cutaneous superficialis (Hoffman-Zurhelle). Arch Dermatol 1962; 85 : 132-4.  Back to cited text no. 1  [PUBMED]  
2.Hoffman E, Zurhelle E. Uber einen Naevus lipomatodes cutaneous superficialis der linken Gilutaalgegend. Arch Dermatol Syphilol 1921; 130: 327-33.  Back to cited text no. 2    
3.Satyanarayana V, Weitzner S. Solitary nevus lipomatosus cutaneous superficialis of f the knee. Arch Dermatol 1978; 114: 1226-7.  Back to cited text no. 3    
4.Weitzner S. Solitary nevus lipomatosus cutaneous superficialis of scalp. Arch Dermatol 1968; 97 : 540-2.  Back to cited text no. 4  [PUBMED]  
5.Dotz W, Prioleau PG. Nevuse lipomatosus cutaneouse superficialis - a light and electron microscopic study. Arch Dermatol 1984; 120: 376-9.  Back to cited text no. 5  [PUBMED]  
6.Jones EW, Marks R, Pongschirun D. Nevus superficialis lipomatosus - a clinico­pathological report of twenty cases. Br J Dermatol 1975; 93: 121-33.  Back to cited text no. 6    
7.Robinson HM, Ellis FA. Naevus lipomatosus subepidermalis seu superficialis cutis. Arch Dermatol 1937; 35 : 485-8.  Back to cited text no. 7    
8.Girglia HS, Bhattacharya SK. Naevus lipomatosus cutaneous superficialis. Int J Dermatol 1975; 14 : 273-6.  Back to cited text no. 8  [PUBMED]  


    Figures

[Figure - 1]

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Chougule, A., Kumari, R., Thappa, D.M.
Indian Journal of Dermatology. 2007; 52(2): 120-121
[Pubmed]
3 Nevus lipomatosus superficialis over neck, an atypical site [8]
Das, J.Kr., Sengupta, S., Gangopadhyay, A.Kr.
Indian Journal of Dermatology, Venereology and Leprology. 2006; 72(1): 66-67
[Pubmed]



 

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