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THROUGH THE LENS
Year : 2014  |  Volume : 5  |  Issue : 1  |  Page : 109-110  

Nevus lipomatosus superficialis: A rare cutaneous hamartoma


1 Department of Pathology, Lady Hardinge Medical College and associated hospitals, New Delhi, India
2 Department of Dermatology, Lady Hardinge Medical College and associated hospitals, New Delhi, India

Date of Web Publication30-Jan-2014

Correspondence Address:
Meenu Pujani
Department of Pathology, Lady Hardinge Medical College and associated hospitals, New Delhi
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2229-5178.126069

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How to cite this article:
Pujani M, Choudhury M, Garg T, Madan NK. Nevus lipomatosus superficialis: A rare cutaneous hamartoma. Indian Dermatol Online J 2014;5:109-10

How to cite this URL:
Pujani M, Choudhury M, Garg T, Madan NK. Nevus lipomatosus superficialis: A rare cutaneous hamartoma. Indian Dermatol Online J [serial online] 2014 [cited 2019 Dec 9];5:109-10. Available from: http://www.idoj.in/text.asp?2014/5/1/109/126069

Nevus lipomatosus superficialis is an uncommon benign hamartomatous skin lesion defined by the presence of aggregates of mature adipose tissue among the collagen bundles of the dermis. It was first reported by Hoffman and Zurhelle in 1921. [1] We report a case of a 15-year-old girl with classic form of this rare skin malformation.

A 15-year-old girl presented with a history of multiple, skin-colored, pedunculated, cerebriform, soft, asymptomatic nodules varying in size from 2 × 1 to 2 × 2 cm on the lower back for 10 years [Figure 1]a. There was no history of any neurological deficit, and general physical examination did not reveal any cafe-au-lait spots. Skin biopsy was performed and microscopy showed an unremarkable epidermis with presence of islands of mature adipose tissue in the dermis interposed among the collagen bundles and comprising more than 50% of the dermis [Figure 1]b, [Figure 2] and [Figure 3].
Figure 1: (a) Gross photograph showing multiple, skin-colored, pedunculated, cerebriform nodules on the lower back; (b) skin biopsy revealing dermis almost replaced by islands of mature adipose tissue [H and E, 100×]

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Figure 2: Microphotograph showing adipose tissue interspersed among collagen bundles [H and E, ×100]

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Figure 3: Microphotograph showing mature adipose tissue replacing most of the dermis [H and E, ×100]

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After considering the above-mentioned characteristic features, a diagnosis of Nevus lipomatosus superficialis was given.

Nevus lipomatosus superficialis is a rare benign idiopathic skin malformation characterized by the presence of ectopic mature adipose tissue in the dermis. The proportion of the fatty tissue varies greatly from more than 50% to < 10% of the dermis. [2] Two clinical types are distinguished: the classical type and the solitary type. The classical type, first reported by Hoffman and Zurhelle, consists of multiple, soft, nontender, pedunculated, cerebriform, yellowish or skin-colored papules or nodules usually situated on the pelvic girdle area in a zonal pattern and occur at birth or during first three decades of life. [1] The solitary form usually occurs after the age of 20 years, presents with a single nodular lesion with no particular predilection sites. [1]

Clinically, the differential diagnosis includes neurofibromatosis, lymphangioma, hemangioma, skin tag or fibroepithelial polyp all of which can be distinguished histologically. Microscopically, adipose tissue in dermis is seen in focal dermal hypoplasia. However, these two entities are clinically distinct. Focal dermal hypoplasia is associated with several ectodermal and mesodermal deformities including syndactyly, hypoplasia of hair, nail, and teeth, cutaneous ulcers with thinning of skin. Microscopically, dermis is almost entirely replaced by fat cells. [3]

Treatment, though not necessary, is done for cosmetic reasons and simple surgical excision appears to be curative. Lesions are generally nonprogressive and systemic abnormalities and malignant alterations have not been associated with this abnormality. [3]

 
   References Top

1.Yap FB. Nevus lipomatosus superficialis. Singapore Med J 2009;50:161-2.  Back to cited text no. 1
    
2.Ragsdale BD. Tumors with fatty, muscular, osseous, and/or cartilaginous differentiation. Lever's histopathology of the skin,10 th ed. Elder DE, Elenitsas R, Jhonson BL, Murphy GF, Xu X editors. Wolters Kluwer-Lippincott Williams & Wilkins; 2009.   Back to cited text no. 2
    
3.Park H, Park C, Yi J, Kim T, Kim C. Nevus lipomatosus superficialis on the face. Int J Dermatol 1997;36:435-7.  Back to cited text no. 3
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]


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