Photoclinic

 

 

 


 

 

 

 

 

 

 

Reza Yaghoobi MD*, Niloofar Sina MD*, Arian Rashidi MD**

 

Authors affiliations: *Department of Dermatology, **Department of Pathology, Ahwaz University of Medical Sciences, Ahwaz, Iran.

•Corresponding author and reprint:   Reza Yaghoobi,  MD, Department of Dermatology, Emam Khomeini Hospital, 61335, Ahwaz, Iran. Fax: +98-611-3360840, E-mail: yaghoobi_rz@yahoo.com.


A

35-year-old woman was referred to us because of asymptomatic linear tumors on the left upper thigh. The tumors had been noted six years earlier and had gradually increased in size. She was otherwise in good health.

 Physical examination showed multiple skin-colored, soft, nontender and 1 to 3 cm nodules confined to the left upper thigh. These lesions had smooth surfaces, and in some areas coalesced to form cerebriform plaques. Results of routine laboratory studies were normal. A biopsy specimen was taken from one of the lesions and stained with hematoxylin and eosin.

   

Your Diagnosis?

See page  316  for the diagnosis

 

 

 

 

 

Photoclinic Diagnosis:      Nevus Lipomatosus Cutaneus Superficialis (Hoffmann-Zurhelle)

Histopathological findings and clinical course

Histopathological examination showed lobules of fat cells embedded among the collagen bundles in the papillary and reticular dermis and these collagen bundles were proliferated irregularly. The lobules were not encapsulated and had no connection with subcutaneous fat tissue.

The epidermal changes of the lesion consisted of epidermal undulation, with mild acanthosis, irregular papillomatosis, and mild hyper-pigmentation of basal layer. Also, hyperkeratosis with some keratotic plugs were noted.

These histological features were consistent with the diagnosis of nevus lipomatosus cutaneus superficialis (NLCS). After surgical removal of the lesions, no evidence of recurrence was found.

NLCS was first described in 1921 by Hoffmann and Zurhelle and consists of an extremely rare idiopathic hamartomatous disorder, characterized histopathologically by the constant presence of isolated adipocytes or mature  adipose tissue in the dermis without a connection to the hypodermis.1 NLCS is classified into two clinical types; where multiple form (or classic type) and solitary form.1 – 3 The multiple form is a rare type, and the multiple skin lesions are seen at birth or develop during the first three decades of  life2 and rarely develop in the elderly.1

Morphologically, the lesions are soft, nontender, skin-colored to yellow papules, and nodules which often coalesce into plaques of varying size. Their surface is either smooth, wrinkled, cerebriform, or verrucoid with comedones and peau d’orange appearance.1 The distribution is usually zonal or zosteriform and the predelication site is the pelvic girdle, most commonly the buttock, sacral or coccygeal region, and upper part of posterior thigh.2, 3

The solitary type of NLCS usually forms a dome-shaped papule or plaque. Lesions mostly appear in adulthood, and grow slowly.4 To date, this type of NLCS has been reported on the face, scalp, forehead, back, axilla, arm, upper thigh, and knee.3 There is neither a sexual predelication nor a familial trend in this disorder. Usually no associated abnormalities are present.1

The origin of NLCS is still unclear. Several theories have been proposed for the origin of adipose tissue in NLCS: 1) the deposition of fat tissue in the dermis is secondary to some degenerative changes in connective tissue; 2) fat cells result from focal heterotopic development of adipose tissue in the dermis; 3) adipose tissue originates as a ‘preadipose’ tissue derived from the blood vessels, and 4) recent electron microscopic studies also suggest that fat cells are derived from a small precursor cell arising or lying in close proximity to blood vessels.1

Treatment is usually not necessary in NLCS unless for cosmetic reasons, in which case simple surgical excision is sufficient. It is commented that this lesion does not recur after excision.3

 

References

 

1       Ioannidou DJ, Stefanidou MP, Panayiotides JG, Tosca AD. Nevus lipomatosus cutaneus superficialis (Hoffmann-Zurhelle) with localized scleroderma-like appearance. Int J Dermatol. 2001; 40: 54 – 57.

2       Inoue M, Ueda K, Hashimoto T. Nevus lipomatosus cutaneus superficialis with follicular papules and hypertrophic pilo-sebaceous units. Int J Dermatol. 2002; 41: 241 – 243.

3       Park HJ, Park CJ, Yi JY, Kim TY, Kim CW. Nevus lipomatosus superficialis on the face. Int J Dermatol. 1997; 36: 435 – 437.

4       Maeda A, Aragane Y, Ueno K, Yamazaki F, Kawada A, Tezuka T. A case of nevus lipomatosus cutaneus superficialis of the scalp associated with pedunculated basal cell carcinoma. Br J Dermatol. 2003; 148:1084 – 1086.

 


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