Indian Journal of Dermatology
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Year : 2013  |  Volume : 58  |  Issue : 4  |  Page : 330
Nevus lipomatosus cutaneous superficialis associated with trichofolliculoma on the lower leg


Department of Dermatology, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, Japan

Date of Web Publication25-Jun-2013

Correspondence Address:
Masanao Sakanoue
Department of Dermatology, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima
Japan
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5154.113999

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How to cite this article:
Sakanoue M, Kawai K, Kanekura T. Nevus lipomatosus cutaneous superficialis associated with trichofolliculoma on the lower leg. Indian J Dermatol 2013;58:330

How to cite this URL:
Sakanoue M, Kawai K, Kanekura T. Nevus lipomatosus cutaneous superficialis associated with trichofolliculoma on the lower leg. Indian J Dermatol [serial online] 2013 [cited 2019 Aug 21];58:330. Available from: http://www.e-ijd.org/text.asp?2013/58/4/330/113999


Sir,

This 54-year-old apparently healthy woman presented with multiple lesions on her lower right leg that had been present for about 50 years without significant changes in their size or shape. Physical examination revealed skin-colored soft papules in zosteriform distribution; they measured approximately 10 cm [Figure 1]a. Skin biopsy showed an ectopic growth of mature fat between collagen bundles in the dermis [Figure 1]b. Electron microscopy revealed premature adipocytes with pinocytosis and marginal condensation [Figure 2]. A centrally located cystic follicular structure accompanied by sebaceous glands embedded in fibrous stroma, compatible with trichofolliculoma, was noted in the subcutaneous fat tissue [Figure 3]. These findings resulted in a diagnosis of Nevus lipomatosus cutaneous superficialis (NLCS) associated with trichofolliculoma on the lower leg.
Figure 1: (a) Skin - colored soft papules in zosteriform distribution on the lower right leg. (b) Ectopic growth of mature fat between collagen bundles in the dermis (H and E stain, ×40)

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Figure 2: Electron microscopically there were premature adipocytes showing pinocytosis (arrow heads) and marginal condensation (arrow) (×700, ×40000)

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Figure 3: Note the centrally located cystic follicular structure accompanied by some sebaceous glands in fibrous stroma (H and E) (×12.5, ×40)

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NLCS, first described by Hoffman and Zurhelle, [1] is histologically characterized by mature ectopic fat tissue in the upper dermis unconnected to subcutaneous fat tissue. It is usually present at birth but can arise later. NLCS is classified as multiple- and solitary type. The multiple type tends to occur in the lumbar region, buttocks, and thigh and usually consists of groups of yellowish or skin-colored soft papules, nodules, or plaques with smooth or wrinkled surfaces. The lesions are usually unilateral and may show a linear, band-like, or zosteriform distribution. The solitary type usually features a single papule or nodule anywhere on the skin. To date, only two cases of NLCS on the lower legs, calf, and sole have been reported; both were solitary type. No multiple type NLCS on the lower leg have been documented.

The pathogenesis of NCLS is not fully understood. Findings in NLCS are reminiscent of fetal adipogenesis; pinocytosis and condensation of the plasma membrane and the many vascular elements in contact with adipocytes suggest a vascular origin of the adipose cells.

Electron microscopically, NLCS in our patient manifested immature lipocytes showing pinocytosis and marginal condensation, suggesting that the ectopic fat cells in the dermis originated from perivascular mesenchymal tissue. A peculiar histopathological finding was the association with trichofolliculoma, a hamartoma of pilosebaceous follicles manifesting as small nodules with protruding tufts of hair primarily on the scalp, face, and neck. While we found no other report of the association of NLCS and trichofolliculoma, there have been four NLCS cases associated with folliculosebaceous cystic hamartoma (FCH), [2],[3],[4] a rare cutaneous hamartoma containing follicular, sebaceous, and mesenchymal elements such as ectodermal and mesodermal components.

Based on a histopathologic study comparing 8 FCH- and 31 trichofolliculoma cases, others [5] posited that FCH is the very late stage of trichofolliculoma. While the relationship between NLCS and trichofolliculoma or FCH remains unclear, this association of two distinct hamartomatous disorders suggests epidermal structures as part of NLCS. [2],[3],[4] The discovery of a case of NLCS with follicular papules and hypertrophic pilo-sebaceous units supports this possibility.

We report the first case of the multiple type NLCS with trichofolliculoma on the lower leg, an extremely unusual site for both lesions.

 
   References Top

1.Hoffmann E, Zurhelle E. Über einen naevus lipomatodes cutaneous superficialis der linken Glutäalgegend. Arch Derm Syphilol 1921;130:327-33.  Back to cited text no. 1
    
2.Brasanac D, Boricic I. Giant nevus lipomatosus superficialis with multiple folliculosebaceous cystic hamartomas and dermoid cyst. J Eur Acad Dermatol Venereol 2005;19:84-6.  Back to cited text no. 2
    
3.Kang H, Kim SE, Park K, Son SJ, Song KY. Nevus lipomatosus cutaneous superficialis with folliculosebaceous cystic hamartoma J Am Acad Dermatol 2007;56(2 Suppl):S55-7.  Back to cited text no. 3
    
4.Bancalari E, Martínez-Sánchez D, Tardío JC. Nevus lipomatosus superficialis with a folliculosebaceous component: Report of two cases. Patholog Res Int 2011;2011:105973.  Back to cited text no. 4
    
5.Schulz T, Hartschuh W. Folliculo-sebaceous cystic hamartoma is a trichofolliculoma at its very late stage. J Cutan Pathol 1998;25:354-64.  Back to cited text no. 5
    


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  [Figure 1], [Figure 2], [Figure 3]



 

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