Medical Journal of Dr. D.Y. Patil Vidyapeeth

CASE REPORT
Year
: 2017  |  Volume : 10  |  Issue : 6  |  Page : 589--591

Solitary form of nevus lipomatosus cutaneous superficialis: A rare entity with review of literature


Krishna Tadepally, Vijaya Gattu 
 Department of Pathology, SVS Medical College and Hospital, Mahabubnagar, Telangana, India

Correspondence Address:
Dr. Krishna Tadepally
SVS Medical College and Hospital, Mahabubnagar, Telangana
India

Abstract

A 45-year-old female presented with a history of pedunculated, soft, nodule on the right leg in popliteal region for 5 years. The lesion started as a small nodule and increased in size for 2 years and remained unchanged thereafter. The histological examination confirmed ectopic mature adipose tissue within dermis that supported the clinical diagnosis of nevus lipomatosus. This case report is of a solitary form of nevus lipomatosis cutaneous superficialis or pedunculated lipofibroma.



How to cite this article:
Tadepally K, Gattu V. Solitary form of nevus lipomatosus cutaneous superficialis: A rare entity with review of literature.Med J DY Patil Univ 2017;10:589-591


How to cite this URL:
Tadepally K, Gattu V. Solitary form of nevus lipomatosus cutaneous superficialis: A rare entity with review of literature. Med J DY Patil Univ [serial online] 2017 [cited 2020 Oct 24 ];10:589-591
Available from: https://www.mjdrdypu.org/text.asp?2017/10/6/589/223365


Full Text



 Introduction



Nevus lipomatosis cutaneous superficialis (NLCS) is a rare idiopathic hamartomatous anomaly, the classic Hoffman–Zurhelle type or multiple form and the pedunculated solitary form. Usually, it presents with asymptomatic grouped, soft, skin-colored to yellow papules and nodules within the first three decades of life. There is neither familial tendency nor sex predilection. Histology is characteristic with ectopic mature adipose tissue within dermis.[1],[2]

 Case Report



A 45-year-old female presented with a 5-year history of nodule on her right leg.

The lesion started as a small, single nodule and increased in size for 2 years and remained unchanged thereafter. The lesion caused no symptoms except for unsightly appearance. There was no family history of similar lesions. Physical examination revealed single soft, skin-colored nodule 1.5 cm × 1 cm on the right leg. Systemic examination revealed no abnormality. All routine laboratory investigations were within normal limits. The suggested clinical differential diagnoses were skin tag, dermolipoma, and wart. The nodule was excised. Received single skin covered tissue bit in formalin measuring 1.5 cm × 1.0 cm × 0.8 cm, cut section was gray yellow. Histopathological examination revealed epidermis with hyperkeratosis. Dermis shows mature adipose tissue [Figure 1] and [Figure 2]. These findings supported the diagnosis of nevus lipomatosus.{Figure 1}{Figure 2}

 Discussion



NLCS is an uncommon idiopathic hamartomatous anomaly, presented with soft, flattened papules or nodules that have smooth or wrinkled surfaces and are skin colored or pale yellow. Mostly the lesions are linearly distributed or may overlap on to the adjacent skin. Histology shows ectopically situated mature adipocytes within the dermis.[1] Clinically, there are two types. The classic (or multiple) type [2] usually presents within the first three decades of life with clusters of soft, cribriform, and peau d'orange appearance, located most commonly on the lower trunk, especially on the back, buttocks, or hips and on the upper posterior thighs.[1],[3],[4],[5]

Rare involvement of the face [6] or scalp [7] has been reported. They are generally present at birth, but they may first appear during childhood or adolescence.[4],[8]

They are almost invariably asymptomatic although occasionally ulceration may occur.[9] The solitary form of NLCS usually appears during the third to sixth decades of life as a single papule or nodule without a favored location.[3],[10],[11] There is no evidence of a familial tendency or sex predilection in either clinical type.[3] There are reports of coexisting café-au-lait macules, leukodermic spots, overlying hypertrichosis, and comedo-like alteration.[3],[12]

Histopathology

Groups and strands of fat cells are found embedded among the collagen bundles of dermis often in papillary dermis. Proportion of fat tissue varies from 10% to more than 50%. The fat cells may all be mature, but in some instances and an occasional small, incompletely lipidized cell may be observed. NLCS contains fat cells but no skin appendages in the dermis.[13]

Pathogenesis

It represents a nevoid anomaly. The ectopic fat cells in the dermis are derived from perivascular mesenchymal tissue.[13] Other theory for etiopathogenesis is adipose tissue metaplasia during the degenerative changes in dermal connective tissue, 2p24 deletion has also been noted.[14]

A recent report described a giant NLCS with multiple folliculosebaceous cystic hamartomas and dermoid cysts.[15] A case of NLCS with localized scleroderma-like appearance has been reported.[16] The differential diagnosis before biopsy may include old nevocellular nevi, sebaceous nevus, neurofibromas, connective tissue nevi, epidermal nevi, the dermal variant of spindle cell lipomas, acrochordones, focal dermal hypoplasia, lipoblastomatosis, and Michelin tire baby syndrome.[3] Sebaceous nevus contains skin appendages in contrast to NLCS.

The dermal variant of spindle cell lipoma contains more spindle-shaped cells as well as a fibromucinous stroma. In focal dermal hypoplasia, fat cells present in dermis, often in proximity to epidermis but have concomitant extreme attenuation of collagen.[3]

Focal dermal hypoplasia is also associated with several ectodermal and mesodermal deformities include syndactyly, hypoplasia of hair, nails, and teeth. Similar dermal collections of the adipose tissue may occur as a component of long-standing intradermal melanocytic nevi and in pedunculated lipofi broma but long-standing intradermal melanocytic nevi associated with nevus cell nests and represent an involutory phenomenon.[17] To date, there have been no reports of malignant degeneration and recurrences are extremely rare, so treatment is not medically necessary.[3]

For cosmetic purposes, surgical excision is the best choice.[3],[18]

No significant complications observed in majority of cases. In some cases, foul-smelling discharge can occur, due to ulceration. In some rare cases, the tumor may recur after surgical excision. At present, the term “pedunculated lipofibroma” is used to denote solitary forms of NLCS.[19]

Another recent report described a rare case of congenital classical NLCS presented as cerebriform lesions with centrally located comedo-like plugs.[20]

Epidermal changes such as mild to moderate acanthosis, basket weave hyperkeratosis, obliteration of rete pegs, increased basal pigmentation, and focal elongation of rete pegs have been reported.[21]

In our case, the lesion was completely excised and no recurrence was observed.

 Conclusion



Solitary form of NLCS is rare compared to classical multiple form; hence, here, we report a rare case of pedunculated lipofibroma.

Physicians should be aware of this rare condition because early recognition enables more conservative resection of the tumor and less invasive reconstruction of the defect. Rare association with other malignancies should be remembered.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

1Abel R, Dougherty JW. Nevus lipomatosus cutaneus superficialis (Hoffman-zurhelle); report of two cases. Arch Dermatol 1962;85:524-6.
2Hoffman E, Zurhelle E. Naevus lipomatosus cutaneous superfi cialis. Arch Dermatol Syphilol 1921;130:327-33.
3Jones EW, Marks R, Pongsehirun D. Naevus superficialis lipomatosus. A clinicopathological report of twenty cases. Br J Dermatol 1975;93:121-33.
4Finley AG, Musso LA. Naevus lipomatosus cutaneus superficialis (Hoffman-zurhelle). Br J Dermatol 1972;87:557-64.
5Dotz W, Prioleau PG. Nevus lipomatosus cutaneus superficialis. A light and electron microscopic study. Arch Dermatol 1984;120:376-9.
6Park HJ, Park CJ, Yi JY, Kim TY, Kim CW. Nevus lipomatosus superficialis on the face. Int J Dermatol 1997;36:435-7.
7Chanoki M, Sugamoto I, Suzuki S, Hamada T. Nevus lipomatosus cutaneus superficialis of the scalp. Cutis 1989;43:143-4.
8Holtz KH. Histology of naevus lipomatodes cutaneous superficialis (Hoffmann-zurelle). Arch Klin Exp Dermatol 1955;199:275-86.
9Girglia HS, Bhattacharya SK. Nevus lipomatosus cutaneous superficialis. Int J Dermatol 1975;14:273-6.
10Knoth W. Naevus lipomatosus cutaneous superfi cialis Hoffman – Zurhelle. Dermatologica 1962;125:161-73.
11Moore JR, Kalus M. Nevus lipomatosus superficialis circumscriptus. Plast Reconstr Surg 1980;66:617-9.
12Robinson HM, Ellis FA. Naevus lipomatosis subepidermalis seu superficialis cutis. Arch Dermatol Syphilol 1973;35:425.
13Elder DE. Lever's Histopathology of Skin. 9th ed. Lippincott Williams and Wilkins; 2008. p. 1062-3.
14Wollina U. Photoletter to the editor-Nevus lipomatosus superficialis (Hoffmann-zurhelle). Three new cases including one with ulceration and one with ipsilateral gluteal hypertrophy. J Dermatol Case Rep 2013;7:71-3.
15Brasanac D, Boricic I. Giant nevus lipomatosus superficialis with multiple folliculosebaceous cystic hamartomas and dermoid cysts. J Eur Acad Dermatol Venereol 2005;19:84-6.
16Ioannidou DJ, Stefanidou MP, Panayiotides JG, Tosca AD. Nevus lipomatosus cutaneous superficialis (Hoffmann-zurhelle) with localized scleroderma like appearance. Int J Dermatol 2001;40:54-7.
17Nogita T, Wong TY, Hidano A, Mihm MC Jr., Kawashima M. Pedunculated lipofibroma. A clinicopathologic study of thirty-two cases supporting a simplified nomenclature. J Am Acad Dermatol 1994;31(2 Pt 1):235-40.
18Lane JE, Clark E, Marzec T. Nevus lipomatosus cutaneus superficialis. Pediatr Dermatol 2003;20:313-4.
19Dhamija A, Mehedra A, D'Souza P, Meena RS. Nevus lipomatosis cutaneous superficialis: An unusual presentation. Indian Dermatol Online J 2012;3:196-8.
20Patil SB, Narchal S, Paricharak M, More S. Nevus lipomatosus cutaneous superficialis: A rare case report. Iran J Med Sci 2014;39:304-7.
21Buch AC, Panicker NK, Karve PP. Solitary nevus lipomatosus cutaneous superficialis. J Postgrad Med 2005;51:47-8.