Generic selectors
Exact matches only
Search in title
Search in content
Post Type Selectors
Filter by Categories
15th National Conference of the IAOMFP, Chennai, 2006
Abstracts from current literature
Acne in India: Guidelines for management - IAA Consensus Document
Art & Psychiatry
Association Activities
Association Notes
Award Article
Book Review
Brief Report
Case Analysis
Case Letter
Case Letters
Case Notes
Case Report
Case Reports
Clinical and Laboratory Investigations
Clinical Article
Clinical Studies
Clinical Study
Conference Oration
Conference Summary
Continuing Medical Education
Cosmetic Dermatology
Current Best Evidence
Current Issue
Current View
Derma Quest
Dermato Surgery
Dermatosurgery Specials
Dispensing Pearl
Do you know?
Drug Dialogues
Editor Speaks
Editorial Remarks
Editorial Report
Editorial Report - 2007
Editorial report for 2004-2005
Fourth All India Conference Programme
From Our Book Shelf
From the Desk of Chief Editor
Get Set for Net
Get set for the net
Guest Article
Guest Editorial
How I Manage?
IADVL Announcement
IADVL Announcements
IJDVL Awards
IJDVL Awards 2018
IJDVL Awards 2019
IJDVL Awards 2020
IJDVL International Awards 2018
Images in Clinical Practice
In Memorium
Inaugural Address
Knowledge From World Contemporaries
Leprosy Section
Letter in Response to Previous Publication
Letter to Editor
Letter to the Editor
Letter to the Editor - Case Letter
Letter to the Editor - Letter in Response to Published Article
Letter to the Editor - Observation Letter
Letter to the Editor - Study Letter
Letter to the Editor - Therapy Letter
Letter to the Editor: Articles in Response to Previously Published Articles
Letters in Response to Previous Publication
Letters to the Editor
Letters to the Editor - Letter in Response to Previously Published Articles
Letters to the Editor: Case Letters
Letters to the Editor: Letters in Response to Previously Published Articles
Medicolegal Window
Miscellaneous Letter
Net Case
Net case report
Net Image
Net Letter
Net Quiz
Net Study
New Preparations
News & Views
Observation Letter
Observation Letters
Original Article
Original Contributions
Pattern of Skin Diseases
Pediatric Dermatology
Pediatric Rounds
Presedential Address
Presidential Address
Presidents Remarks
Report of chief editor
Report of Hon : Treasurer IADVL
Report of Hon. General Secretary IADVL
Research Methdology
Research Methodology
Resident page
Resident's Page
Resident’s Page
Residents' Corner
Residents' Corner
Residents' Page
Review Article
Review Articles
Revision Corner
Self Assessment Programme
Seminar: Chronic Arsenicosis in India
Seminar: HIV Infection
Short Communication
Short Communications
Short Report
Special Article
Specialty Interface
Study Letter
Study Letters
Symposium - Contact Dermatitis
Symposium - Lasers
Symposium - Pediatric Dermatoses
Symposium - Psoriasis
Symposium - Vesicobullous Disorders
Symposium Aesthetic Surgery
Symposium Dermatopathology
Symposium-Hair Disorders
Symposium-Nails Part I
Symposium-Nails-Part II
Systematic Reviews and Meta-analyses
Systematic Reviews and Meta-analysis
Therapeutic Guideline-IADVL
Therapeutic Guidelines
Therapeutic Guidelines - IADVL
Therapy Letter
View Point
What’s new in Dermatology
View/Download PDF

Translate this page into:

Letter to the Editor
doi: 10.4103/0378-6323.140330
PMID: 25201860

Classic form of nevus lipomatosis cutaneous superficialis of vulva

Nidhi Singh1 , Rashmi Kumari1 , Devinder Mohan Thappa1 , Rakhee Kar2 , Sivashankari Kulandaisamy1
1 Department of Dermatology, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, India
2 Department of Pathology, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, India

Correspondence Address:
Rashmi Kumari
Department of Dermatology, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry - 605 006
How to cite this article:
Singh N, Kumari R, Thappa DM, Kar R, Kulandaisamy S. Classic form of nevus lipomatosis cutaneous superficialis of vulva. Indian J Dermatol Venereol Leprol 2014;80:473-475
Copyright: (C)2014 Indian Journal of Dermatology, Venereology, and Leprology


Nevus lipomatosis cutaneous superficialis is a rare idiopathic hamartoma of adipocytes. We report a classic form of nevus lipomatosis which occurred on the vulva, an unusual site for this lesion. Nevus lipomatosis involving the genitalia can cause psychological trauma to the child as well as parents and hence requires treatment and counseling.

A 10-year-old girl was brought to us by her anxious parents for a polypoidal growth over the right vulva. It began as an asymptomatic, soft, hypopigmented swelling since birth which was gradually increasing in size for two years. Physical examination revealed a non-tender, well defined, yellow-colored, soft plaque measuring 15 × 9 cm over the right labium majorum, mons pubis, inguinal fold and upper medial thigh. There was a soft pedunculated polyp measuring 1.5 × 1 cm over the right labium majorum [Figure - 1]. The polyp was excised and the surrounding plaque was subjected to a skin biopsy [Figure - 2]. Histopathological examination revealed orthokeratosis, follicular plugging and acanthosis with lobules of mature adipose tissue separated by bundles of collagen in the reticular dermis [Figure - 2]. These findings were consistent with the clinical diagnosis of nevus lipomatosis cutaneous superficialis. The girl was referred to a plastic surgeon for staged excision.

Figure 1: Well-defined yellow colored soft plaque over the right labium majorum and mons pubis extending onto the inguinal fold and upper medial thigh, with a soft pedunculated polyp over the right labium majorum`
Figure 2: Lobules of mature adipose tissue separated by bundles of collagen in the reticular dermis (H and E, ×40)

Nevus lipomatosis cutaneous superficialis is a hamartoma of adipocytes, first reported by Hoffmann and Zurhelle [1] in 1921. It is histologically characterized by the presence of mature adipocytes in the dermis that lack any connection with the subcutaneous fat. [2] The pathogenesis of nevus lipomatosis is unknown and several theories including adipose metaplasia, heterotopias of adipocytes, and adipocytes originating from dermal vessel walls have been proposed. [3]

There is no gender predilection and the condition is not heritable. It consists of two clinical subtypes, the classic multiple and the solitary form. [2],[3] The classic form usually appears at birth or within the first two decades of life and presents as multiple, usually unilateral lesions. It may be present in a band-like, linear or zosteriform pattern. It has a predilection for the pelvic girdle area i.e.,buttocks and upper part of posterior thigh but can also occur on the chest, abdomen and face. It presents as multiple dome-shaped papules that are sessile or pedunculated. These usually appear simultaneously and are stable though a few papules may increase in size. The surface of nevus lipomatosis is usually smooth, as was observed in our case. But in rare cases, the surface can be wrinkled, cerebriform or have a peau d′ orange texture. The rare solitary form appears as a plaque or linear lesion in the third to sixth decade of life and has no predilection for any specific site. [3]

Our case is a classic form of nevus lipomatosis cutaneous superficialis of vulva that appears not to have been previously reported though there are reports of the solitary form of nevus lipomatosis involving the labium majorum [4] and clitoris. [5] The differential diagnosis includes lipoblastoma and congenital lipoma. [4] Lipoblastoma, a rare benign mesenchymal tumor of infancy arising from fetal adipose tissue shows lobules of immature adipocytes in a myxoid matrix. Congenital lipoma shows mature adipocytes in subcutaneous tissue unlike nevus lipomatosis cutaneous superficialis, where mature adipocytes are seen in the dermis without having any connection to the subcutaneous fat. [4]

Nevus lipomatosis cutaneous superficialis usually does not require any treatment but surgical excision or intralesional phosphatidylcholine can be tried if it involves the genitalia and causes psychological trauma to the patient. [6] Intralesional sodium deoxycholate (24 mg/ml) followed by intralesional phosphatidylcholine (50 mg/ml) has been reported to cause clinical and histological resolution but post-injection erythema, postinflammatory pigmentation and scarring have been observed. [7] The detergent action of sodium deoxycholate causes fat cell destruction and the released fatty acids are emulsified by phosphatidylcholine. [8],[9] The optimal concentration and the injection depth of these drugs is not yet established.

Hoffmann E, Zurhelle, E. Über einen Naevus lipomatodes cutaneous superficialis der linken Glutaalgegend. Arch Dermatol Syphilol 1921;130:327-33.
[Google Scholar]
Jones EW, Marks R, Pongsehirun D. Naevus superficialis lipomatosus: A clinicopathological report of twenty cases. Br J Dermatol 1975;93:121-33.
[Google Scholar]
Dotz W, Prioleau PG. Nevus lipomatosus cutaneous superficialis. A light and electron microscopic study. Arch Dermatol 1984;120:376-9.
[Google Scholar]
Nakashima K, Yoshida Y, Yamamoto O. Nevus lipomatosus cutaneous superficialis of the vulva. Eur J Dermatol 2010;20:859-60.
[Google Scholar]
Hattori R, Kubo T, Yano K, Tanemura A, Yamaguchi Y, Itami S, et al. Nevus lipomatosus cutaneous superficialis of the clitoris. Dermatol Surg 2003;29:1071-2.
[Google Scholar]
de Paula Mesquita T, de Almeida HL Jr, de Paula Mesquita MC. Histologic resolution of naevus lipomatosus superficialis with intralesional phosphatidylcholine. J Eur Acad Dermatol Venereol 2009;23:714-5.
[Google Scholar]
Kim HS, Park YM, Kim HO, Lee JY. Intralesional phosphatidylcholine and sodium deoxycholate: A possible treatment option for nevus lipomatosus superficialis. Pediatr Dermatol 2012;29:119-21.
[Google Scholar]
Rotunda AM, Suzuki H, Moy RL, Kolodney MS. Detergent effects of sodium deoxycholate are a major feature of an injectable phosphatidylcholine formulation used for localized fat dissolution. Dermatol Surg 2004;30:1001-8.
[Google Scholar]
Salti G, Ghersetich I, Tantussi F, Bovani B, Lotti T. Phosphatidylcholine and sodium deoxycholate in the treatment of localized fat: A double-blind, randomized study. Dermatol Surg 2008;34:60-6.
[Google Scholar]

Fulltext Views

PDF downloads
Show Sections