Generic selectors
Exact matches only
Search in title
Search in content
Search in posts
Search in pages
Filter by Categories
15th National Conference of the IAOMFP, Chennai, 2006
Abstract
Abstracts from current literature
Acne in India: Guidelines for management - IAA Consensus Document
Addendum
Announcement
Art & Psychiatry
Article
Articles
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
Commentary
Conference Oration
Conference Summary
Continuing Medical Education
Correspondence
Corrigendum
Cosmetic Dermatology
Cosmetology
Current Best Evidence
Current View
Derma Quest
Dermato Surgery
Dermatopathology
Dermatosurgery Specials
Dispensing Pearl
Do you know?
Drug Dialogues
e-IJDVL
Editor Speaks
Editorial
Editorial Remarks
Editorial Report
Editorial Report - 2007
Editorial report for 2004-2005
Errata
Erratum
Focus
Fourth All India Conference Programme
From Our Book Shelf
From the Desk of Chief Editor
General
Get Set for Net
Get set for the net
Guest Article
Guest Editorial
History
How I Manage?
IADVL Announcement
IADVL Announcements
IJDVL Awards
IJDVL AWARDS 2015
IJDVL Awards 2018
IJDVL Awards 2019
IJDVL Awards 2020
IJDVL International Awards 2018
Images in Clinical Practice
In Memorium
Inaugural Address
Index
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 - LETTERS IN RESPONSE TO PUBLISHED ARTICLES
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 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
Messages
Miscellaneous Letter
Musings
Net Case
Net case report
Net Image
Net Letter
Net Quiz
Net Study
New Preparations
News
News & Views
Obervation Letter
Obituary
Observation Letter
Observation Letters
Oration
Original Article
ORIGINAL CONTRIBUTION
Original Contributions
Pattern of Skin Diseases
Pearls
Pediatric Dermatology
Pediatric Rounds
Perspective
Presedential Address
Presidential Address
Presidents Remarks
Quiz
Recommendations
Regret
Report
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
Retraction
Review
Review Article
Review Articles
Revision Corner
Self Assessment Programme
SEMINAR
Seminar: Chronic Arsenicosis in India
Seminar: HIV Infection
Short Communication
Short Communications
Short Report
Special Article
Specialty Interface
Studies
Study Letter
Supplement-Photoprotection
Supplement-Psoriasis
Symposium - Contact Dermatitis
Symposium - Lasers
Symposium - Pediatric Dermatoses
Symposium - Psoriasis
Symposium - Vesicobullous Disorders
SYMPOSIUM - VITILIGO
Symposium Aesthetic Surgery
Symposium Dermatopathology
Symposium-Hair Disorders
Symposium-Nails Part I
Symposium-Nails-Part II
Tables
Technology
Therapeutic Guidelines
Therapeutic Guidelines - IADVL
Therapeutics
Therapy
Therapy Letter
View Point
Viewpoint
What’s new in Dermatology
View/Download PDF
Letter to the Editor
2009:75:4;407-408
doi: 10.4103/0378-6323.53149
PMID: 19584473

Giant nevus lipomatosus cutaneous superficialis

Sujay Khandpur1 , Smita A Nagpal1 , Subhash Chandra1 , Vinod K Sharma1 , Seema Kaushal2 , Rajni Safaya2
1 Department of Dermatology and Venereology, All India Institute of Medical Sciences, New Delhi, India
2 Department of Pathology, All India Institute of Medical Sciences, New Delhi, India

Correspondence Address:
Sujay Khandpur
Departments of Dermatology and Venereology, All India Institute of Medical Sciences, New Delhi - 110 029
India
How to cite this article:
Khandpur S, Nagpal SA, Chandra S, Sharma VK, Kaushal S, Safaya R. Giant nevus lipomatosus cutaneous superficialis. Indian J Dermatol Venereol Leprol 2009;75:407-408
Copyright: (C)2009 Indian Journal of Dermatology, Venereology, and Leprology

Sir,

Nevus lipomatosus cutaneous superficialis (NLCS), first described by Hoffman and Zurhelle in 1921, is a rare dermatosis characterised by multiple, variable-sized, flesh-colored to yellowish sessile plaques with cerebriform surface or small solitary nodules due to nevoid fatty growth within the papillary and reticular dermis. [1] We report two plaques of NLCS in an 18-year-old woman, one over the left face and the other extending from the upper back to the thigh (giant NLCS). This case is being reported for the rarity of the disorder and the large size of the lesion. As far as ascertained, the second plaque is the largest lesion being reported in the literature.

An 18-year-old woman presented with two asymptomatic, skin-colored cerebriform plaques over the left side of the face and neck and the left upper back extending to the mid thigh since 10 years of age. The face lesion was preceded by trauma. She presented to us for cosmetic reasons. There were no systemic complaints. Dermatological examination revealed two large plaques, the first 13.5cm x 4cm in size, composed of multiple skin-colored, soft, coalescent papules over the left side of the face and neck with a skin laxity below the left eyelid [Figure - 1] and the second, a large cerebriform plaque 40cm x 28cm in size, extending from the left upper back and flank to the antero-lateral aspect of the mid thigh [Figure - 2]. The overlying skin was nonhairy and normally pigmented.

Skin biopsies from both the thigh and the neck showed collection of mature adipocytes intermingled with collagen bundles in the papillary and upper reticular dermis. The adipocytes along with chronic inflammatory infiltrate were also present around the blood vessels. The histological features were compatible with nevus lipomatosus [Figure - 3]a and b. On magnetic resonance imaging, abnormal areas of fat deposition on the left side of the face, left flank and thigh in the subcutaneous plane with no infiltration of the internal organs and muscles were seen.

The patient was referred to the plastic surgery department for surgical excision.

NLCS is a rare disorder characterized by nevoid accumulation of mature adipocytes in the dermis. It often presents at birth but can appear within the first two decades of life, as in our case. [2] There is no familial or sex predilection.

Two clinical types are recognized. The classical type, reported by Hoffmann and Zurhelle, consists of multiple flesh-colored or yellowish sessile lesions with a tendency to coalesce into plaques with a smooth or corrugated surface and zonal distribution and following natural cleavage lines of the skin. They have a predilection for the pelvic girdle and sacral and lumbar regions. The second type manifests as small solitary nodules mimicking skin tags and occurring over the arms, knees, axillae, ears and scalp. They appear at a later stage of life. Occurrence over atypical sites like the nose, calf and clitoris has also been reported. In our patient, both the plaques were of the classical type, the second one being a giant NLCS, 40cm x 28cm in size. The largest size reported so far has been a 20cm x 30cm-sized bilateral cerebriform plaque extending from the lower abdomen to the left leg in a 36-year-old Brazilian woman. [3]

NLCS has been reported in association with cafι au- lait macules, leucodermic spots, hypertrichosis over the nevus, comedo-like lesions and angiokeratoma of Fordyce. [4]

The pathogenesis of NLCS is unknown and several theories have been put forth. Proposed pathogenesis include adipose metaplasia during the course of degenerative changes in dermal connective tissue, as stated by Hoffman and Zurhelle and supported by Nikolowsky, and developmental displacement (heterotopia) of adipocytes or development of mature adipocytes by mesenchymal perivascular cells present initially as primitive lipoblasts that subsequently transform into mature adipocytes. [2]

On histopathology, the distinguishing feature is presence of ectopic mature adipocytes in the dermis intermingled with collagen bundles and perivascular infiltration of dermis and subcutis with chronic inflammatory cells. [2]

This condition may be confused with plexiform neurofibroma, connective tissue nevus, vascular malformation, lipomatosis or lipoblastomatosis.

The treatment of choice is surgical excision. [5] Patients unwilling for surgery may undergo cryotherapy, which yields partial though satisfactory results. [4]

References
1.
Hoffmann E, Zurhelle E. UBER einen Naevus lipomatodes cutaneaous superficialis der linken Glutaalgegend. Arch Dermatol Syphilol 1921;130:327.
[Google Scholar]
2.
Jones EW, Marks R, Pongsehirum D. Naevus superficialis lipomatosus. A clinical and pathological report of twenty cases. Br J Dermatol 1975;93:485-8.
[Google Scholar]
3.
Knuttle R, Silver EA. A cerebriform mass on the right buttock. Dermatol Surg 2003;29:780-1.
[Google Scholar]
4.
Al-Mutairi N, Joshi A, Nour-Eldin O. Naevus lipomatosus cutaneaous superficialis of Hoffmann-Zurhelle with Angiokeratoma of Fordyce. Acta Derm Venereol 2005;86:92-3.
[Google Scholar]
5.
Lane JE, Clark E, Marzec T. Naevus lipomatosus cutaneaous superficialis. Pediatr Dermatol 2003;20:313-4.
[Google Scholar]

Fulltext Views
104

PDF downloads
46
Show Sections