Through the Lens
Nevus lipomatosus superficialis: A rare cutaneous hamartoma Meenu Pujani, Monisha Choudhury, Taru Garg1, Neha K. Madan Departments of Pathology, 1Dermatology, Lady Hardinge Medical College and associated hospitals, New Delhi, India
Nevus lipomatosus superficialis is an uncommon benign hamartomatous skin lesion defined by the presence of aggregates of mature adipose tissue among the collagen bundles of the dermis. It was first reported by Hoffman and Zurhelle in 1921.[1] We report a case of a 15-year-old girl with classic form of this rare skin malformation. A 15-year-old girl presented with a history of multiple, skin-colored, pedunculated, cerebriform, soft, asymptomatic nodules varying in size from 2 × 1 to 2 × 2 cm on the lower back for 10 years [Figure 1a]. There was no history of any neurological deficit, and general physical examination did not reveal any cafe-au-lait spots. Skin biopsy was performed and microscopy showed an unremarkable epidermis with presence of islands of mature adipose tissue in the dermis interposed among the collagen bundles and comprising more than 50% of the dermis [Figures 1b, 2 and 3].
Access this article online Website: www.idoj.in DOI: 10.4103/2229-5178.126069 Quick Response Code:
Address for Correspondence: Dr. Meenu Pujani, Department of Pathology, Lady Hardinge Medical College and associated hospitals, New Delhi, India. E-mail: drmeenupujani@ gmail.com
in the dermis. The proportion of the fatty tissue varies greatly from more than 50% to < 10% of the dermis.[2] Two clinical types are distinguished: the classical type and the solitary type. The classical type, first reported by Hoffman and Zurhelle, consists of multiple, soft, nontender, pedunculated, cerebriform, yellowish or skincolored papules or nodules usually situated on the pelvic girdle area in a zonal pattern and occur at birth or during first three decades of life.[1] The solitary form usually occurs after the age of 20 years, presents with a single nodular lesion with no particular predilection sites.[1]
After considering the above-mentioned characteristic features, a diagnosis of Nevus lipomatosus superficialis was given. Nevus lipomatosus superficialis is a rare benign idiopathic skin malformation characterized by the presence of ectopic mature adipose tissue
a
Figure 2: Microphotograph showing adipose tissue interspersed among collagen bundles [H and E, ×100]
b
Figure 1: (a) Gross photograph showing multiple, skin-colored, pedunculated, cerebriform nodules on the lower back; (b) skin biopsy revealing dermis almost replaced by islands of mature adipose tissue [H and E, 100×]
Indian Dermatology Online Journal - January-March 2014 - Volume 5 - Issue 1
Figure 3: Microphotograph showing mature adipose tissue replacing most of the dermis [H and E, ×100]
109
Pujani, et al.: Nevus lipomatosus superficialis
Clinically, the differential diagnosis includes neurofibromatosis, lymphangioma, hemangioma, skin tag or fibroepithelial polyp all of which can be distinguished histologically. Microscopically, adipose tissue in dermis is seen in focal dermal hypoplasia. However, these two entities are clinically distinct. Focal dermal hypoplasia is associated with several ectodermal and mesodermal deformities including syndactyly, hypoplasia of hair, nail, and teeth, cutaneous ulcers with thinning of skin. Microscopically, dermis is almost entirely replaced by fat cells.[3] Treatment, though not necessary, is done for cosmetic reasons and simple surgical excision appears to be curative. Lesions are generally nonprogressive and systemic abnormalities and malignant alterations have not been associated with this abnormality.[3]
REFERENCES 1. 2.
3.
Yap FB. Nevus lipomatosus superficialis. Singapore Med J 2009;50:161-2. Ragsdale BD. Tumors with fatty, muscular, osseous, and/or cartilaginous differentiation. Lever’s histopathology of the skin,10th ed. Elder DE, Elenitsas R, Jhonson BL, Murphy GF, Xu X editors. Wolters KluwerLippincott Williams & Wilkins; 2009. Park H, Park C, Yi J, Kim T, Kim C. Nevus lipomatosus superficialis on the face. Int J Dermatol 1997;36:435-7.
Cite this article as: Pujani M, Choudhury M, Garg T, Madan NK. Nevus lipomatosus superficialis: A rare cutaneous hamartoma. Indian Dermatol Online J 2014;5:109-10. Source of Support: Nil, Conflict of Interest: None declared
Research Methodology Workshops-2014 Organized by the IADVL Academy of Dermatology with the IADVL Delhi, Karnataka, Maharashtra and West Bengal state branches
Schedule, Venues and Contact Persons •
9 am-5 pm, February 15 & 16: BYL Nair Hospital, Mumbai. Contact Dr. Manish Gautam, Hon. Secretary, IADVL (MSB) (09821012573; [email protected]).
•
12.30-8 pm, February 22 & 8 am-5 pm, February 23: Command Hospital (Eastern Command), Kolkata. Contact Dr. Arghya Ghosh (09432443277, [email protected]) Dr. Manas Chatterjee (08697709387, [email protected]) or Dr. Saumya Panda (09831163975; saumyapan@gmail. com)
•
9 am-5 pm, March 8 & 9: Bangalore Medical College, Bangalore. Contact Dr. Umashankar N., Hon. Secretary, IADVL (KSB) (09448151468; [email protected], [email protected]).
•
May 3 & 4: Maulana Azad Medical College, Delhi. Contact Dr. Atul M. Kochhar, President, IADVL (DSB) (09811386390, 09868392790; [email protected]) or Dr. Pikun Gangwani, Hon. Secretary, IADVL (DSB) (09711825992; [email protected]).
Registration Fees Rs 500 (both days) or Rs 300 (one day) Dr. Deepak Parikh President, IADVL
Dr. Rashmi Sarkar Hon. General Secretary, IADVL
Dr. Manas Chatterjee Chairperson IADVL Academy of Dermatology
Dr. Ameet Valia Convener, IADVL Academy of Dermatology
Supported by an educational grant from MSD (Fulford) India Ltd. 110
Indian Dermatology Online Journal - January-March 2014 - Volume 5 - Issue 1
Copyright of Indian Dermatology Online Journal is the property of Medknow Publications & Media Pvt. Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
Nevus lipomatosus superficialis: A rare cutaneous hamartoma Meenu Pujani, Monisha Choudhury, Taru Garg1, Neha K. Madan Departments of Pathology, 1Dermatology, Lady Hardinge Medical College and associated hospitals, New Delhi, India
Nevus lipomatosus superficialis is an uncommon benign hamartomatous skin lesion defined by the presence of aggregates of mature adipose tissue among the collagen bundles of the dermis. It was first reported by Hoffman and Zurhelle in 1921.[1] We report a case of a 15-year-old girl with classic form of this rare skin malformation. A 15-year-old girl presented with a history of multiple, skin-colored, pedunculated, cerebriform, soft, asymptomatic nodules varying in size from 2 × 1 to 2 × 2 cm on the lower back for 10 years [Figure 1a]. There was no history of any neurological deficit, and general physical examination did not reveal any cafe-au-lait spots. Skin biopsy was performed and microscopy showed an unremarkable epidermis with presence of islands of mature adipose tissue in the dermis interposed among the collagen bundles and comprising more than 50% of the dermis [Figures 1b, 2 and 3].
Access this article online Website: www.idoj.in DOI: 10.4103/2229-5178.126069 Quick Response Code:
Address for Correspondence: Dr. Meenu Pujani, Department of Pathology, Lady Hardinge Medical College and associated hospitals, New Delhi, India. E-mail: drmeenupujani@ gmail.com
in the dermis. The proportion of the fatty tissue varies greatly from more than 50% to < 10% of the dermis.[2] Two clinical types are distinguished: the classical type and the solitary type. The classical type, first reported by Hoffman and Zurhelle, consists of multiple, soft, nontender, pedunculated, cerebriform, yellowish or skincolored papules or nodules usually situated on the pelvic girdle area in a zonal pattern and occur at birth or during first three decades of life.[1] The solitary form usually occurs after the age of 20 years, presents with a single nodular lesion with no particular predilection sites.[1]
After considering the above-mentioned characteristic features, a diagnosis of Nevus lipomatosus superficialis was given. Nevus lipomatosus superficialis is a rare benign idiopathic skin malformation characterized by the presence of ectopic mature adipose tissue
a
Figure 2: Microphotograph showing adipose tissue interspersed among collagen bundles [H and E, ×100]
b
Figure 1: (a) Gross photograph showing multiple, skin-colored, pedunculated, cerebriform nodules on the lower back; (b) skin biopsy revealing dermis almost replaced by islands of mature adipose tissue [H and E, 100×]
Indian Dermatology Online Journal - January-March 2014 - Volume 5 - Issue 1
Figure 3: Microphotograph showing mature adipose tissue replacing most of the dermis [H and E, ×100]
109
Pujani, et al.: Nevus lipomatosus superficialis
Clinically, the differential diagnosis includes neurofibromatosis, lymphangioma, hemangioma, skin tag or fibroepithelial polyp all of which can be distinguished histologically. Microscopically, adipose tissue in dermis is seen in focal dermal hypoplasia. However, these two entities are clinically distinct. Focal dermal hypoplasia is associated with several ectodermal and mesodermal deformities including syndactyly, hypoplasia of hair, nail, and teeth, cutaneous ulcers with thinning of skin. Microscopically, dermis is almost entirely replaced by fat cells.[3] Treatment, though not necessary, is done for cosmetic reasons and simple surgical excision appears to be curative. Lesions are generally nonprogressive and systemic abnormalities and malignant alterations have not been associated with this abnormality.[3]
REFERENCES 1. 2.
3.
Yap FB. Nevus lipomatosus superficialis. Singapore Med J 2009;50:161-2. Ragsdale BD. Tumors with fatty, muscular, osseous, and/or cartilaginous differentiation. Lever’s histopathology of the skin,10th ed. Elder DE, Elenitsas R, Jhonson BL, Murphy GF, Xu X editors. Wolters KluwerLippincott Williams & Wilkins; 2009. Park H, Park C, Yi J, Kim T, Kim C. Nevus lipomatosus superficialis on the face. Int J Dermatol 1997;36:435-7.
Cite this article as: Pujani M, Choudhury M, Garg T, Madan NK. Nevus lipomatosus superficialis: A rare cutaneous hamartoma. Indian Dermatol Online J 2014;5:109-10. Source of Support: Nil, Conflict of Interest: None declared
Research Methodology Workshops-2014 Organized by the IADVL Academy of Dermatology with the IADVL Delhi, Karnataka, Maharashtra and West Bengal state branches
Schedule, Venues and Contact Persons •
9 am-5 pm, February 15 & 16: BYL Nair Hospital, Mumbai. Contact Dr. Manish Gautam, Hon. Secretary, IADVL (MSB) (09821012573; [email protected]).
•
12.30-8 pm, February 22 & 8 am-5 pm, February 23: Command Hospital (Eastern Command), Kolkata. Contact Dr. Arghya Ghosh (09432443277, [email protected]) Dr. Manas Chatterjee (08697709387, [email protected]) or Dr. Saumya Panda (09831163975; saumyapan@gmail. com)
•
9 am-5 pm, March 8 & 9: Bangalore Medical College, Bangalore. Contact Dr. Umashankar N., Hon. Secretary, IADVL (KSB) (09448151468; [email protected], [email protected]).
•
May 3 & 4: Maulana Azad Medical College, Delhi. Contact Dr. Atul M. Kochhar, President, IADVL (DSB) (09811386390, 09868392790; [email protected]) or Dr. Pikun Gangwani, Hon. Secretary, IADVL (DSB) (09711825992; [email protected]).
Registration Fees Rs 500 (both days) or Rs 300 (one day) Dr. Deepak Parikh President, IADVL
Dr. Rashmi Sarkar Hon. General Secretary, IADVL
Dr. Manas Chatterjee Chairperson IADVL Academy of Dermatology
Dr. Ameet Valia Convener, IADVL Academy of Dermatology
Supported by an educational grant from MSD (Fulford) India Ltd. 110
Indian Dermatology Online Journal - January-March 2014 - Volume 5 - Issue 1
Copyright of Indian Dermatology Online Journal is the property of Medknow Publications & Media Pvt. Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
