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Nevus lipomatosus superficialis over neck, an atypical site
Jayanta Kr Das
Department of Dermatology, VIMS, Ramakrishna Mission Seva Pratisthan, 99, Sarat Bose Road, Kolkata 700026, West Bengal
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Das JK, Sengupta S, Gangopadhyay AK. Nevus lipomatosus superficialis over neck, an atypical site. Indian J Dermatol Venereol Leprol 2006;72:66-67
Nevus lipomatosus cutaneous superficialis (NLCS) is a fairly uncommon condition classically presenting as a clustered group of soft, fleshy, skin-colored, or yellowish nodules, usually on the buttocks, back, or abdomen. The nodules are sessile or pedunculated domed growths with a smooth, wrinkled, or cerebriform surface. They may be present at birth, but most commonly they develop during the first two decades of life. The histopathological hallmark is groups and strands of fat cells embedded in the collagen bundles of the dermis, often as high as the papillary dermis. The proportion of fatty tissue in the dermis varies greatly, and can be as high as 50%. We report on a case of NLCS at an atypical site.
A 12-year-old girl presented with multiple growths on the left side of the neck. The biggest of the lesions started about 4 years back as a small nodule on the left side of the lower part of neck, and the lesion had gradually spread to attain the present size. The smaller lesions appeared later on the adjacent area of the neck, and they too had gradually increased in size. There was history of slight but recurrent bleeding from different parts of the lesions. On questioning, the girl admitted to picking the lesions in her attempt to remove them.
On examination, there were several brownish, well-defined, soft, asymptomatic, and sessile growths with a cerebriform surface, spreading over an area of approx 10 x 7 cm2 on the left side of the neck. There were a few smaller, but similar, soft papulonodules lying in between the larger ones on the left side of the neck [Figure - 1].
General cutaneous examination as well as systemic examination was unremarkable. Routine investigations such as hemogram, blood biochemistry including serum lipid profile, and urine analysis were within normal limits. Histopathology of one of the neck lesions showed groups of mature fat cells embedded in the collagen bundles in the mid-dermis. The girl was referred to the surgery department where the lesion was excised.
NLCS is a developmental anomaly that may be present at birth, but it appears most commonly during the first two decades of life. There are two clinical forms. The classic form presents as soft, fleshy, skin-colored or yellow nodules on the trunk, usually on the lower back or buttocks, at birth or in childhood. The other form presents as a domed or sessile papule later in life and occurs over several other sites apart from the lower back. There are reports of hairy lesions as well as lesions with comedo-like plugs., They are sometimes associated with cafι-au-lait and hypopigmented macules. Unusual features such as large size, extension beyond the midline, and ulceration have been reported in one case from India. The histopathology, showing a dermal collection of adipose tissue, is characteristic. Intradermal melanocytic nevus and Goltz syndrome also show similar histopathological features, but can easily be clinically distinguished from nevus lipomatosus cutaneous superficialis.,
Our patient had a classical form of this uncommon condition at an atypical site.
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