Nevus lipomatosus cutaneous superficialis: An unusual presentationDepartment Of Dermatology, Venereology, and Leprosy, R.G. Kar Medical College, Kolkata, India. email@example.com
Sudip Kumar Ghosh MD DNB, Debabrata Bandyopadhyay MD, Nazmus Saadat Jamadar MBBS DDVL
Dermatology Online Journal 16 (7): 12
Nevus lipomatosus cutaneous superficialis (NLCS) is an uncommon benign hamartomatous condition characterized by the presence of mature ectopic adiopocytes in the dermis. Clinically they are classified into two forms. The classical form is characterized by groups of multiple, non-tender, soft, pedunculated, cerebriform, yellowish or skin-colored papules, nodules, or plaques. The other form of NLCS clinically manifests as a solitary dome-shaped or sessile papule. The classical NLCS is mostly reported to involve the pelvic or gluteal region. We report here a case of adult-onset classical NLCS on the pinna because of its rarity and unusual location. In addition, our patient also had some rare features of NLCS, such as onset in the latter part of life, presence of foul-smelling discharge, and comedo-like plugs on the lesions.
Nevus lipomatosus cutaneous superficialis (NLCS) is a rare benign hamartomatous condition characterized by the presence of mature ectopic adiopocytes in the dermis [1, 2]. They are usually present at birth or emerge during the first two decades of life . There is no gender predilection or hereditary predisposition of this disease . Clinically they are classified into two forms. The classical form , originally described by Hoffman and Zurhelle in 1921, is characterized by groups of multiple, non-tender, soft, pedunculated, cerebriform, yellowish or skin-colored papules or nodules [1, 2]. They often coalesce to form a plaque. The other form  of NLCS clinically manifests as a solitary dome-shaped or sessile papule. This solitary form is thought to represent a fibroepithelial polyp containing adipose tissue . The classical NLCS is mostly reported to involve the pelvic or gluteal region , but can also rarely occur on the abdomen, chest, and face . We report here a case of adult-onset classical NLCS on the pinna because of its rarity and unusual location. In addition, our patient also had some rare features of NLCS.
A 51-year-old man presented with a gradually increasing swelling on his left pinna for the preceding 15 years. He gave a history of occasional foul-smelling discharge from the lesion. There was no history of any skin change over the pinna before the appearance of the present swelling.
|Figure 1||Figure 2|
|Figure 1. Skin-colored, cerebriform plaque on pinna
Figure 2. Multiple open comedones on the surface of the lesion
Examination revealed an oblong-shaped, yellowish to skin-colored, soft, cerebriform plaque, measuring 8 cm x 5 cm, on his left pinna (Figure 1). The surface of the lesion was studded with multiple open comedones (Figure 2). There was no tenderness, ulceration, or induration. A few discrete, soft, yellowish, papules were also scattered around the lesion. Neither regional lymphadenopathy nor other cutaneous abnormalities were present. Systemic examination was non-contributory. Routine laboratory examination was normal. Histopathological examination (Figures 3 and 4) of the incisional biopsy specimen showed irregular acanthosis and follicular plugging of the epidermis. Skin appendages were preserved. Aggregates of mature adipocytes were seen around the dermal blood vessels, eccrine glands, and between the collagen bundles.
|Figure 3||Figure 4|
|Figure 3. Aggregates of mature adipocytes were seen around the dermal blood vessels (H&E, x100)
Figure 4. Aggregates of mature fat cells are seen around the dermal blood vessels (H&E, x400)
Based on the clinical and histopathological features, a diagnosis of the classical variety of NLCS was made. The patient was referred to the plastic surgery department for surgical excision.
The classical form of NLCS is usually unilateral and may be band-like, linear, or zosteriform in distribution . When there are multiple papules, they usually appear simultaneously and vary in size. Although some lesions continue to progress for many years, they usually remain static, once formed. Occasionally, they may ulcerate, probably owing to external trauma or ischemia . Foul-smelling discharge, as seen in our patient, has rarely been reported .
There are sporadic case reports of coexistent anomalies in the form of café-au-lait macules and scattered leukoderma . Increased hairiness and comedo-like lesions  on the surface of the NLCS have also been reported, though rarely. Our patient also had comedo-like lesions. The classic variety of NLCS is either present at birth or can arise at any other time within the first two decades of life . Onset of classical NLCS in the fourth decade as seen in our case has been reported very rarely .
The histopathology of NLCS usually shows a normal or slightly attenuated epidermis associated with a dermal proliferation of mature adipocytes in the reticular dermis that may extend to the papillary dermis. The adipocytes most commonly form small aggregates around blood vessels or eccrine glands, but may also be present as solitary adipocytes between collagen bundles. The proportion of the dermal fat is variable, ranging from less than 10 percent of the dermis to over 50 percent . The adipocytes may show connections to the underlying subcutaneous fat or be separated from the subcutis by collagen. Less commonly, spindle cells representing immature fat cells may also be present.
Nevus lipomatosus cutaneous superficialis should be differentiated from nevus sebaceous, skin tags, neurofibroma, lymphangioma, hemangioma, and focal dermal hypoplasia (Goltz syndrome). Histopathological evaluation usually helps in the differentiation. No fat cells are present in the dermis in the case of skin tags. Similar dermal collections of adipocytes on histopathological examination are also present in some melanocytic nevi, pedunculated lipofibromas, and in Goltz syndrome. Lipofibromas contain fat cells, but no skin appendages, in the dermis. In the case of Goltz syndrome there is an absence of collagen in the atrophic dermis and skin appendages are absent.
The precise etiopathogenesis of NLCS is not understood. There is no specific explanation behind the predilection of the lesions for the pelvic area . Deposition of adipose tissue may be caused by the degenerative changes in dermal collagen and elastic tissue [3, 10]. Proposed pathogenesis of NLCS includes adipose metaplasia in dermal connective tissue, developmental displacement of adipose tissue; alternatively, the lesions could be explained by the possible origin of adipocytes from the pericytes of dermal vessels .
Treatment is not necessary other than for cosmetic reasons . Systemic abnormalities and malignant changes have not been associated with NLCS. Excision is curative and recurrence after surgery is rare . In our case, excision was not done because the patient was unwilling to undergo any surgical procedure. The occurrence of classical NLCS on the pinna has been unreported previously. Our case also highlights some of the rare features of classical NLCS, like onset in the latter part of life, presence of foul-smelling discharge, and comedo-like plugs on the surface.
References1. Yap FB. Nevus lipomatosus superficialis. Singapore Med J. 2009; 50(5):e161-2. [PubMed]
2. Buch AC, Panicker NK, Karve PP. Solitary nevus lipomatosus cutaneous superficialis. J Postgrad Med. 2005; 51(1):47-8. [PubMed]
3. Metin A, Calka O, Akpolat N. Nevus lipomatosus cutaneous superficialis (Hoffmann-Zurhelle). Eastern J Med 2001; 6(1):26-8.
4. Atherton DJ, Moss C. Naevi and other developmental defects. In Burns T, Breathnach S, Cox N, Griffiths C. eds. 7th ed. Blackwell Sciences, Oxford.2004; 15.1-15.114
5. Brenn T. Neoplasms of subcutaneous fat. In: Wolf K, Goldsmith LA, Katz SI, Gilcrest BA, Paller AS, Leffel DJ eds. Fitzpatrick’s Dermatology in General Medicine.7th ed. Mcgraw-Hill, New York. 2008; 1190-98
6. Nevus lipomatosus cutaneous superficialis with perifollicular fibrosis. Takashima H, Toyoda M, Ikeda Y, Kagoura M, Morohashi M. Eur J Dermatol. 2003; 13(6):584-6. [PubMed]
7. Dhar S, Kumar B, Kaur I. Naevus lipomatosus superficialis of Hoffman and Zurhelle. Indian J Dermatol Venereol Leprol 1994; 60(1):39-40
8. Khandpur S, Nagpal SA, Chandra S, Sharma VK, Kaushal S, Safaya R.Giant nevus lipomatosus cutaneous superficialis. Indian J Dermatol Venereol Leprol. 2009; 75(4):407-8. [PubMed]
9. Ragsdale BD. Tumors with Fatty, Muscular, Osseous, and/ or Cartilaginous Differentiation. In: Lever’s histopathology of the skin (Elder DE, Elenitsas R, Jhonson BL, Murphy GF, Xu X eds), 10th ed. Wolters Kluwer- Lippincott Williams & Wilkins, New Delhi. 2009:1057-1106
10. Thappa DM, Sharma RC, Lal S, Logani KB. Naevus lipomatosus cutaneous superficialis : Report of 2 cases. Indian J Dermatol Venereol Leprol 1992; 58(1):27-9
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