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47-Year-Old Male with Asymptomatic Nodular Lesion

Can you diagnose this case?

Alexander K. C. Leung, MD, and Benjamin Barankin, MDSeries Editor

Signs and Symptoms

A 47-year-old man presented with an asymptomatic, slow-growing, nodular lesion on the left arm. He had first noted it approximately 7 years ago as a pea-sized lesion, and it had gradually increased to the present size. There was no history of ulceration or discharge from the lesion or trauma or insect bite in the area. The patient’s medical history was unremarkable. No other family members were affected with similar lesions.

Physical examination revealed a soft, skin-colored, nontender, well-defined, discrete, dome-shaped nodule on the lateral aspect of the left arm. No other skin abnormalities were noted. In particular, there were no comedo-like lesions, café au lait spots, or excessive hair growth on the surface of the lesion. There was no axillary lymphadenopathy. The rest of the physical examination findings were unremarkable.

What’s your diagnosis

Choose one to reveal diagnosis and discussion

Nevus lipomatosus superficialis
Keloid
Dermatofibroma
Xanthogranuloma

ANSWER: Nevus lipomatosus superficialis

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Excisional biopsy of the lesion was performed and revealed globules of mature adipocytes around blood vessels and eccrine glands in the papillary and reticular dermis, along with hyperkeratosis and acanthosis of the overlying epidermis, confirming the clinical diagnosis of nevus lipomatosus superficialis.

Nevus lipomatosus superficialis is a hamartoma that is characterized by the presence of ectopic mature adipose tissue in the dermis.1 Hoffmann and Zurhelle in 1921 were the first to report this skin anomaly.2 They described the case of a 25-year-old man with multiple congenital nodules in the left gluteal region and termed this entity naevus lipomatodes cutaneus superficialis. The condition is classified into 2 clinical types: the classical multiple type (also known as the Hoffmann-Zurhelle type) and the solitary type.3,4

EPIDEMIOLOGY

The exact incidence is not known, with the literature on this condition limited to case reports and case series. Suffice it to say, nevus lipomatosus superficialis is an uncommon condition. In a retrospective study, 8 cases were seen in an 11-year-period from 2001 to 2011 at a facility in Chandigarh, India.5 In another retrospective study, 8 cases were seen in a 14-year-period from January 1997 to December 2010 at a hospital in Tunis, Tunisia.6 The classical type is usually present at birth or may emerge in the first 2 decades of life.4,7 The solitary type, on the other hand, often appears later in life between the third and sixth decades.8,9 There is no familial or gender predilection.4,7,10,11

ETIOPATHOGENESIS

The condition is usually idiopathic, and the exact pathogenesis is not known.1,3 Theories such as mesenchymal perivascular differentiation of lipoblasts, focal heterotopic development of adipose tissue, adipose metaplasia of dermal connective tissue, and degenerative changes in dermal collagen bundles and elastic tissue causing deposition of adipose tissue in the dermis have been proposed to account for the heterotopic occurrence of adipose tissues.1,5-7,12 Some authors suggest that the ectopic adipose tissue may originate from pericytes, given that immature lipocytes containing numerous lipid droplets in close association with the capillaries can be detected using electron microscopy.1,13 Recently, deletion of 2p24 has been described, suggesting genes may have a role.8,14

HISTOPATHOLOGY

Histologically, the lesion is characterized by the presence of mature adipocytes embedded between collagen bundles in the papillary and reticular dermis, mostly around the perivascular area.7,15,16 The proportion of adipose tissue in the dermis varies greatly, usually from 10% to 50% of the lesion.17 There is no connection between the ectopic adipose tissue and subcutaneous adipose tissue.16,18 Epidermal acanthosis, obliteration of rete pegs, focal elongation of rete pegs, basket-weave hyperkeratosis, and increased basal pigmentation may also be seen.17

CLINICAL MANIFESTATIONS

Typically, the classical type presents as asymptomatic groups of multiple, soft, nontender, yellow or fleshy, skin-colored, sessile or pedunculated papules or nodules, often coalescing into plaques with a smooth, wrinkled, cerebriform surface, or with a orange-peel appearance.7,9 The distribution of the lesions may be linear, zosteriform, or segmental, or along the lines of skin folds.4,8,19-21 The lesions usually appear simultaneously but may vary in size.10,22

Giant lesions have been described.11 Khandpur and colleagues reported the case of an 18-year-old woman with a giant lesion measuring 28 × 40 cm and extending from the left upper back and flank to the anterolateral aspect of the mid thigh.11 Taş and Top reported the case of a 45-year-old man with a giant lesion measuring 50 × 50 cm on the sacral area that caused sciatic nerve compression.23 Sites of predilection include the pelvic girdle, buttocks, gluteal region, lower trunk, flank, and upper thigh.1,4,7-10 Lesions are usually unilateral and rarely cross the midline.19

The solitary type typically presents as an asymptomatic, solitary, yellowish to skin-colored, dome-shaped or sessile papule or nodule.8,9,22 The lesion has no predilection for any particular site and can develop anywhere on the body, including the scalp, neck, face, eyelid, nose, knee, axilla, arm, sole, nipple, scrotum, vulva, and clitoris, all of which are unusual sites for the classical type.1,4,8,9,24

Rarely, the 2 types of nevus lipomatosus superficialis may coexist. Sendhil Kumaran and colleagues reported the case of a 58-year-old woman who had both the classical and solitary types of the condition.5

The lesion of nevus lipomatosus superficialis is usually slow-growing or remains static in size.10,25 On the other hand, lesions close to the genital area tend to grow faster than do lesions elsewhere on the body.26

Coexisting anomalies that have been reported include perifollicular fibroma, trichofolliculoma, deep-penetration nevus, comedo-like lesions, café au lait macules, leukodermic spots, overlying hypertrichosis, calcinosis cutis, hemangioma, and angiokeratoma of Fordyce.3,4,7,12,15,20,21,27,28

DIAGNOSIS

The diagnosis is usually clinical, based on typical physical findings. The diagnosis can be aided by dermoscopy, which typically shows the presence of yellow structureless areas representing dermal adipocytes, with some of them in a perifollicular distribution around terminal hair follicles.29 The diagnosis, if necessary, can be confirmed by histopathology.

DIFFERENTIAL DIAGNOSIS

The differential diagnosis includes lipofibroma, lipoma, lipomatosis, leiomyoma cutis, angiolipoma, neurofibroma, common blue nevus, mucinous nevus, acrochordon (skin tag), nevus sebaceous, verrucous epidermal nevus, keratoacanthoma, juvenile xanthogranuloma, dermatofibroma, cylindroma, trichoepithelioma, keloid, lymphangioma, hemangioma, and focal dermal hypoplasia (Goltz syndrome).3,5,7

COMPLICATIONS

The condition can be unsightly and socially embarrassing if the lesions affect a visible area.7 Occasionally, ulceration may occur after trauma or ischemia.4,7,18,22 Foul-smelling discharge, calcinosis cutis, and perifollicular fibrosis have rarely been reported.3,4,22 Although the lesion is usually slow-growing, it can reach a large size if left untreated.10,23 Rarely, giant lesions can be complicated by compression neuropathy.23,25

COMPLICATIONS

The condition can be unsightly and socially embarrassing if the lesions affect a visible area.7 Occasionally, ulceration may occur after trauma or ischemia.4,7,18,22 Foul-smelling discharge, calcinosis cutis, and perifollicular fibrosis have rarely been reported.3,4,22 Although the lesion is usually slow-growing, it can reach a large size if left untreated.10,23 Rarely, giant lesions can be complicated by compression neuropathy.23,25

PROGNOSIS AND MANAGEMENT

The condition is benign, without systemic abnormalities or malignant potential.4,7,10 Postsurgical recurrence, although rare, has been reported.6,22 Treatment is usually not necessary apart from reassurance and watchful observation, unless the lesion is symptomatic, the diagnosis is in doubt, or there is a cosmetic concern.7,22 Complete surgical excision is the treatment of choice for the solitary lesion.6,11,22 Surgical excision allows histologic examination of the lesion. However, surgical excision may be impractical for the classical type of clustered lesions in which a large area is involved.24 Other treatment options include cryotherapy, CO2 laser therapy, electrosurgery, and, uncommonly, intralesional phosphatidylcholine and sodium deoxycholate injections.4,6,24,30

Alexander K. C. Leung, MD, is a resident physician in the Abrazo Central Family Medicine Residency Program in Phoenix, Arizona.

Benjamin Barankin, MD, is a dermatologist and the medical director and founder of the Toronto Dermatology Centre in Toronto, Ontario, Canada.

References
    1. Bairwa S, Sharma M, Sangwaiya A, Singla S, Gupta K, Yadav A. Nevus lipomatosus cutaneous superficialis with unusual presentation over the nipple. Indian J Dermatol. 2017;62(4):429-431.
    2. Hoffmann E, Zurhelle E. Über einen Naevus lipomatodes cutaneus superficialis der linken Glutäalgegend. Arch Dermatol Syph. 1921;130(1):327-333.
    3. Chopra R, Al Marzooq YM, Siddiqui FA, Aldawsari S, Al Ameer A. Nevus lipomatosus cutaneous superficialis with focal lipocytic pagetoid epidermal spread and secondary calcinosis cutis: a case report. Am J Dermatopathol. 2015;37(4):326-328.
    4. Goyal M, Wankhade VH, Mukhi JI, Singh RP. Nevus lipomatosus cutaneous superficialis—a rare hamartoma: report of two cases. J Clin Diagn Res. 2016;10(10):WD01-WD02.
    5. Sendhil Kumaran M, Narang T, Dogra S, Saikia UN, Kanwar AJ. Nevus lipomatosus superficialis unseen or unrecognized: a report of eight cases. J Cutan Med Surg. 2013;17(5):335-339.
    6. Goucha S, Khaled A, Zéglaoui F, Rammeh S, Zermani R, Fazaa B. Nevus lipomatosus cutaneous superficialis: report of eight cases. Dermatol Ther (Heidelb). 2011;1(2):25-30.
    7. Patil SB, Narchal S, Paricharak M, More SS. Nevus lipomatosus cutaneous superficialis: a rare case report. Iran J Med Sci. 2014;39(3):304-307.
    8. Bhushan P, Thatte SS, Singh A. Nevus lipomatosus cutaneous superficialis: a report of two cases. Indian J Dermatol. 2016;61(1):123.
    9. Leung AKC, Barankin B. Nevus lipomatosus superficialis on the left proximal arm. Case Rep Dermatol Med. 2017;2017:6906750. doi:10.1155/2017/6908750
    10. Jung S-T, Park H-W, Yun S-J. Giant nevus lipomatosus cutaneous superficialis with intramuscular lipomatosis. J Am Acad Dermatol. 2012;67(4):e168-e170.
    11. 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):407-408.
    12. Kim HJ, Lee MG. Nevus lipomatosus superficialis associated with deep penetrating nevus. Indian J Dermatol Venereol Leprol. 2015;81(4):410-411.
    13. Pujani M, Choudhury M, Garg T, Madan NK. Nevus lipomatosus superficialis: a rare cutaneous hamartoma. Indian Dermatol Online J. 2014;5(1):109-110.
    14. Cardot-Leccia N, Italiano A, Monteil MC, Basc E, Perrin C, Pedeutour F. Naevus lipomatosus superficialis: a case report with a 2p24 deletion. Br J Dermatol. 2007;156(2):380-381.
    15. Carvalho GSM, Cavalcanti SMM, Herênio AS, Teixeira MAG, Alencar ERB, Gonçalves SPM. Nevus lipomatosus cutaneous superficialis. An Bras Dermatol. 2016;91(5 suppl 1):114-115.
    16. Sakanoue M, Kawai K, Kanekura T. Nevus lipomatosus cutaneous superficialis associated with trichofolliculoma on the lower leg. Indian J Dermatol. 2013;58(4):330.
    17. Buch AC, Panicker NK, Karve PP. Solitary nevus lipomatosus cutaneous superficialis. J Postgrad Med. 2005;51(1):47-48.
    18. Wollina U. Photoletter to the editor: nevus lipomatosus superficialis (Hoffmann-Zurhelle): three new cases including one with ulceration and one with ipsilateral gluteal hypertrophy. J Dermatol Case Rep. 2013;7(2):71-73.
    19. Akoglu G, Dincer N, Metin A. Giant polypoid mass on thigh: a child with nevus lipomatosus cutaneous superficialis. An Bras Dermatol. 2016;91(4):554-555.
    20. Anzai A, Halpern I, Rivitti-Machado MC. Nevus lipomatosus cutaneous superficialis with perifollicular fibromas. Am J Dermatopathol. 2015;37(9):704-706.
    21. Das D, Das A, Bandyopadhyay D, Kumar D. Huge nevus lipomatosus cutaneous superficialis on back: an unusual presentation. Indian J Dermatol. 2015;60(3):296-297.
    22. Dhamija A, Meherda A, D’Souza P, Meena RS. Nevus lipomatosus cutaneous superficialis: an unusual presentation. Indian Dermatol Online J. 2012;3(3):196-198.
    23. Taş S, Top H. Giant nevus lipomatosus cutaneous superficialis with intramuscular lipomatosis caused sciatic nerve compression. J Cutan Med Surg. 2014;18(4):221-222.
    24. Sardana K, Bansal S, Garg VK, Khurana N. Treatment of nevus lipomatosus cutaneous superficialis with CO2 laser. J Cosmet Dermatol. 2017;16(3):333-335.
    25. Tuncer S, Sezgin B, Yilmaz G, Gocun PU, Kucuker I. Compression neuropathy caused by an unusual lesion: nevus lipomatosus cutaneus superficialis. Plast Reconstr Surg. 2011;127(3):e72-e74.
    26. Sato Y, Nobeyama Y, Omori Y, Nakagawa H. Female case of nevus lipomatosus cutaneous superficialis growing depending on the occurrence site. J Dermatol. 2017;44(1):107-108.
    27. Al-Mutairi N, Joshi A, Nour-Eldin O. Naevus lipomatosus cutaneous superficialis of Hoffmann-Zurhelle with angiokeratoma of Fordyce. Acta Derm Venereol. 2006;86(1):92-93.
    28. Lee WJ, Park OJ, Won CH, et al. Nevus lipomatosus superficialis with dilated hair follicles: an epithelial component in hamartomatous nevoid skin anomalies. Am J Dermatopathol. 2012;34(7):784-787.
    29. Vinay K, Sawatkar GU, Saikia UN, Kumaran MS. Dermatoscopic evaluation of three cases of nevus lipomatosus cutaneous superficialis. Indian J Dermatol Venereol Leprol. 2017;83(3):383-386.
    30. 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(1):119-121.
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