CLINICAL HISTORY:

Signs and symptoms:  A 16 year-old well developed female presents to the clinic for evaluation of a lesion on her back that has been present since she was an infant. The lesion has continued to increase in size as she has developed. The lesion is asymptomatic and the patient denies tenderness, drainage, bleeding, or color change. No one else in the family has a similar lesion. She has been healthy since birth and there is no significant family medical history reported. The patient is on no medication.

Previous Treatment:  The lesion has been manipulated by the mother multiple times trying to express material from the lesion. She has been unsuccessful at these attempts.

Other information: 

PHYSICAL EXAM:

A well-defined 3cm x 1.5cm flesh colored papillomatous plaque on the upper mid back. There were few surface comedones scattered throughout the lesion. The lesion had a soft rubbery consistency and was not fixed to deeper structures.

Fig. 1 Mid upper back

Fig. 2 Mid upper back close up

LABORATORY TESTS:

None

DERMATOHISTOPATHOLOGY:

Histological examination demonstrated slight papillomatosis as well as borderline basal layer pigmentation. The underlying dermis shows the usual fibrocollagenous tissue. Mature adipose tissue is present within the dermis between collagen bundles. No spindle cell lesion or prominent inflammation is identified.

Fig 3.

DIFFERENTIAL DIAGNOSIS:

1.   Plexiform Neurofibroma
2.   Connective Tissue Nevus
3.   Penduculated lipofibromas
4.   Nevus lipomatosus superficialis
5.   Focal dermal hypoplasia

SCROLL DOWN FOR ANSWER AND DISCUSSION.

CORRECT DIAGNOSIS:

Nevus lipomatosus superficialis

DISCUSSION:

Nevus lipomatosus superficialis (NLCS), also known as nevus lipomatosus of Hoffmann-Zurhelle, was first described in 1921.(1) This lesion presents at birth or early childhood as a flesh-colored or yellow papule with smooth or corrugated surface. This lesion favors the pelvic girdle, but has been reported in a wide variety of anatomical locations. These lesions are not usually associated with other developmental abnormalities. (2)

Two clinical variations of NLCS have been described in the literature. The first is the classic or multiple type. This appears from birth to over the next two decades of life and is a flesh-colored to yellow plaque with a smooth or corrugated surface following natural cleavage lines of the skin. The classic usually occurs on the sacral, lumbar, or pelvic girdle regions.(1-3) The second is the solitary type, which may not appear until the fifth decade of life.(4) They present as small solitary nodules similar to skin tags appear over the arms, knees, axillae, ears, and scalp.(1)

The pathogenesis of NLCS is unknown but there are several theories. These theories include; adipose metaplasia during the course of degenerative changes in dermal connective tissue, developmental displacement of adipocytes, developmental displacement of mature adipocytes from the dermal vessel walls.(1,2)

Histopathological diagnosis is made by the presence of ectopic mature adipocytes in the dermis. Varying amounts of adipose tissue may be present and routinely does not connect with the underlying subcutaneous fat. Fat can comprise anywhere from 10-70% of the lesion. Thickening of the collagen bundles, increased deeper elastic tissue, and decreased superficial elastic tissue, and increased blood vessels in the papillary dermis may also be present.(5)

TREATMENT:

The treatment of choice is surgical excision, but treatment is not necessary.

REFERENCES:

1. Khandpur S, Nagpal S, Chandra S, et. Al. Giant nevus lipomatosus cutaneous superficialis. Indian J Dermatol Venereol Lepro. 2009;74(4):407-408.

2. Lane JE, Clark E, Marzec T. Nevus lipomatosus cutaneus superficialis. Pediatr Dermatol. 2003;20(4):313-314.

3. Park M, Kim Y. A Soft Lesion on the Scrotum: A Quiz. Acta Drmato-Venerelogica. 2009;89: 549-550.

4. Ragsdale, Bruce. “Tumors with Fatty, Muscular, Osseous, and Carliagenous Differentiation.” In Levers: Histopathology of the Skin. Ed. David E. Elder, Rosaline E. Leuitsas, Bernett L. Johnson Jr. , George F. Murphy. Philidelphia, PA: Lippincott Williams and Wilkins, 2005. 1061-1107.

5. Weedon, David. “Tumors of Fat.” In Weedon Skin Pathology. Ed. Weedon, David. Elsevier Limited, 2010. 846.

Additional Comment:

© Copyright 2003-2006 by AOCD Grand Rounds

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