Nevus Lipomatosus Cutaneous Superficialis: A Report of Two Cases.

Sir,

Nevus lipomatosus cutaneous superficialis (NLCS) is a rare cutaneous hamartoma of adipocytes that is characterized by the presence of mature adipocytes in papillary and reticular dermis. It appears in two forms; classical and solitary. Herein, we present two cases of classical NLCS one over left shoulder and other over left buttock in 22-year-old and 14-year-old patient, respectively. Shoulder involvement is not commonly reported.

Case 1 - A 22-year-old unmarried female came to us with complaint of multiple asymptomatic lesions over her left shoulder since 10 years. On clinical examination, multiple, well-defined, discrete and confluent, skin colored to yellowish, soft, sessile nodules, and plaques with cerebriform surface were present over an area of 15 cm × 5 cm over left shoulder. Comedo-like lesions were present over few nodules along with hypertrichosis of the area [Figure 1].

Figure 1

Cerebriform lesions along with comedo-like lesions and hypertrichosis

Case 2 - A 14-year-old girl was brought by her parents to us with complaint of few asymptomatic lesions over left buttock that started appearing since 4 years. On clinical examination, multiple, skin colored, discrete and confluent, soft, sessile, round, papules, and nodules were present near perianal area over the left buttock. Only few lesions had cerebriform surface. There were no associated comedo-like lesions, hypertrichosis, and cafι-au-lait or hypopigmented macules [Figure 2].

Figure 2

Multiple, skin colored soft, sessile, papules, and nodules

In both patients, rest of the mucocutaneous examination was not significant and histopathological examination revealed presence of mature adipocytes embedded within the collagen bundles in the dermis [Figure 3] along with follicular plugging in case 1 [Figure 4]. Based on the clinical and histopathological examination a final diagnosis of the classical type of NLCS was made.

Figure 3

Mature adipocytes present in the dermis embedded within the collagen bundles (H and E, ×100)

Figure 4

Follicular plugging along with mature adipocytes in dermis embedded within collagen bundles (H and E, ×100)

The classical form of NLCS, as described first by Hoffman and Zurhelle in 1921, has multiple, soft, sessile or pedunculated, skin colored to yellowish papules or nodules with cerebriform surface that are present in segmental pattern.[1] Lesion may be present since birth or may appear within first two decades of life. The most common sites are pelvic girdle, lower trunk, gluteal region, and thigh.[2] In the solitary form of NLCS, lesions appear as skin colored to yellowish, dome-shaped papule or nodule, generally later in life around third to sixth decade. It can occur over any site including scalp, eyelids, nose, and clitoris, which are unusual sites for NLCS.[2]

The exact pathogenesis of NLCS is not known. Few proposed hypotheses are; dermal adipocytes may originate from pericytes,[3] NLCS may be a connective tissue nevus,[4] and deletion of 2p24 in NLCS thus supporting role of genetic factors in development of NCLS.[5] As in our first case, NLCS is known to occur in association with comedo-like lesions and hypertrichosis over the nevus, angiokeratoma of fordyce, café-au-lait or vitiligo-like macules, hemangioma, and basal cell carcinoma.[4] Important differential diagnoses of NLCS are nevus sebaceous, plexiform neurofibroma, focal dermal hypoplasia, connective tissue nevus, angiolipoma, and lipomatosis. Treatment of choice is surgical excision and is curative.[2]

Nevus lipomatosus cutaneous superficialis is typically described to involve pelvic area or thighs. Shoulder involvement is probably underrecognized. Our two cases highlight that NLCS may occur over shoulder area apart from usual sites mentioned in the literature.