Unusual Presentation of naevus Lipomatosus Cutaneus Superficialis

Abstract

Sir, Nevus lipomatosuscutaneussuperficialis (NLCS) is a rare benign hamartomatous condition characterized by the presence of mature ectopic adiopocytes in the dermis. [1] A 45 year old male presented with asymptomatic swelling over the upper back since childhood. The swelling started as apea-sized lesion which gradually progressedto the present size. Newer lesions were also noticed around the primary lesion during adulthood. There was no history of trauma and no systemic complaints. Cutaneous examination revealed multiple skin coloured cerebriform plaques over the left scapular region, the largest being 13 × 8 cm with few smaller satellite papules to plaques around it. Surface was smooth and was studded by multiple comedones [Figure 1]. On palpation, swelling was soft in consistency and non tender. General physical examination and systemic examination was normal. Histopathology revealed a normal epidermis overlying fibrillarpappilary dermis containing spindle and stellate cells in the stroma. Small lobules of adipose tissue are seen in the upper dermis and mid dermis surrounded by thick collagen fibres. Mild perivascular lymphocytic infiltrate and few mast cells are seen [Figure 2]. A diagnosis of naevus lipomatosus cutaneous superficialis was made and the patient was referred to plastic surgery forsurgery. Figure 1 Multiple skin colouredcerebriform plaque surface studded with comedone like plugs Figure 2 A normal epidermis overlying fibrillarpappilarydermis with small lobules of adipose tissue are seen in the upper dermis and mid dermis (H and E, ×40) NLCS is a rare disorder characterized by collection of mature adipocytes ectopically situated in the dermis. It generally presents at birth but can appear within the first two decades of life, as in our case. [1 2] Exact pathogenesis is not known. But since theelectron microscopy shows the presence oflipocytesin close association with the capillaries, it has been suggested that they may originate from the pericytes, as in fetal lipogenesis. [3] It has two clinical presentation: A classic type and a solitary type. In the classical type, originally described by Hoffman and Zurhelle in 1921, the lesions are multiple, non-tender, soft, pedunculated, cerebriform, yellowish or skin-colored papules or nodules and are present usually at birth orwithin the first two to three decades of life. [2] The solitary type of NLCS consists of a solitary, domed papule or nodule, which develops in adult life and has no specific distribution. [4] The solitary form is thought to represent a fibroepithelial polyp containing adipose tissue. [5] Morphologically, the lesions are skin colored to yellow papules and nodules, which often coalesce into plaques varying in size. Their surface is either smooth, wrinkled, cerebriform or verrucoid and peaud’orange texture. Comedo-like plug, as seen in our case, hair, cafι au lait spots and ulceration are very rare. [6] These lesions usually remain static, although some continue to extend for many years. Lesion are present most commonly on the lower trunk, especially over the back, buttock or hips or abdomen or thighwith only a few cases occurring in the scalp, face, shoulder. [4] Rarely lesion may be confluent linear plaques along skin fold called as Micheleintyre man appearence. [5] Various differential diagnosis include plexiform neurofibroma, nevus sebaceous, connective tissue nevus, vascular malformation, lipoblastomatosis, focal dermal hypoplasia (Goltz syndrome). On histopathology, the distinguishing feature is presence of ectopic mature adipocytes in the dermis intermingled with collagen bundles and perivascular infiltration of dermis and subcutis with chronic inflammatory cells. [4] Treatment is necessary for cosmetic reason. The treatment of choice is surgical excision. This case is reported for its clinical rarity.