Nevus lipomatosus cutaneous superficialis (Hoffmann-Zurhelle) with localized scleroderma like appearance

An otherwise healthy 56-year-old woman presented with three symptomless unilateral plaques extending from the right lower lip to the submandibular region and the posterior lateral cervical area. Initially, one year previously, a solitary 2x3cm plaque appeared with irregular distinctive borders that extended progressively. Two new plaques with the same characteristics developed slowly near the initial one within six months. Their color was ivory-pale, with a hyperpigmented margin. The lesions were primarily soft but later became firm and indurated with smooth, shiny, mild scaling, and wrinkled surfaces. A unilateral distribution with no contraction of the skin over the affected area was observed (Fig. 1). There was no history of neurologic abnormalities or existence of skin lesions in other members of the family. X-Ray of the mandibular area was normal as well as electroencephalogram and magnetic resonance imaging scanning of the brain. Laboratory examinations including blood cell counts, urinalysis, routine blood chemistry, immunoserologic and endocrinic functions tests, were within normal limits or negative. Antibodies against Borrelia burgdorferi were also negative. Histology showed slightly flattened epidermal ridges, and a slight mononuclear inflammatory infiltrate around capillaries of the papillary dermis. The thickness of the dermis was considerably reduced and adipocytes were present extending up to a short distance from the epidermis. The focal presence within the dermis of thickened collagen bundles was ascertained both in hematoxylin and eosin stained sections and in sections stained with Masson's trichrome. These bundles did not encircle cutaneous appendages, but extended instead into the underlying adipose tissue, delimiting clusters of adipocytes. A Verhoeff-Van Gieson stain showed a marked reduction in the amount and caliber of elastic fibers in the dermis. All adipocytes had a unilocular cytoplasm without any nuclear abnormalities or size variation (Fig. 2a,b). These histological features were consistent with the diagnosis of nevus lipomatosus cutaneous superficialis (NLCS). The presumptive clinical diagnosis was that of a connective tissue disorder classified as localized scleroderma (morphea) or a connective tissue nevus. Plastic surgery was not considered in our case mainly because of the extent and particular location of the NLCS.