Extensive Epidermal Nevus Research Articles

Intraoral Manifestations in a Patient with Epidermal Nevus Syndrome

To report rare findings of oral and periodontal manifestations in a patient with Epidermal nevus syndrome (ENS). The ENS describes the rare association of an epidermal nevus with abnormalities of central nervous system,ocular and skeletal abnormalities. Reports of oral involvement have been few. Also, most of the intraoral lesions have been reported in patients with nevi that do not fulfill the criteria for the diagnosis of ENS. This report describes a case of ENS that, in addition to cutaneous manifestations showed skeletal involvement and intraoral manifestations such as the extension of the nevi on the face intraorally involving the labial mucosa, hypoplasia, hypodontia of teeth and severe periodontal destruction. Patients with extensive epidermal nevi and systemic abnormalities should be suspected of having the ENS. Evaluation and management of patients with ENS requires a multidisciplinary team approach involving the dermatologist, pediatrician, ophthalmologist, neurologist, genetist, plastic surgeon and orthopedic services. Although uncommonly described in association with ENS, significant intraoral lesions do occur. Periodontal manifestations as in our patient, which to our knowledge has not been described in association with ENS so far, may also be present. Alteration of the response of periodontal tissues to dental plaque in the presence of certain systemic diseases has been reported, but not in association with ENS. Severe periodontal destruction due to exaggerated response to dental plaque was seen in the present case. Hence, emphasis on oral hygiene maintenance in such patients is essential. Patients with ENS must be evaluated periodically as they show a persistent predisposition for the development of tumors.

Systematized Epidermal Nevus with Epidermolytic Hyperkeratosis Improving with Topical Calcipotriol/Betametasone Dipropionate Combination Ointment

We present the case of a 5-year-old boy who presented with systematized epidermal nevus with evidence of epidermolytic hyperkeratosis on skin biopsy. He was otherwise well, with normal growth and development and no evidence of other organ system involvement. He had experienced only mild improvement with potent topical corticosteroids. He reported more-marked improvement after 2 months of topical calcipotriol/betamethasone dipropionate combination ointment once a day to the lesions. Extensive epidermal nevi can lead to severe cosmetic disfigurement. We recommend a trial of topical calcipotriol/betamethasone dipropionate ointment for patients with extensive epidermal nevus not amenable to surgical treatment.

Sebaceous nevus syndrome: A case report of a child with nevus sebaceus, mental retardation, seizures, and mucosal and ocular abnormalities

A 12-year-old girl presented with extensive epidermal nevi, new onset seizures, mental retardation, and oral and ocular abnormalities. We briefly review the case and epidermal nevus syndrome (ENS), which is characterized by epidermal nevi occurring in conjunction with neurologic, ocular, skeletal, and/or other system involvement.

Systematized organoid epidermal nevus with eccrine differentiation, multiple facial and oral congenital scars, gingival synechiae, and blepharophimosis: A novel epidermal nevus syndrome

Epidermal nevus syndrome is a clinically variable and genetically heterogeneous group of mosaic conditions characterized by the concurrence of extensive epidermal nevus with additional cutaneous and extracutaneous manifestations. This term groups together well-characterized clinical entities, as well as dozens of apparently unique associations, which need further delineation. We report on a 23-year-old woman presenting the previously undescribed combination of widespread eccrine proliferation, multiple facial and oral pox-like lesions, gingival synechiae, blepharophimosis, body asymmetry, and mental retardation. The patient has a healthy monozygotic twin. The eccrine proliferation is intermingled with areas of unaffected skin with a linear/segmental distribution on the limbs. The clinical presentation of such a complex phenotype fits well with the genetic mosaicism theory. The histologic findings, consisting of proliferation of immature to well-formed eccrine duct-like structures located in the deep dermis and interspersed with an abundant fibrous stroma constituted of horizontally oriented collagen fibers, seem a possible hallmark of this condition.

Epidermal nevus syndrome associated with polyostotic fibrous dysplasia, CNS lipoma, and aplasia cutis

Epidermal nevus syndrome is a rare congenital sporadic neurocutaneous disorder characterized by an epidermal nevus and various developmental abnormalities of the skin, eyes, nervous, cardiovascular and urogenital systems. We describe a patient with an extensive epidermal nevus associated with various organ abnormalities, particularly polyostotic fibrous dysplasia, central nervous system lipoma, and aplasia cutis. Our patient demonstrates the polymorphic spectrum of involvement in epidermal nevus syndrome.

Epidermal nevus syndrome and intraspinal hemorrhage

The epidermal nevus syndrome (ENS) is a rare neurocutaneous disease characterized by extensive epidermal nevi and a wide variety of abnormalities involving brain, eyes, and skeleton. Neurological symptoms in ENS include seizures, paresis, and mental retardation and are usually ascribed to hemimegalencephaly and various migration disorders. It was suggested that in some patients neurological symptoms might be secondary to vascular abnormalities. We report a case of a patient with diagnosed ENS without any primary CNS lesions, who developed paraplegia resulting from spinal cord hemorrhage. The patient presented many vascular and skeletal anomalies.

A method for pulsed carbon dioxide laser treatment of epidermal nevi

J Am Acad Dermatol 2002;46:554-6.

Hypophosphatemic Vitamin D—Resistant Rickets, Precocious Puberty, and the Epidermal Nevus Syndrome

The association of multisystem pathologic conditions and epidermal nevi, known as the epidermal nevus syndrome, includes disorders of bone, central nervous system, eye, kidney, vasculature, body symmetry, and skin. Rarely, vitamin D-resistant rickets has been observed in association with this syndrome. Precocious puberty is another rare finding associated with epidermal nevus syndrome, having been observed in 3 patients. A female infant with an extensive epidermal nevus, hypophosphatemia, and precocious puberty is described. Despite medical therapy, the patient's phosphate levels continued to be very low (0.87-0.97 mmol/L), establishing the diagnosis of hypophosphatemic vitamin D-resistant rickets. At 21 months of age, areas of the nevus were excised. Laboratory values obtained shortly after the operation showed a significant, but transient, improvement in the serum phosphate level. After a second excision, maintenance of her serum phosphate level in the range of 1.29 to 1.61 mmol/L was possible. There is evidence that epidermal nevi produce a potent phosphaturic factor. We hypothesize that limited excision debulked the nevus sufficiently to allow medical management of the hypophosphatemia. Surgical intervention should be considered for patients affected with vitamin D-resistant rickets with epidermal nevi. The cause of the precocious puberty is unknown. It may be hypothesized that the nevus released a factor that induced puberty.

Epidermal nevus syndrome: Association with central precocious puberty and woolly hair nevus

The epidermal nevus syndrome is characterized by the association of epidermal nevi with abnormalities of the skin, skeletal system, central nervous system, eyes, and cardiovascular system, as well as with malignant conditions. We describe a 2-year-old girl with an extensive epidermal nevus involving the left side of the body (nevus unius lateris) and associated with a woolly hair nevus on the left parietal area of the scalp and multiple acquired melanocytic nevi. Idiopathic central precocious puberty characterized by premature breast and pubic hair development and advanced bone age developed at the age of 20 months. A sharp increase in serum gonadotropins after a luteinizing hormone releasing hormone (LHRH) stimulation test confirmed the presence of central precocious puberty. This is the third reported case of precocious puberty associated with the epidermal nevus syndrome.

Linear sebaceous nevus syndrome.

To the Editor .—Lansky et al's 1 article on the Linear Sebaceous Nevus Syndrome may be misleading to pediatricians not familiar with the dermatological nomenclature of epidermal nevi. The authors should have referred to three fairly recent articles which would have clarified the diagnosis of the patients presented. 2-4 From the pictures presented in the article by Lansky et al, it would appear that the patients had extensive epidermal nevi, but not necessarily of the type referred to as or nevus sebaceus of Jadassohn, which has a characteristic histologic pattern and usually occurs on the head and neck as single lesions. More extensive presentations of sebaceous nevi as well as linear varieties have been reported but are uncommon. No histologic evidence for this type of nevus is presented in this paper. It would seem that the authors are describing children who fall into the type of neurocutaneous disease outlined

The Epidermal Nevus Syndrome

Nevus unius lateris, bilateral linear epidermal nevi, and ichthyosis hystrix may all be considered clinical variants of epidermal nevi. They were found in ten of 12 patients examined to be accompanied by congenital skeletal disorders. Five of these patients also had central nervous system (CNS) disease and two had other uncommon abnormalities including a conjunctival fibroma and hepatosplenomegaly. The high frequency of associated abnormalities of bone and CNS in this study as well as the frequent reports of such findings in the literature strongly suggests the possibility that extensive epidermal nevi form part of a syndrome including skeletal and CNS abnormalities. The presence of extensive epidermal nevi should be considered an indication for careful history and examination of the entire patient.