Nevus Unius Lateris Research Articles

Nevus Unius Lateris (NUL) in a Theree-Year-Old Child Treated by Tretinoin 0.025%, Desoxymethasone 0.25%, and Urea 20% Cream

Background: Linear verrucous epidermal nevus is the most frequent variant of the epidermal nevus classification. Linear verrucous epidermal nevus is characterized by the proliferation of epithelium arranged in a configuration that follows Blaschko's line. Nevus unius lateris (NUL) is a variant of the verrucous epidermal nevus, which has a unilateral distribution of lesions. Lesions are usually found at birth or in the first year of life as brown to grey verrucous papules or papillomatous plaques. The management of NUL is challenging as the results are varied and there is a high risk of recurrence. Purpose: to report a case of NUL and its management, especially in children. Case: A 3-year-old girl presented with brownish spots and multiple small lumps on the left buttock that have extended to the left leg since she was 9-days-old. On dermatologic examination, there were numerous hyperpigmented verrucous papules and plaques along the Blaschko line over the affected area. In this case, the diagnosis of NUL, is based on clinical symptoms and dermoscopy examination showed multiple large brown oval or round structures with hyperpigmented brown border. The patient was treated with combination topical therapy of tretinoin 0.025%, corticosteroid desoxymethasone 0.25%, and urea 20% cream, and the lesion improved within four weeks. Discussion: Epidermal nevus is often cosmetically disturbing. The treatment is still challenging and various, including surgical and non-surgical, but none is ideal and could potentially recur over months or years. dst.

Nevus Unius Lateris with Hemophilia: A rare case report

Latar Belakang: Nevus Unius Lateris (NUL) adalah kelainan bawaan jaringan kulit langka, ditandai distribusi lesi unilateral mengikuti garis Blaschko. Lesi ditemukan pada tahun pertama kehidupan dengan bercak coklat kekuningan, batas yang jelas, dan permukaan verukosa. Pemeriksaan multidisiplin dianjurkan untuk menegakkan diagnosis dan memberikan penatalaksanaan yang tepat. Terapi bertujuan untuk mengangkat dan menghancurkan lesi hiperkeratosis dengan pembedahan/non-pembedahan. Elektrokauter dapat menjadi pilihan untuk membantu mencegah perdarahan setelah operasi dermatologis, terutama hemofilia. Kasus: Tn. A, 24 tahun, pekerjaan wiraswasta, datang dengan keluhan utama beberapa plak yang meninggi di belakang lehernya. Keluhan pertama muncul sebagai bintik pada usia 6 tahun dan naik perlahan, dan menyebar luas. Pasien memiliki riwayat hemofilia A. Pemeriksaan dermatologis, beberapa nodul verukosa hiperpigmentasi bertangkai dengan batas teratur, garis yang jelas tersusun secara linier mengikuti garis Blaschko di daerah coli posterior. Hasil pemeriksaan dermoskopi menunjukkan pola sirkular hiperpigmentasi dengan struktur epidermis eksofitik dan pola serebriform. Pemeriksaan histopatologi menunjukkan hiperkeratosis, akantosis, papilomatosis minimal, dan deposit melanin dengan kepadatan ringan-sedang. Pasien didiagnosis dengan nevus unius lateris dan diterapi bedah listrik. Pasien diberikan antibiotik sistemik oral dan analgesik selama lima hari setelah prosedur dan disarankan untuk kontrol pada hari ketiga, dan pengangkatan jahitan dilakukan pada hari ketujuh. Kesimpulan: Beberapa perawatan medis dan bedah telah dicoba, tetapi tidak ada pengobatan yang ideal atau dapat diterima secara universal. Namun, kekambuhan dapat terjadi berbulan-bulan atau bertahun-tahun setelah pengangkatan lesi dengan metode apa pun. Terapi dengan bedah listrik dapat dipilih untuk membantu mencegah perdarahan yang mungkin terjadi setelah prosedur melalui target hemostasis dan untuk kerusakan jaringan superfisial.

Isolated nevus unius lateralis in a patient from Uganda.

Isolated nevus unius lateralis in a patient from Uganda.

Giant nevus unius lateralis: Two cases

Verrucous epidermal nevus is a common type of keratinocyte hamartoma present at birth or occurring later in life. Widespread multiple epidermal nevi (EN) may reflect genetic or chromosomal mosaicism. Blaschko has documented characteristic linear, zosteriform, unilateral or systematized patterns with streaks and swirls. They are resistant to treatment with the risk of recurrence. Giant congenital nevus unius lateralis (NUL) is known for its benign course with good supportive treatment however one particular case had a fatal neonatal outcome due to associated complications. Two interesting rare cases will be reviewed here.

Adult Onset of Nevus Unius Lateris

Dear Editor: A 48-year-old Korean female patient complained of brownish papules with verrucous surface, and a linear distribution. She had recognized cutaneous lesions about 15 years previously, and these lesions became more remarkable in coloration and linear distribution for the past year. The skin lesions seemed as a linear pattern following the lines of Blaschko, involving the right leg, which were extended to same side of her sole manifest as linear distributed, verrucous, and hyperkeratotic plaques (Fig. 1). She had no past medical history and family history. Histopathological examination from the right calf showed hyperkeratosis, acanthosis and hyperpigmentation of the basal layer (Fig. 2A). The histopathology from her ipsilateral sole showed verrucous surfaced epidermal alterations which were represented by definite hyperkeratosis, epidermal acanthosis, parakeratosis, and rete ridge elongations (Fig. 2B). She was diagnosed with nevus unius lateris. She had a surgical excision of the plantar lesion subsequently reconstruction with a skin graft. Alternatively, topical treatment with retinoic acid and topical corticosteroid were applied on her right leg lesions. Fig. 1 (A, B, C) The skin lesions appeared in a linear fashion, following the lines of Blaschko, involving primarily the right leg, which were extended to ipsilateral sole. Right sole manifest as yellowish, thick, linear distributed, verrucous, and hyperkeratotic ... Fig. 2 (A) A biopsy specimen from the right calf shows hyperkeratosis, acanthosis and hyperpigmentation of the basal layer (H&E stain, ×100). (B) Histology performed on a biopsy specimen from a keratotic lesion on the sole reveals verruciform ... Epidermal nevus is a mosaic disorder as a result of somatic mutations that have occurred during fetal life1. Epidermal nevus has a tendency to follow the Blaschko lines. Eighty percent of lesions appear within the first year of life, with the majority of lesions appearing by age 14 years. Our patient recognized her leg and sole lesions in her thirties. Such late-developing epidermal nevi probably represent lesions that have always been present sub-clinically, but recent growth resulted in clinical recognition. Congenital lesions tend not to expand significantly, and the majority of epidermal nevi remain quiescent after adolescence. But, in our patient, her lesions were progressed, and became more remarkable in coloration and linear distribution. The histopathologic features of epidermal nevi are described as acanthosis, hyperkeratosis, and papillomatosis. The rete ridges are elongated, and focal thickening of the granular layer and parakeratotic columns are seen2. Frequently, hyperpigmentation in the basal cell layer is evident. This corresponds with the brownish skin lesions. In addition, the margins of skin lesions are sharply demarcated from the surrounding normal skin on microscopy3. This correlates with well-described papules with distinct demarcations. Linear epidermal nevus that is clinically indistinguishable from inflammatory linear verrucous epidermal nevus (ILVEN) may have different histologic appearances. ILVEN has distinguishing histopathology which are hyperkeratosis, parakeratosis (parakeratotic areas alternate with orthokeratotic), slight spongiosis and lymphocytic exocytosis4. Treatment of linear epidermal nevus depends on surgical excision, extending into the deep dermis to prevent a recurrence. Unfortunately, this is not always possible in some cases of extensive involvement. In these instances, other treatment modalities are available to treat these lesions. Laser ablation, cryotherapy, electrofulguration and variable degree of chemical peels may offer partial or full destruction of lesions. Although topical corticosteroids, topical retinoids, and calcipotriene offer little improvement, these medications can be used as an adjunctive treatment to increase the efficacy of the surgical intervention5. Our patient has no recurrence after excision of plantar lesion. And her leg lesions remain quiescent with topical corticosteroids and topical retinoids.

CLOVE Syndrome with Nevus Unis Lateris: Report of a Case

Congenital lipomatous overgrowth, vascular malformations, and epidermal nevi (CLOVE) syndrome is a recently described syndrome similar to Proteus syndrome but lacking the progressive or distorting bony overgrowth of Proteus syndrome. We describe a neonate with features of CLOVE syndrome and nevus unius lateris.

Review of linear epidermal nevus with oral mucosal involvement – series of five new cases

Linear epidermal nevi are sporadic hamartomatous alterations of the epidermis and superficial dermis that clinically appear as verrucous papules and plaques distributed in a linear pattern following Blaschko's lines. Their extent varies from unilateral involvement (nevus unius lateris) to extensive bilateral involvement (ichthyosis hystrix). Oral mucosal lesions have rarely been described. We review the literature, focusing on the rare intraoral manifestations of linear epidermal nevus. We present a series of five new cases with oral mucosal involvement. Four cases had associated cutaneous lesions and one case had oral lesions exclusively. Histopathologic evaluation of lesional tissue in four cases showed hyperkeratosis, acanthosis, epithelial hyperplasia, and papillomatosis. Dental abnormalities, consisting of enamel hypoplasia and congenitally missing teeth, were noted in one patient adjacent to the oral lesions.

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.

Management of linear verrucous epidermal nevus with topical 5-fluorouracil and tretinoin

Linear verrucous epidermal nevus (LYEN) is an uncommon cutaneous hamartoma first described by von Baerensprung in 18631 and later reported by Unna in 1896.2 It has since been given a variety of names, including epidermal linear nevus, ichthyosis hystrix, ichthyosis linearis neuropathica, nevusunius lateris, and inflammatory linear verrucous epidermal nevus.I? Three types of LYEN exist and include the localized, systemic, and the inflammatory linear verrucous varieties. Surgical and nonsurgical management of these nevi has proved difficult, even in localized cases. We report marked improvement of a large LYEN with a combination of topical tretinoin (Retin-A) 0.1% cream and 5% 5-fluorouracil (5-FU) (Efudex) cream under occlusion.

Nevus unius lateris and intraoral verrucous nevi

A case of nevus unius lateris and two cases with similar intraoral lesions but no evidence of cutaneous involvement are described. The terminology of papillary intraoral epithelial nevi is discussed, and it is suggested that lesions of this type, confined solely to the mouth, should be called verrucous nevi.

Ｅｐｉｄｅｒｍａｌ Ｎｅｖｕｓ Ｓｙｎｄｒｏｍｅ

A seven year old male child had epidermal nevus syndrome manifesting as delayed mile stones, micro-cephaly, short stature, central nervous system abnormalities like ocular palsies with nystagmus, cutaneous manifestation of nevus unius lateris and a bone cyst at the lower end of right femur.

Unilateral, Linear, Zosteriform Epidermal Nevus With Acantholytic Dyskeratosis

An infant has a nevus unius lateris with the histopathologic condition of acantholytic dyskeratosis. Similar cases have, in the past, been classified as unilateral Darier's disease. Since it is now recognized that the pathologic changes of acantholytic dyskeratosis are not specific for a given disease entity, we believe the concept of unilateral keratosis follicularis deserves reevaluation.

Epidermal nevus syndrome: Report of a case with unusual oral manifestations

A case of epidermal nevus syndrome in a 10-year-old Negro boy is reported. Oral features are described which have not been reported previously. The clinical and histologic findings would seem to indicate nevus unius lateris, but the associated findings could also suggest nevus sebaceus of Jadassohn as part of a neurocutaneous syndrome. There is simultaneous involvement of the scalp, neck, face, eyes, buccal mucosa, tongue, maxillary alveolar mucosa, and gingiva. The teeth are hypoplastic.

Zosteriform Porokeratosis of Mibelli

Porokeratosis of Mibelli in a zosteriform distribution occurred in two girls. Although this has been previously described, the true incidence is not known. The diagnosis is most confused with lichen striatus, keratosis follicularis, nevus unius lateris, or other epidermal nevi.

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.

Nevus Unius Lateralis, Brain Tumor, and Diencephalic Syndrome

A patient with nevus unius lateralis, brain tumor, and diencephalic syndrome of childhood is presented. The diencephalic syndrome is a rare condition occurring in very young children manifested by failure to thrive and emaciation. It is almost invariably caused by an astrocytoma of the anterior hypothalamus. While there are many dermatological conditions associated with neurological abnormalities, an association between nevus unius lateralis and a neurological disorder has not previously been reported. Whether this case represents such an association or merely the chance occurrence of these two entities in the same patient is unknown at this time.

Nevus unius lateralis, brain tumor, and diencephalic syndrome

Nevus unius lateralis, brain tumor, and diencephalic syndrome

VARIATIONS OF CONGENITAL. ICHTHYOSIFORM ERYTHRODERMA. REPORT OF CASES OF ICHTHYOSIS HYSTRIX AND NEVUS UNIS LATERIS.

Microscopic examination of biopsy specimens of four patients with ichthyosis hystrix and one of nevus unius lateris showed a pattern characteristic of congenital ichthyosiform erythroderma. Based on these cases and on some evidence in the literature it is our belief that, in some instances, ichthyosis hystrix and, at times, nevus unius lateris are abortive forms of congenital ichthyosiform erythroderma.

Lichen striatus; a study of fifteen new cases with special emphasis on the histopathological changes and a review of the literature.

The incidence of lichen striatus is so rare that when such a condition occurs we are more prone to discard it for one of the other commoner linear dermatoses, such as lichen planus, nevus unius lateris, linear psoriasis, and even herpes zoster. It is easily understandable that such confusion may occur when one realizes that, in spite of the statements by different authors, 1,2 a clear-cut clinical picture does not exist; that the therapeutic response is, as we shall see later, of no help at all, and that the histopathologic changes occurring in lichen striatus are still a confusing and undecided factor. The diagnosis of lichen planus and psoriasis does not present any difficulty in cases where a widespread eruption is present, but we may also be confronted with a combination of lichen striatus and lichen planus, as the case described by H. Pinkus 3 has clearly

Failure of dermabrasion in nevus unius lateris.

From The Department of Dermatology and Syphilology, College of Medicine, University of Cincinnati. It seems natural that with the initial interest and overenthusiasm in dermabrasion that this technique would be used, even when not indicated, for many different dermatological lesions. Rattner and Rein 1 have emphasized the basic value and limitations of this technique. An effort was made to study the effect of dermabrasion in nevus unius lateris, both in early childhood and in the adult, since Kurtin * believed this technique should be limited to adult form of nevus unius lateris. Three extensive cases, two children, under general anesthesia, and one adult, were treated by means of dermabrasion. All the patients showed recurrence of the lesions a short time after the operation. It is evident that portions of the pilosebaceous apparatus (and the epidermis?) remaining after the operation are adequate to serve as a stimulus for regeneration of this