An update on Becker's nevus: Pathogenesis and treatment.

Becker's nevus (BN) manifests as a hyperpigmented, sometimes hypertrichotic plaque/patch over the chest and shoulder, and it is in the category of benign cutaneous hamartomas. BN has elongation and fusion of the rete ridge, keratotic plugging, sebaceous hyperplasia, smooth muscle hyperplasia, and hyperpigmentation of the basal/suprabasal layer histologically. This article highlights all issues involved in pathogenesis and treatment options of BN. According to current research, postzygotic ACTB mutations induce BN and Becker's nevus syndrome (BNS). Although several therapy strategies were utilized to treat the pigmentary and hypertrichotic aspects of BN, no definitive standard treatment was identified to far, and further research is needed to better educate BN care.

[Segmental overgrowth syndromes and therapeutic strategies]. / Les syndromes de surcroissance segmentaire et les stratégies thérapeutiques.

Overgrowth syndromes are a large group of rare disorders characterized by generalized or segmental excessive growth. Segmental overgrowth syndromes are mainly due to genetic anomalies appearing during the embryogenesis and leading to mosaicism. The numbers of patients with segmental overgrowth with an identified molecular defect has dramatically increased following the recent advances in molecular genetic using next-generation sequencing approaches. This review discusses various syndromes and pathways involved in segmental overgrowth syndromes and presents actual and future therapeutic strategies.

TITLE: Les syndromes de surcroissance segmentaire et les stratégies thérapeutiques. ABSTRACT: Les syndromes de surcroissance sont un groupe de pathologies caractérisées par une croissance excessive généralisée ou segmentaire. Les syndromes de surcroissance segmentaires sont principalement dus à des anomalies génétiques apparaissant durant l'embryogenèse et aboutissant à un mosaïcisme. Le nombre de patients atteints d'un syndrome de surcroissance avec une mutation identifiée a fortement augmenté grâce à des avancées récentes en génétique moléculaire, en utilisant le séquençage de nouvelle génération (NGS). Cette revue détaille les différents syndromes de surcroissance segmentaire ainsi que les voies moléculaires impliquées et les options thérapeutiques envisageables.

Nevus epidérmico verrucoso inflamatorio lineal: una revisión de la literatura / Inflammatory linear verrucous epidermal nevus: a review of the literature

El nevus epidérmico verrucoso inflamatorio lineal (NEVIL) es un tipo de nevus epidérmico queratinocítico, poco frecuente, de aparición predominante en la infancia, con preponderancia sobre el sexo femenino. Se caracteriza por la presencia de pápulas eritematosas descamativas de aspecto psoriasiforme, intensamente pruriginosas, que tienden a coalescer para formar placas que se distribuyen siguiendo las líneas de Blaschko. Suele presentarse de forma unilateral en extremidades inferiores y tiene pobre respuesta al tratamiento.

Inflammatory linear verrucous epidermal nevus (ILVEN) is a rare type of keratinocytic epidermal nevus that predominantly appears in childhood and female sex. It is characterized by the presence of psoriasiform, scaly, intensely itchy erythematous papules that tend to coalesce to form plaques that are distributed along Blaschko's lines. It usually affects the lower extremities unilaterally, and responds poorly to treatment.

Inflammatory linear verrucous epidermal nevus responsive to 308-nm excimer laser treatment.

Inflammatory linear verrucous epidermal nevus (ILVEN) is a rare cutaneous disease that presents as linear psoriasiform plaques with associated prominent pruritus. The lesions commonly pre-sent on the legs with onset during childhood. Inflammatory linear verrucous epidermal nevus typically is refractory to treatment. Therapies range from topical treatments to lasers and surgical options. It is clinically and histopathologically similar to psoriasis, suggesting it may respond to established psoriasis treatments such as the excimer laser. We report the case of an otherwise healthy 20-year-old woman with dry, pruritic, red lesions on the right leg that had been present since infancy. Biopsy revealed psoriasiform hyperplasia with a verruciform surface. Multiple topical treatments including ablative CO2 laser therapy showed no remarkable improvement. The patient was then treated with a UV 308-nm excimer laser and showed noticeable clinical improvement. Because of its clinical and histopathological similarities to psoriasis, we hypothesized that the excimer laser may be useful in the treatment of these lesions.

Somatic KRAS mutation in an infant with linear nevus sebaceous syndrome associated with lymphatic malformations: A case report and literature review.

RATIONALE: Linear nevus sebaceous syndrome (LNSS) is a rare neurocutaneous syndrome, characterized by nevus sebaceous,central nervous system (CNS), ocular and skeletal abnormalities. The present study describes KRAS somatic mosaic mutation in a case of LNSS with lymphatic malformations (LMs). PATIENT CONCERNS: A 4-month-old female with a clinical diagnosis of LNSS presented with infantile spasms, mental retardation, skull dysplasia, ocular abnormalities, congenital atrial septal defect, and LMs. DIAGNOSIS: Cervical ultrasonography revealed a 4.6 × 4.6 × 2.2cm no echo packet with clear boundary in the subcutaneous tissues of the right neck. The neck MRI indicated a cyst in the subcutaneous tissues of the right neck. Whole-exome sequencing revealed a low-level heterozygous mutation of the KRAS gene (c.35C > T; p.G12D, 19%) in the skin lesion sample. This mutation was not present in the blood samples of the patient and her parents. INTERVENTIONS: The patient received sclerotherapy with paicibanil (OK-432) injection for the cyst. OUTCOMES: Following 1 year of treatment, the patient exhibited fewer seizures. The mental and motor development was significantly improved. The patient can currently walk with assistance and speak simple words. LESSONS: LNSS is a rare, congenital neurocutaneous syndrome consisting of a spectrum of abnormalities involving the skin, central nervous system, eyes, LMs and other systems. LNSS can be caused by postzygotic somatic mutation in the RAS family of genes. Multidisciplinary evaluation and treatment is needed.

Sebaceus and Becker's Nevus: Overview of Their Presentation, Pathogenesis, Associations, and Treatment.

Nevus sebaceus (NS) and Becker's nevus (BN) are two variants of epidermal nevi. NS clinically presents as a yellowish-orange, hairless plaque on the scalp, face, or neck, while BN presents as a tan-to-brown hyperpigmented, sometimes hypertrichotic, plaque typically on the chest and shoulder. Histologically, NS displays mature or nearly mature sebaceus glands as well as acanthosis and fibroplasia of the papillary dermis. BN shows variable papillomatosis, acanthosis, and hyperkeratosis, with hyperpigmentation of the basal/suprabasal layer. While the genetic basis of NS is thought to be due to post-zygotic mutations in the Harvey rat sarcoma viral oncogene homolog (HRAS)/Kristen rat sarcoma viral oncogene homolog (KRAS) genes, the genetic basis of BN is relatively unknown and is implicated with paradominant inheritance. In some patients, NS and BN can each be associated with additional cutaneous and extra-cutaneous anomalies, ranging from benign or malignant tumors to multiple organ irregularities. Due to the wide range of possible associations, treatment for NS and BN is devised on a case-by-case basis. In this article, we review the features, etiology, and diagnosis/management of NS and BN, with a focus on associations. We also report a patient who concomitantly presents with both lesions.

Rapidly growing pigmented tumor on a scalp nevus sebaceous of a pediatric patient: Observation or excision.

A 14-year-old girl presented with a new, rapidly growing, pigmented tumor on a previously existing yellowish, verrucous plaque on the scalp. The patient received complete surgical excision. Routine histology ruled out basal cell carcinoma (BCC) and the histological diagnosis was pigmented trichoblastoma arising in nevus sebaceous (NS). It is important to define management for new lesions developing in pediatric patients with existing nevus sebaceus.

Verrucous epidermal nevus.

A 64-year-old man presented with a three-year history of an enlarging, pruritic, linear, verrucous plaque on his left lower extremity. Histopathologic examination was consistent with a verrucous epidermal nevus, which is a benign epidermal hamartoma, most commonly observed in the pediatric population. Verrucous epidermal nevi are often refractory to treatment and have high rates of recurrences, causing them to be therapeutic challenges. We review the treatment modalities reported to be effective in verrucous epidermal nevi.

Treatment of verrucous epidermal nevus: experience with 71 cases.

OBJECTIVE: The aim of this study was to evaluate the clinical results of 71 cases with verrucous epidermal nevus (VEN) treated by cryotherapy or CO2 laser. METHODS: The files and photographs of patients with VEN who were treated in the laser unit of a tertiary hospital between January 2005 and December 2011 were evaluated. Pretreatment and 12-month follow-up photographs were evaluated using a 5-point scale: 'excellent' (75-100% clearance), 'good' (50-75% clearance), 'fair' (25-50% clearance), 'poor' (<25% clearance) or 'worse'. RESULTS: Of 71 patients, 62 responded well to cryotherapy alone, and 9 facial VEN required CO2 laser treatment. Small VEN required relatively few treatments (mean 3.4) with 90% scoring 'excellent' and 10% scoring 'good'. Larger VEN required more treatments (mean 7.4) and did not respond as well, with 71% scoring 'excellent', 14% scoring 'good' and 14% scoring 'fair'. CONCLUSION: Our experience suggests that cryotherapy is the ideal treatment for small facial VEN, while ablation by CO2 laser should be considered an option for resistant facial lesions.

Inflammatory linear verrucous epidermal nevus with genital involvement.

An 18-year-old woman was admitted to our clinic complaining of pruritic lesions on her inguinal and genital areas that had been present since birth. She had previously used topical steroids and a combination of topical steroids and calcipotriol for approximately 6 months; however, the treatment was unsuccessful. Her medical history was unremarkable. On dermatologic examination, mild erythematous, lichenified, and verrucous papules occurring in a linear pattern on the right inguinal area and on the region extending from the right labium majus to the perianal area were noted (Figure). Additionally, an erythematous area with central erosion surrounded by maceration was noted on the intergluteal area. Two separate punch biopsy samples were obtained from the erythematous, lichenified, verrucous, papular lesion on the inguinal area and from the erythematous, eroded, macerated lesion on the intergluteal area. Histopathological examination of both biopsy specimens revealed a thin orthokeratotic layer and scattered parakeratotic layers, as well as papillomatosis and acanthosis of the epidermis with a slight hyperpigmentation of the basal layer. A mild, perivascular, chronic inflammatory cell infiltration was noted in the dermis. Based on the clinical and histopathological findings, the patient was considered to have inflammatory linear verrucous epidermal nevus, and cryotherapy was initiated. At the 2-week follow-up after the first application, it was observed that the itching complaint decreased substantially and the eroded lesions in the intergluteal area were re-epithelialized. On clinical follow-up, no improvement was observed in the papular component of the lesion after 4 sessions of cryotherapy. The patient voluntarily discontinued the follow-up after 4 sessions of cryotherapy.