Inflammatory Linear Verrucous Epidermal Nevus Research Articles

Effect of secukinumab on inflammatory linear verrucous epidermal nevus with a somatic mutation in CARD14.

We report a patient who presented with erythematous plaques with scales distributed all over the body, following the lines of Blaschko. The patient was diagnosed clinically with inflammatory linear verrucous epidermal naevus. Deep next-generation sequencing of the lesion showed a somatic mutation in CARD14. The patient was treated with secukinumab and experienced significant improvement.

Inflammatory linear verrucous epidermal nevus should be genotyped to direct treatment and genetic counseling

Inflammatory linear verrucous epidermal nevus should be genotyped to direct treatment and genetic counseling

Inflammatory Linear Verrucous Epidermal Nevus – Rare Presentation in a Young Female

Inflammatory linear verrucous epidermal nevus (ILVEN) is a rare variant of epidermal nevus characterized by psoriasiform epidermal hyperplasia, pruritic, warty lesions, in a linear configuration, following the lines of Blaschko. We report a rarer incidence of ILVEN in a young adult female presented with pruritic linear focally warty lesion over dorsum of right foot since birth. Histopathological examination of excision biopsy was suggestive of ILVEN. Although generally considered benign, 15-20% ILVEN cases can show malignant transformation. Despite variable treatment options, treatment is custom-made depending upon the nature and extent of the lesion. Therefore, the correct diagnosis of ILVEN is vital for choosing early and prompt treatment planning. Keywords: Adult, ILVEN, Female, Epidermal, Nevus

Epidermal nevi and epidermal nevus syndromes in pediatric practice

Epidermal nevi are hamartomas of ectodermal origin. Epidermal nevus syndromes are defined as the presence of an epidermal nevus with the involvement of other organ systems. Epidermal nevi are a manifestation of cutaneous mosaicism. Mosaicism may result from postzygotic somatic mutations. Epidermal nevi are usually located along the Blaschkos lines. There are organoid (sebaceous nevus, comedonic nevus, eccrine nevus) and non-organoid epidermal nevus (keratinocytic epidermal nevus, inflammatory linear verrucous epidermal nevus). Epidermal nevi can be combined with extracutaneous malformations: skeletal anomalies, neurological changes, eye defects, and then it can be related with epidermal nevus syndrome. The presence in a child of large epidermal nevi and nevi located on the face and scalp requires a multidisciplinary approach involving a pediatrician, neurologist, ophthalmologist and geneticist. Currently, several well-defined epidermal nevus syndromes characterized by organoid and non-organoid (keratocytic) nevi have been described: Schimmelpennings syndrome, phakomatosis pigmentosa, Beckers nevus syndrome, comedonal nevus syndrome, angora hairy nevus syndrome, Proteus syndrome, and CHILD syndrome (congenital hemidisplasia with ichthiosiform nevus and limb defects). Thus, follow-up of patients with epidermal nevus syndromes should be individualized and include a thorough history, clinical examination, and, if necessary, genetic testing. Typically, children with small, isolated epidermal nevus do not require further evaluation.

Linear Psoriasis: A Rare and Often Underdiagnosed form of Psoriasis in Children

Blaschko-linear psoriasis (PBL) is a rare form of psoriasis that presents as longitudinal lines on the skin, along Blaschko lines. This form of psoriasis is often observed in children and can be difficult to distinguish from other acquired Blaschko linear dermatoses such as inflammatory linear verrucous epidermal nevus (ILVEN) and lichen striatus. The prevalence of PBL is unknown, but it is generally observed in children and is more common in boys than in girls. The clinical and histological similarity of PBL to ILVEN presents a diagnostic challenge. In this case report, we present the case of a 7-year-old child with PBL and discuss the diagnostic and therapeutic aspects of this rare form of psoriasis. We were able to confirm the diagnosis of PBL after treatment with white petroleum jelly and salicylic acid petroleum jelly 5% resulted in a significant improvement of the lesions. Topical treatments, phototherapy, and systemic medications can be used in the case of PBL. It is important to select the most appropriate treatment based on the severity of the lesions, the location, and the patient's tolerance, and to keep in mind that PBL can have long-term effects on the quality of life of patients and their families.

An Unusual Presentation of Lichen Striatus.

An Unusual Presentation of Lichen Striatus.

Giant inflammatory linear verrucous epidermal nevus: difficulties in the management.

Giant inflammatory linear verrucous epidermal nevus: difficulties in the management.

Inflammatory linear verrucous epidermal nevus (ILVEN) encompasses a spectrum of inflammatory mosaic disorders.

Inflammatory linear verrucous epidermal nevus (ILVEN) is a rare skin disease characterized by pruritic erythematous scaly plaques distributed along the lines of Blaschko. Two cases of ILVEN with CARD14 mutations and one case with a GJA1 mutation have been previously reported. To elucidate the genetic cause of a cohort of patients diagnosed based on clinical and histopathological evaluation with ILVEN. We recruited patients diagnosed with ILVEN based on clinical and histopathological criteria. Exome sequencing of affected skin with or without blood/saliva was performed and germline and somatic pathogenic variants were identified. Five patients were enrolled. All had skin lesions from birth or early childhood. Two patients developed psoriasis vulgaris after the diagnosis of ILVEN. The first had a germline heterozygous CARD14 mutation and a post-zygotic hotspot mutation in KRT10. The histopathologic evaluation did not show epidermolytic hyperkeratosis. The second had a post-zygotic hotspot mutation in HRAS. Her ILVEN became itchy once psoriasis developed. One patient was re-diagnosed with linear porokeratosis based on a germline mutation in PMVK and a post-zygotic second-hit mutation. Two patients were re-diagnosed with congenital hemidysplasia with ichthyosiform nevus and limb defect nevus based on germline NSDHL mutations. ILVEN is a clinical descriptor for a heterogenous group of mosaic inflammatory disorders. Genetic analysis has the potential to more precisely categorize ILVEN and permits pathogenesis-directed therapies in some cases.

Differences in clinical characteristics and lesion proteomics between inflammatory linear verrucous epidermal nevus and local verrucous epidermal nevus

BackgroundVerrucous epidermal nevus (VEN) are keratinocytic epidermal nevus that appear at birth or in early childhood. They exhibit a range of manifestations, depending on the patient's age. VEN are rarely encountered in clinical practice, and the systemic and comprehensive clinical characteristics of VEN have not been well investigated. Furthermore, the association between tandem mass tag (TMT)-based quantitative proteomics and the VEN phenotype is still unclear. ObjectivesThis study investigated the differences in the clinical characteristics and lesion proteomics between inflammatory linear VEN (ILVEN) and local VEN. MethodsThis retrospective study enrolled 125 patients with histopathologically diagnosed VEN who presented to our hospital between 2019 and 2021. We collected the clinical data of all patients with VEN using a self-designed questionnaire. The expression of proteins in VEN lesions was analyzed using TMT proteomics technology. ResultsIn total, there were 125 patients with VEN that were evaluated, including 67 (53.60%) patients with local VEN and 58 (46.40%) with ILVEN. No significant differences were found in sex, onset age, and lesion location between patients with local VEN and those with ILVEN (all P > 0.05). Significant differences were found in the onset site and pruritus scores between patients with ILVEN and those with local VEN (all P < 0.05). According to the TMT proteomics results, 89 proteins were up or downregulated with at least 1.3-fold (upregulated: 38, downregulated: 51; P < 0.05) in ILVEN lesions relative to VEN lesions. The top 10 differentially expressed proteins between ILVEN and local VEN lesions were OGN, NT5C3A, ADD1, OLFML1, DHRS1, CALML5, SAMHD1, SFRP2, SPRR1B, and SERPINB13. The upregulated proteins are mainly involved in neutrophil activation, neutrophil-mediated immunity, and p53 signaling pathway (hsa04115). The downregulated proteins are mainly involved in cellular response to cytokine stimulus, cell adhesion, Th1 and Th2 cell differentiation. In total, based on the differentially expressed proteins between ILVEN and local VEN, five pathways that may be associated with the pathogenesis of inflammation, including CAMs (P = 0.006), Th1 and Th2 cell differentiation (P = 0.017), PPAR signaling pathway (P = 0.023), Th17 cell differentiation (P = 0.024), and p53 signaling pathway (P = 0.041). ConclusionsClinical data of the patients revealed that ILVEN lesions presented with intense pruritus and inflammatory change. Differentially expressed proteins between ILVEN and local VEN are mainly involved in multiple inflammation related pathways associated with the pathogenesis mechanisms of pruritus. LimitationsThe small sample size in clinical characteristic and proteomics study is one of the most significant limitations in our study. The inflammation associated proteins and signal pathways in the pathogenesis of pruritus in ILVEN is not explored. SignificanceIn this study, we found the lesions of ILVEN patients presented with intense pruritus and inflammational change. A total of 89 proteins were up or downregulated with at least 1.3-fold (upregulated: 38, downregulated: 51; P < 0.05) in ILVEN lesions relative to VEN lesions. On the other hand, the etiology of itch in ILVEN mainly associated with inflammation, but the exact mechanisms was still unclear. We found the differentially expressed proteins between ILVEN and local VEN enriched five pathways that may be associated with the pathogenesis of inflammation and pruritus.

Benign skin neoplasms in children

There are many different types of skin neoplasms in children and most are benign. Dermatologists should be able to differentiate between the various types of benign skin neoplasms and be able to recommend optimal treatment to concerned patients. Surgical removal of benign skin neoplasms is often the only treatment option. The aim of this paper is to provide a general overview of epidermal lesions (e.g., inflammatory linear verrucous epidermal nevus [ILVEN], nevus sebaceous), tumors of the epidermal appendages (e.g. pilomatrixoma), cutaneous neoplasms (e.g. angiofibroma), fibromatoses (e.g., knuckle pads), tumors comprised of fat, muscle, or bone tissue (e.g., osteoma cutis, subungual exostosis), epidermal cysts and pseudocysts, and pathologic fibrosis reactions after dermal injury (keloid). Scientific data are scarce and only in the last decade has it been possible to perform histopathological examinations and genetic studies together with detailed phenotyping to gain new understanding of the pathogenetic mechanisms.

Verrucous epidermal nevus: diagnostic difficulties.

Along Blachko’s lines are distributed diseases with Mendelian inheritance such as incontinentia pigmenti, polygenic hereditary diseases such as psoriasis or atopic dermatitis (3), nevi such as verrucous epidermal nevus (VEN) and its inflammatory variant (1), and acquired diseases such as lichen striatus (LS). All these diseases share a condition of mosaicism, that is, they presuppose the existence of a gene mutation, often post-zygotic, that gives rise to a clone of cells different from all the others. The type of mutated gene is responsible for the different morphology of these diseases, but the distribution on the skin of the mutated clone is always the same; therefore, it does not depend on the gene, but on the time in which the mutation occurred and on the stereotyped movements of the migrations that skin cells undergo during fetal life. If the nevus is very extensive and therefore secondary to an early mutation, other organs may be involved such as the nervous system, the eye, the skeletal system, as happening in epidermal nevus syndrome (4) or even some gonadal cells with the risk that the mutation is transmitted to the zygote of the offspring thus giving rise to a generalized inherited disease (5). VEN is usually present at birth; when it is localized in hidden locations or when it initially manifests with just hyperpigmented lesions it may become evident later. However, it is exceptional that it manifests itself at 8 years as in the present case. Its earliness and its morphology are able in most cases to easily differentiate it from an acquired inflammatory condition such as LS. LS is a self-healing inflammatory skin disease, usually asymptomatic, which affects the first years of age, usually between 5 and 15 years, and is distributed linearly along Blaschko’s lines, suggesting that on the line in which it occurs there is a clone of mutated cells as a result of a post-zygotic mutation: this mutation would give the cells involved a particular susceptibility towards an exogenous agent that, upon its arrival, would cause the transient inflammatory reaction (2). There are various hypotheses on the pathogenetic mechanisms that lead to the inflammatory reaction and its spontaneous regression. The present case has posed problems of differential diagnosis between VEN and LS. The histological findings were not able to eliminate the clinical doubt, indeed they added another possible differential diagnosis, i.e. inflammatory linear verrucous epidermal nevus. The latter diagnosis was discarded due to the lack of itching and obvious clinical signs of inflammation. Only prolonged clinical observation over time resolved the diagnostic doubt thanks to the persistence of the lesions and the greater evidence of papillomatous and warty structures.

25959 Clinical and histopathologic evolution of inflammatory linear verrucous epidermal nevus in a pediatric patient

An 11-year-old girl presented with lines of “pink bumps” down her right arm, which first appeared 2 years prior and had worsened over the preceding 2-3 months. The lesions were intensely pruritic. She denied exposures or trauma to the area. She reported a history of asthma, seasonal allergies, and eczema. Physical examination revealed 50 4- to 9-mm pink-brown, flat-topped erythematous and scaly papules arranged linearly following a Blaschkoid distribution along the right dorsolateral arm, forearm, and wrist. Punch biopsy demonstrated superficial and deep perivascular and perieccrine lymphocytic inflammation, suggestive of lichen striatus (LS), which correlated with the initial clinical presentation. The patient was counseled regarding the self-limited nature of LS; however, she remained symptomatic for 2 more years with minimal response to topical or intralesional steroids. The papules became more verrucous and inflammatory, and this change, coupled with the lack of expected resolution, led to rebiopsy. Histopathology revealed areas of parakeratosis overlying an absent granular layer alternating with orthokeratosis overlying a thickened granular layer, a pattern indicative of inflammatory linear verrucous epidermal nevus (ILVEN). ILVEN is a rare form of congenital epidermal nevus that, unlike LS, may be characterized by a chronic, progressive course. Early ILVEN can mimic LS and linear hyperkeratotic lesions including linear psoriasis, linear lichen planus, and linear verruca. This case highlights the clinical and histopathological evolution of ILVEN and emphasizes the importance of subsequent biopsies for definitive diagnosis in refractory cases. Recognition of ILVEN points clinicians toward alternative treatments, including laser therapy, which is currently under consideration.

Molecular Genetic Dissection of Inflammatory Linear Verrucous Epidermal Naevus Leads to Successful Targeted Therapy

Molecular Genetic Dissection of Inflammatory Linear Verrucous Epidermal Naevus Leads to Successful Targeted Therapy

Dermatoscopic and confocal microscopy features of widespread inflammatory linear verrucous epidermal nevus.

article: Dermatoscopic and confocal microscopy features of widespread inflammatory linear verrucous epidermal nevus - Italian Journal of Dermatology and Venereology 2022 February;157(1):117-8 - Minerva Medica - Journals

Bilateral Systematized Inflammatory Linear Verrucous Epidermal Nevus Treated with Topical and Systemic Retinoids: A Case Report

Bilateral Systematized Inflammatory Linear Verrucous Epidermal Nevus Treated with Topical and Systemic Retinoids: A Case Report

A Rare Case of Bilateral Inflammatory Linear Verrucous Epidermal Nevus

Inflammatory linear verrucous epidermal nevus (ILVEN) is rare, mosaicism-induced linear inflammatory dermatoses with early childhood presentation and persistence for whole life in majority of cases. No definite gene or genetic locus has been nominated yet. Treatment is mostly aimed for relief of symptoms and for cosmetic concerns.Here, authors are reporting a case of 6-year-old girl presented with history of extremely pruritic, erythematous, linear plaques following the lines of Blaschko over all 4 limbs, trunk and flexures since infancy.Clinical impression of bilateral ILVEN was confirmed by histopathology. No systemic abnormality was detected.Bilateral ILVEN is extremely rare entity and it should be documented whenever encounter.

伴银屑病家族史的炎性线状疣状表皮痣一例

伴银屑病家族史的炎性线状疣状表皮痣一例

Off-Label Therapeutic Potential of Crisaborole

Crisaborole, a topical phosphodiesterase-4 inhibitor, was recently approved in 2016 for the treatment of mild to moderate atopic dermatitis in adults and children greater than 2 years of age. Since that time, several case reports and a small randomized controlled trial have been published regarding the off-label use of crisaborole for the treatment of other inflammatory dermatologic disorders. This paper reviews the current, albeit limited, evidence for off-label use of crisaborole for psoriasis, seborrheic dermatitis, vitiligo, and inflammatory linear verrucous epidermal nevus. Additional potential therapeutic uses for crisaborole are also postulated, based on its mechanism of action. Future studies are required to elucidate the full therapeutic potential of crisaborole; however, it is a welcome addition to the current nonsteroid topical treatments for inflammatory dermatologic disease.

Dermoscopy of Inflammatory Linear Verrucous Epidermal Nevus: Brown and Red Glomerular Structures Over a White Background as an Identifying Feature

Inflammatory linear verrucous epidermal nevus (ILVEN) is an uncommon variant of keratinocytic epidermal nevus that typically presents as linear erythematous and verrucous papules which often coalesce into plaques. ILVEN is characteristically intensely pruritic and is most commonly found unilaterally on an extremity, the trunk, or buttock in a Blaschkoid pattern. Lesions may be present as early as birth most commonly developing in early childhood before the age of five years old and persisting for months to years. Females are more often affected. We present the case of an adult patient who presented to our clinic for evaluation of her long-standing, treatment-resistant ILVEN. Dermoscopy was used to aid in the diagnosis and exhibited alternating red and brown glomerular-type structures over a white background. We propose that these findings may be added to other previously documented dermoscopic characteristics of ILVEN, as they correspond to known histopathological features of the dermatosis and may aid in non-invasive diagnosis.

Nail lichen striatus: Is dermoscopy useful for the diagnosis?

Lichen striatus is a unilateral inflammatory dermatosis that rarely affects the nail unit. When the inflammation involves the nail unit, classic lichenoid nail changes are easily detectable, more often limited to only one portion of the nail. Usually the nail dystrophy coexists with periungual skin papules following Blaschko's lines, but it could also be an isolated feature. Because a nail unit biopsy presents difficulties in execution, especially in a child, the aim of our study has been to describe the nail unit dermoscopy features of lichen striatus hoping to provide a valid aid to clinicians in the diagnosing this rare disorder. We reviewed the images of five pediatric patients with a clinical diagnosis of lichen striatus. Data about sex, age, localization, predisposing/triggering factors, and associated disorders are reported. The diagnosis of lichen striatus can be challenging as there are clinically overlapping features with related dermatoses. A common diagnostic pitfall occurs with inflammatory linear verrucous epidermal nevus and lichen planus. Involvement of only one part of the nail plate with linear longitudinal fissuring, ridging, and distal splitting, especially if seen with perionychial skin lesions, is characteristic of lichen striatus. Nail lichen striatus is rare, and there is sparse published literature on it. When the changes in lichen striatus are limited to the nail, the diagnosis may easily be missed. We therefore believe that dermoscopy is an important diagnostic maneuver, which should be integrated into the evaluation of patients with potential lichen striatus, and in particular is helpful for clinicians unwilling or unable to perform a nail unit biopsy.