Linear Verrucous Research Articles

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

Neonatal Tuberous Sclerosis: is skin really the window?

We report a case of a neonate who was diagnosed to have intracardiac mass as a fetus and presented with white linear papules that was diagnosed to be Linear Verrucous Epidermal Nevus. Apart from the intracardiac mass, most likely rhabdomyoma, MRI Brain also showed presence of tubers and the Next Generation Sequence Analysis confirmed the diagnosis of Neonatal Tuberous Sclerosis. The neonate remained asymptomatic and was discharged home and remains under close follow up without any symptoms. It is thus suggested that Linear Verrucous Epidermal Nevus, a cutaneous manifestation not described with Tuberous Sclerosis could be considered under the ever-expanding cutaneous signs of Tuberous Sclerosis and should alert the physician toward its possibility.

A clinical study on linear dermatoses at a teriary care teaching hospital in Davangere

<p class="abstract"><strong>Background:</strong> Skin lesions present with innumerable patterns like discoid, petaloid, arcuate, annular, polycyclic, livedo, reticulate, target, stellate, digitate, linear, serpiginous and whorled. Most of the linear lesions follow the Blaschko’s lines. Aim was to study the incidence of linear dermatoses, the age and sex incidence, various types of clinical presentation, various sites of distribution and their clinical correlation. </p><p class="abstract"><strong>Methods:</strong> Detailed history including family history, History of disease documented with clinical examination. After counselling and after recording their consent for the test, skin biopsy along with routine investigations was done wherever needed and the results were evaluated. <strong></strong></p><p class="abstract"><strong>Results:</strong> Lichen striatus was seen in 29.91% followed by linear herpes zoster in 24.7%, linear lichen planus in 18.22%, linear verrucous epidermal nevus in 14.01%, segmental vitiligo in 3.73%, nevus depigmentation in 2.33% and others. Maximal cases were asymptomatic and reported for cosmetic reason. Intense itching was the main reason to bring the lichen planus patients and few cases of the lichen striatus patients for treatment. </p><p class="abstract"><strong>Conclusions:</strong> Lichen striatus was found to be more common, female preponderance. Majority of patients showed unilateral distribution more on the extremities. Histopathological correlation shows the importance of histopathology which ultimately changes the management in any given condition.</p><p> </p>

A rare association of aplasia cutis congenita with ipsilateral linear verrucous epidermal naevus

Aplasia cutis congenita (ACC) refers to congenital absence of skin occurring alone or in association with other anomalies. Congenital epidermal naevi are benign naevi of epidermal component present at birth or appearing in the first few years of life in characteristic developmental pattern. We are reporting the case of a 9-month-old female baby born of a non-consanguineous marriage reported to the department of dermatology for loss of skin over the right side of the scalp and lesions over the right upper limb since birth. Systemic examination did not reveal any abnormalities. The mother's antenatal history was uneventful. On examination erythematous, thin parchment-like hairless scar with crusting is present over the right frontoparietal region of the scalp. Hyperpigmented verrucous papules with surrounding erythematous skin present over right axilla, dorsum of right ring finger and hypopigmented macules present over extensor aspect of right forearm in blaschkoid distribution. According to modified Frieden's classification, aplasia cutis has been categorised into nine types. Around 86% of cases are seen over the vertex of the scalp. In the third type, ACC is associated with epidermal naevi. This is an unusual association, hence being reported here.

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.

Linear verrucous epidermal nevus with oral manifestations: report of two cases

Linear verrucous epidermal nevi (LVEN) are characterized by verrucous papules often coalescing into well-demarcated skin-colored or brown plaques following the lines of Blaschko. We present two new cases of LVEN with oral mucosa involvement and briefly discuss this very rare finding. In both cases, oral biopsies showed hyperkeratosis, acanthosis, and papillomatosis. Although several treatment modalities have been reported for the cutaneous lesions, there is no consensus for the management of oral lesions so far.

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.

314 Molecular genetic dissection of ILVEN leads to successful targeted therapy

Inflammatory linear verrucous epidermal naevus (ILVEN) is a childhood disease characterised by Blaschko-linear erythematous scaly skin. It is highly pruritic, causes distressing disfigurement, and is so notoriously treatment-resistant that it forms part of the diagnostic criteria. The cause is unknown. Using deep next generation whole exome sequencing of affected skin and blood we discover here mosaic mutations in gene CARD14 as the cause of ILVEN in two patients with treatment-resistant disease. Dramatic clinical improvement was seen with IL12/IL23-inhibitor Ustekinumab, chosen as a direct result of the genetic finding, underlining the power of molecular characterisation alongside clinical 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.

Laser treatment of epidermal nevi: A multicenter retrospective study with long-term follow-up

Patients with epidermal nevi strongly demand cosmetic improvement. Laser treatment appears appealing and is frequently used in clinical practice. Nevertheless, large series with long-term follow-up are missing, preventing definitive conclusions about its real benefit. To evaluate the long-term effectiveness and safety of lasers for epidermal nevi. Bicentric, retrospective, cohort study, including all patients treated with a laser for an epidermal nevus with more than a 1-year follow-up. Seventy patients were treated for different types of epidermal nevi, mostly with ablative lasers: 23 verrucous epidermal nevi, 16 nevi sebaceous, 26 Becker nevi, 2 inflammatory linear verrucous epidermal nevi, 1 smooth-muscle hamartoma, 1 rounded and velvety epidermal nevus, and 1 nevus lipomatosus superficialis. The follow-up period was a median of 37months (range, 12-127months). Better results, fewer recurrences, and higher patient satisfaction were noted in treatments for verrucous epidermal nevi than for nevi sebaceous. Q-switched lasers failed to show any degree of improvement in almost all patients with Becker nevus. The retrospective nature of the study. Ablative lasers can treat verrucous epidermal nevi with good long-term esthetic results but have limited long-term efficacy for nevus sebaceous. Q-switched lasers failed to improve Becker nevi.

Generalized ILVEN or Blaschkoid Psoriasis: A Persistant Dilemma

A 24-year-old man presented with itchy verrucous, scaly red-brown papules, & linear plaques distributed in a blaschkoid pattern all over the body since last 20 years associated with itching. A skin biopsy was consistent with ILVEN (inflammatory linear verrucous epidermal nevus). Histopathologically, it can be difficult to distinguish it from linear psoriasis. It may respond to conventional anti-psoriatic therapies like potent topical corticosteroids applied under occlusion, or systemic treatments like acitretin.

Connexin43 mutations linked to skin disease have augmented hemichannel activity

Mutations in the gene (GJA1) encoding connexin43 (Cx43) are responsible for several rare genetic disorders, including non-syndromic skin-limited diseases. Here we used two different functional expression systems to characterize three Cx43 mutations linked to palmoplantar keratoderma and congenital alopecia-1, erythrokeratodermia variabilis et progressiva, or inflammatory linear verrucous epidermal nevus. In HeLa cells and Xenopus oocytes, we show that Cx43-G8V, Cx43-A44V and Cx43-E227D all formed functional gap junction channels with the same efficiency as wild-type Cx43, with normal voltage gating and a unitary conductance of ~110 pS. In HeLa cells, all three mutations also localized to regions of cell-cell contact and displayed a punctate staining pattern. In addition, we show that Cx43-G8V, Cx43-A44V and Cx43-E227D significantly increase membrane current flow through formation of active hemichannels, a novel activity that was not displayed by wild-type Cx43. The increased membrane current was inhibited by either 2 mM calcium, or 5 µM gadolinium, mediated by hemichannels with a unitary conductance of ~250 pS, and was not due to elevated mutant protein expression. The three Cx43 mutations all showed the same gain of function activity, suggesting that augmented hemichannel activity could play a role in skin-limited diseases caused by human Cx43 mutations.

Porokeratotic Eccrine Ostial and Dermal Duct Nevus and Porokeratotic Eccrine and Hair Follicle Nevus: Is Nomenclature "Porokeratotic Adnexal Ostial Nevus" More Appropriate?

Porokeratotic Eccrine Ostial and Dermal Duct Nevus and Porokeratotic Eccrine and Hair Follicle Nevus: Is Nomenclature "Porokeratotic Adnexal Ostial Nevus" More Appropriate?

Study on linear dermatoses

<p class="abstract"><strong>Background:</strong> The objective of the study was to study the incidence, age and sex distribution, association, distribution, clinical presentation, histopathological correlation of linear dermatoses at the skin Outpatient Department.</p><p class="abstract"><strong>Methods:</strong> A prospective study was conducted over a period of 1 year in 90 patients, attending the Department of Dermatology, at a tertiary care hospital, Chennai. Data collection, clinical examination and skin biopsy were recorded and analyzed statistically. Inclusion criteria were all patient with linear dermatoses. Exclusion criteria were Koebner phenomenon.<strong></strong></p><p class="abstract"><strong>Results:</strong> The incidence of linear dermatoses in our outpatient department- 0.2% per year. Among the linear dermatoses, lichen striatus was found to be the most common. The other dermatoses following Blaschko’s lines, in the descending order of frequency seen in this study were linear epidermal nevus, linear lichen planus, linear morphoea, inflammatory linear verrucous epidermal nevus, segmental vitiligo, hypomelanosis of ito, linear lichenoid dermatitis, linear psoriasis, segmental neurofibromatosis, linear whorled nevoid hypermelanosis, incontinentia pigmenti, nevus depigmentosus, linear porokeratosis. Female preponderance was noted. Majority of patients showed unilateral distribution mostly on the extremities.</p><p class="abstract"><strong>Conclusions:</strong> Most of the linear lesions are arranged along Blaschko’s line. The importance of histopathological correlation is obvious. Cases which were clinically diagnosed as lichen striatus, showed histopathological features of psoriasis and linear epidermal verrucous nevus. One case diagnosed clinically as epidermal nevus was found to be super imposed by psoriasis histologically. Another case clinically diagnosed as linear psoriasis clinically, was found to be linear porokeratosis on histopathology. Very few associations were noted.</p>

Case Report: "Incognito" proteus syndrome.

Proteus syndrome (PS) is a postnatal mosaic overgrowth disorder, progressive and disfiguring. It is clinically diagnosed according to the criteria reported by Biesecker et al. We describe the case of a 49-year-old woman who presented with a 10-year history of pauci-symptomatic infiltrating plaque lesions on the sole and lateral margin of the left foot, which had been diagnosed as a keloid. The patient had a positive history for advanced melanoma and a series of subtle clinical signs, such as asymmetric face, scoliosis, multiple lipomas on the trunk, linear verrucous epidermal nevi, and hyperpigmented macules with a mosaic distribution. Even if the clinical presentation was elusive, she had enough criteria to be diagnosed with PS. This case describes the first evidence, to the best of our knowledge, of pauci-symptomatic PS in adulthood, reports its rare association with advanced melanoma, and illustrates the importance of even minor cutaneous clinical signs, especially when atypical, in formulating the diagnosis of a complex cutaneous condition such as this.

Linear Psoriasis: A Case Report of a 71-Year-Old Female

A 71-year-old woman presented with a one-month history of pruritus, burning, flaky, and erythematous plaques. The lesions had a very narrow lineal distribution, extending from the lateral left sole to the left popliteal region. Microscopically, the skin biopsy sample revealed orthokeratosis, parakeratosis and traumatized Koebnerization. Elongation of rete ridges was not prominent. The patient had a good clinical response to oral prednisone. Linear psoriasis is a rare variant of psoriasis of unknown etiology. Approximately 23 reports have been published in the English literature. The main differential diagnosis is Inflammatory linear verrucous epidermal nevus (ILVEN). Thorough clinicopathologic findings and response to treatment are all required in the diagnosis and management of this rare form of psoriasis.