Ichthyosis linearis circumflexa in a child. Response to narrowband UVB therapy.

Singer, Ralfi; Copur, Mehmet Sitki; Yüksel, Ece Nur; Kocatürk, Emek; Erhan, Selma Şengiz

doi:10.3315/jdcr.2015.1214

Cited by 10 publications

(9 citation statements)

References 6 publications

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“…The efficacy of phototherapy was reported in a few case reports, in four children treated with narrowband UVB and in two adults treated with psoralen–UVA (PUVA) or UVA‐1 . Short‐term efficacy was reported in all but one .…”

Section: Particularities Of Congenital Ichthyosismentioning

confidence: 99%

Management of congenital ichthyoses: European guidelines of care, part two

et al. 2018

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These guidelines for the management of congenital ichthyoses have been developed by a multidisciplinary group of European experts following a systematic review of the current literature, an expert conference held in Toulouse in 2016, and a consensus on the discussions. These guidelines summarize evidence and expert-based recommendations and intend to help clinicians with the management of these rare and often complex diseases. These guidelines comprise two sections. This is part two, covering the management of complications and the particularities of some forms of congenital ichthyosis.

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Section: Particularities Of Congenital Ichthyosismentioning

confidence: 99%

Management of congenital ichthyoses: European guidelines of care, part two

et al. 2018

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“…Several case reports describe clinical improvement of skin lesions in NS patients after treatment with NB-UVB [96,123,124], PUVA [125] or UVA1 [126] phototherapy.…”

Section: Phototherapymentioning

confidence: 99%

Advances in understanding of Netherton syndrome and therapeutic implications

Petrova

Hovnanian

2020

Expert Opinion on Orphan Drugs

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Introduction: Netherton syndrome (NS) is a rare and severe ichthyosis characterized by superficial scaling, skin inflammation, a specific hair shaft defect, severe atopic manifestations and multisystemic complications. It is an orphan disease with currently no satisfactory treatment. NS is caused by loss-offunction mutations in SPINK5 encoding the serine protease inhibitor LEKTI. NS patients present with ichthyosiform erythroderma or ichthyosis linearis circumflexa and show considerable clinical variability. Areas covered: Uncontrolled serine protease activity leads to a profound skin barrier defect and the release of pro-inflammatory and pro-allergic mediators by keratinocytes and immune cells. Improved understanding of NS pathogenesis has led to the successful use of repurposed biologics such as intravenous immunoglobulins and anti-IL-17A blockers. Between April 1, 2020 and November 18, 2020, authors searched for NS-relevant information in the following databases: MEDLINE, DrugBank, ClinicalTrials.gov, and patent datasets accessed through lens.org. Expert opinion: Specific KLK5 and/or KLK7 inhibitors represent the most promising disease-modifying treatments. They are currently being developed by several companies. Comprehension of the determinants of NS variability, flares and modification over time will be the foundation for precision medicine. While improved knowledge of the inflammatory and allergic pathways involved is still needed, clinical trials using repurposed biologics have already begun.

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“…In a case report of a newborn with NS, treated with a total of 170 sessions of NB-UVB phototherapy, with an aggregate dose of 240 J over 3 years, there was the appearance of nevi and multiple pigmented lesions ( 41 ). In another case, of a boy treated with NB-UVB three times a week for a total of 30 sessions and an aggregate dose of 54.7 J/cm 2 , no side effects were reported, and the treatment led to significant improvement of the lesions ( 42 ).…”

Section: Resultsmentioning

confidence: 99%

“…The specific mechanism of function of phototherapy in NS is not known. However, it has been proposed that phototherapy may increase the production of serine protease inhibitors that balance the function of the defective LEKTI ( 42 ). Additionally, it may have immunomodulatory and apoptosis-inducing effects on keratinocytes, dendritic cells, mast cells, and T-lymphocytes ( 43 ).…”

Section: Resultsmentioning

confidence: 99%

Netherton Syndrome in Children: Management and Future Perspectives

et al. 2021

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Netherton syndrome (NS) is a genetic, multisystemic disease classically distinguished by a triad of clinical manifestations: congenital ichthyosiform erythroderma, hair shaft abnormalities, and immune dysregulation. Due to the complex pathogenesis of the disease, there are no specific therapies currently accessible for patients with NS. An early diagnosis is crucial to start the correct management of these patients. A multidisciplinary approach, including specialists in immunology, allergology, and dermatology, is necessary to set up the best therapeutic pathway. We conducted a review with the aim to summarize the different therapeutic strategies currently accessible and potentially available in the future for children with NS. However, given the limited data in the literature, the best-tailored management should be decided upon the basis of the specific clinical characteristics of the patients with this rare clinical condition. Further comprehension of the pathophysiology of the disease could lead to more efficacious specific therapeutic options, which could allow a change in the natural history of NS.

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Ichthyosis linearis circumflexa in a child. Response to narrowband UVB therapy.

Cited by 10 publications

References 6 publications

Management of congenital ichthyoses: European guidelines of care, part two

Management of congenital ichthyoses: European guidelines of care, part two

Advances in understanding of Netherton syndrome and therapeutic implications

Netherton Syndrome in Children: Management and Future Perspectives

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