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Netherton Syndrome - Epidemiology Forecast to 2030

  • ID: 5387730
  • Drug Pipelines
  • July 2021
  • Region: Global
  • 60 pages
  • DelveInsight
This ‘Netherton Syndrome - Epidemiology Forecast - 2030' report delivers an in-depth understanding of the historical and forecasted epidemiology of Netherton Syndrome in the United States, EU5 (Germany, Spain, Italy, France, and the United Kingdom), and Japan.

Netherton Syndrome: Disease Understanding


Netherton Syndrome Overview


Netherton syndrome (NS) is a rare and severe genetic autosomal recessive disorder of ichthyosis. It is an inherited skin condition, often present at birth or in the first few weeks of life, where the skin is very red with fine dry scales. The condition tends to last for a lifetime, but the severity can vary from person to person.

It was described by Comèl (1949) and Netherton (1958). The syndrome is sometimes called Comèl-NS and is characterized by a clinical trial of ichthyosiform erythroderma, a specific hair shaft abnormality (trichorrhexis invaginata, or bamboo hair), atopic manifestations, and multisystemic complications.

NS is caused by loss of function mutations in the SPINK5 gene that encodes a serine peptidase inhibitor, Lympho-epithelial Kazal-type-related inhibitor (LEKTI). The loss of LEKTI leads to dysregulation of epidermal proteases and severe skin barrier impairment. Kallikrien-related peptidases, such as KLK7, and the epidermal elastase 2 (ELA2), which LEKTI inhibits, are reported to play major roles in NS pathology.

NS is diagnosed based on the symptoms and confirmed by genetic testing for changes in the SPINK5 gene. Other types of testing, such as close examination of the hair and a skin biopsy to obtain a small skin sample for examination under the microscope, can also be helpful for diagnosis. Often babies or children are not diagnosed for months or even years as red skin at birth can be a symptom of other conditions, such as other types of ichthyosis, severe eczema, or other forms of immune deficiency. Thus, differential diagnosis plays an important role.

There is no approved treatment for NS. Current approaches are all limited to symptom relief or supportive care with marginal efficacy and undesirable side effects. The treatment includes cleansing skin by non-detergent liquid cleansing oil, use of emollients, moisturizers, keratolytic, antipruritics, anti-histamines, antibiotics, topical corticosteroids, retinoids, calcipotriol, and Calcineurin inhibitors. Intravenous immunoglobulin and anti-TNF-a are therapeutic options for severe illness.

There is an urgent need for a targeted, effective and well-tolerated therapy. Further understanding of the underlying pathophysiology of skin changes will lead to more effective therapeutic modalities, including possible gene therapy.

Netherton Syndrome: Epidemiology


The Netherton Syndrome epidemiology division provides insights into the historical and current patient pool, along with the forecasted trend for every seven major countries. It helps recognize the causes of current and forecasted trends by exploring numerous studies and views of key opinion leaders. This part of the report also provides the diagnosed patient pool and their trends, along with assumptions undertaken.

Key Findings


The disease epidemiology covered in the report provides historical and forecasted Netherton Syndrome epidemiology segmented as Prevalent Population of Netherton Syndrome and Severity-specific cases of Netherton Syndrome. The report includes the prevalent scenario of Netherton Syndrome in the 7MM covering the United States, EU5 countries (Germany, France, Italy, Spain, and the United Kingdom), and Japan from 2018 to 2030.

Country-wise Netherton Syndrome Epidemiology


The epidemiology segment also provides the Netherton Syndrome epidemiology data and findings across the United States, EU5 (Germany, France, Italy, Spain, and the United Kingdom), and Japan.

The total prevalent population of Netherton Syndrome in the 7MM countries was estimated to be 3,411 cases in 2020.

As per the estimates, the United States had the largest prevalence of Netherton Syndrome in 2020. Among the EU5 countries, Germany had the largest prevalent population of Netherton Syndrome with 400 cases, followed by France in 2020. On the other hand, Spain had the lowest prevalent population of 249 cases in 2020.

Scope of the Report

  • Netherton Syndrome report covers a detailed overview explaining its causes, symptoms, classification, pathophysiology, diagnosis, and treatment patterns.
  • Netherton Syndrome Epidemiology Report and Model provide an overview of the risk factors and global Netherton Syndrome trends in the seven major markets (7MM: US, France, Germany, Italy, Spain, UK, and Japan).
  • The report provides insight into the historical and forecasted patient pool of Netherton Syndrome in seven major markets covering the United States, EU5 (Germany, Spain, France, Italy, UK), and Japan
  • The report helps recognize the growth opportunities in the 7MM concerning the patient population.
  • The report assesses the disease risk and burden and highlights the unmet needs of Netherton Syndrome.
  • The report provides the segmentation of the Netherton Syndrome epidemiology by Prevalent cases of Netherton Syndrome in the 7MM
  • The report provides the segmentation of the Netherton Syndrome epidemiology by Severity-specific cases of Netherton Syndrome in the 7MM.

Report Highlights

  • 10-year Forecast of Netherton Syndrome epidemiology
  • 7MM Coverage
  • Prevalent Cases of Netherton Syndrome
  • Severity-specific Cases of Netherton Syndrome

KOL Views


The publisher interviews KOLs and obtain SME's opinion through primary research to fill the data gaps and validate our secondary research. The opinion helps understand the total patient population and current treatment pattern. This will support the clients in potential upcoming novel treatment by identifying the overall scenario of the indications.

Key Questions Answered

  • What will be the growth opportunities in the 7MM for the patient population pertaining to Netherton Syndrome?
  • What are the key findings pertaining to the Netherton Syndrome epidemiology across 7MM, and which country will have the highest number of patients during the forecast period (2018-2030)?
  • What would be the total number of patients with Netherton Syndrome across the 7MM during the forecast period (2018-2030)?
  • Among the EU5 countries, which country will have the highest number of patients during the forecast period (2018-2030)?
  • At what CAGR the patient population is expected to grow by in the 7MM during the forecast period (2018-2030)?
  • What are the disease risk, burdens, and unmet needs of Netherton Syndrome?
  • What are the currently available treatments for Netherton Syndrome?

Reasons to Buy


Netherton Syndrome Epidemiology report will allow the user to:

  • Develop business strategies by understanding the trends shaping and driving the global Netherton Syndrome market
  • Quantify patient populations in the global Netherton Syndrome market to improve product design, pricing, and launch plans
  • Organize sales and marketing efforts by identifying the age groups and sex that present the best opportunities for Netherton Syndrome therapeutics in each of the markets covered
  • Understand the magnitude of the Netherton Syndrome population by its prevalent cases.
  • Understand the magnitude of the Netherton Syndrome population by its severity-specific cases.
  • The Netherton Syndrome epidemiology report and model were written and developed by Masters and PhD level epidemiologists
  • The Netherton Syndrome Epidemiology Model developed by the publisher is easy to navigate, interactive with dashboards, and epidemiology based on transparent and consistent methodologies. Moreover, the model supports data presented in the report and showcases disease trends over a 10-year forecast period using reputable sources

Key Assessments

  • Patient Segmentation
  • Disease Risk and Burden
  • Risk of disease by the segmentation
  • Factors driving growth in a specific patient population

Geographies Covered

  • The United States
  • EU5 (Germany, France, Italy, Spain, and the United Kingdom)
  • Japan
Study Period: 2018-2030

As per the National Organization of Rare Disorders (NORD), NS is a rare hereditary disorder with approximately 150 cases reported in the medical literature. However, the actual number of affected individuals may be much higher due to diagnostic difficulties and overlapping symptoms with common atopic dermatitis and other congenital Ichthyosis. The incidence and prevalence of NS have been estimated to be 1/200,000 births and 1-9/million, respectively; similar numbers are published in most of the literature available worldwide. Moreover, it has been reported that that NS could account for up to 18% of congenital erythroderma.
Note: Product cover images may vary from those shown
1. Key Insights

2. Report Introduction

3. Netherton Syndrome Market Overview at a Glance
3.1. Market Share (%) Distribution of Netherton Syndrome in 2018
3.2. Market Share (%) Distribution of Netherton Syndrome in 2030

4. Executive Summary of Netherton Syndrome

5. Disease Background and Overview
5.1. Introduction
5.2. Clinical Manifestations
5.3. Pathogenesis
5.4. Diagnosis
5.5. Treatment of Netherton Syndrome (NS)

6. Epidemiology and Patient Population
6.1. Key Findings
6.2. 7MM Total Prevalent Population of Netherton Syndrome
6.3. Epidemiology of Netherton Syndrome
6.4. The United States
6.4.1. Prevalent Cases of Netherton Syndrome in the United States
6.4.2. Severity-specific cases of Netherton Syndrome in the United States
6.5. EU5
6.5.1. Germany
6.5.1.1. Prevalent Cases of Netherton Syndrome in Germany
6.5.1.2. Severity-specific cases of Netherton Syndrome in Germany
6.5.2. France
6.5.2.1. Prevalent Cases of Netherton Syndrome in France
6.5.2.2. Severity-specific cases of Netherton Syndrome in France
6.5.3. Italy
6.5.3.1. Prevalent Cases of Netherton Syndrome in Italy
6.5.3.2. Severity-specific cases of Netherton Syndrome in Italy
6.5.4. Spain
6.5.4.1. Prevalent Cases of Netherton Syndrome in Spain
6.5.4.2. Severity-specific cases of Netherton Syndrome in Spain
6.5.5. United Kingdom
6.5.5.1. Prevalent Cases of Netherton Syndrome in the UK
6.5.5.2. Severity-specific cases of Netherton Syndrome in the United Kingdom
6.6. Japan
6.6.1. Prevalent Cases of Netherton Syndrome in Japan
6.6.2. Severity-specific cases of Netherton Syndrome in Japan

7. Organizations contributing toward the fight against NS

8. SWOT Analysis

9. Unmet Needs

10. Appendix
10.1. Bibliography
10.2. Report Methodology

11. Publisher Capabilities

12. Disclaimer

13. About the Publisher

List of Tables
Table 1: Summary of Netherton Syndrome, Market, Epidemiology, and Key Events (2018-2030)
Table 2: Prevalence Population of Netherton Syndrome in 7MM (2018-2030)
Table 3: Prevalent Cases of Netherton Syndrome in the United States (2018-2030)
Table 4: Severity-specific cases of Netherton Syndrome in the United States (2018-2030)
Table 5: Prevalent Cases of Netherton Syndrome in Germany (2018-2030)
Table 6: Severity-specific cases of Netherton Syndrome in Germany (2018-2030)
Table 7: Prevalent Cases of Netherton Syndrome in France (2018-2030)
Table 8: Severity-specific cases of Netherton Syndrome in France (2018-2030)
Table 9: Prevalent Cases of Netherton Syndrome in Italy (2018-2030)
Table 10: Severity-specific cases of Netherton Syndrome in Italy (2018-2030)
Table 11: Prevalent Cases of Netherton Syndrome in Spain (2018-2030)
Table 12: Severity-specific cases of Netherton Syndrome in Spain (2018-2030)
Table 13: Prevalent Cases of Netherton Syndrome in the United Kingdom (2018-2030)
Table 14: Severity-specific cases of Netherton Syndrome in the United Kingdom (2018-2030)
Table 15: Prevalent Cases of Netherton Syndrome in Japan (2018-2030)
Table 16: Severity-specific cases of Netherton Syndrome in Japan (2018-2030)
Table 17: Organizations contributing toward the fight against NS

List of Figures
Figure 1: The common cutaneous and extra-cutaneous manifestations observed in Netherton syndrome patients
Figure 2: Clinical signs and symptoms of Netherton syndrome
Figure 3: Schematic representation of NS patient skin in comparison to healthy skin.
Figure 4: Signaling pathways underlying the pathophysiology of Netherton syndrome
Figure 5: Treatment options in Netherton Syndrome
Figure 6: Prevalent Population of Netherton Syndrome in 7MM (2018-2030)
Figure 7: Prevalent Cases of Netherton Syndrome in the United States (2018-2030)
Figure 8: Severity-specific cases of Netherton Syndrome in the United States (2018-2030)
Figure 9: Prevalent Cases of Netherton Syndrome in Germany (2018-2030)
Figure 10: Severity-specific cases of Netherton Syndrome in Germany (2018-2030)
Figure 11: Prevalent Cases of Netherton Syndrome in France (2018-2030)
Figure 12: Severity-specific cases of Netherton Syndrome in France (2018-2030)
Figure 13: Prevalent Cases of Netherton Syndrome in Italy (2018-2030)
Figure 14: Severity-specific cases of Netherton Syndrome in Italy (2018-2030)
Figure 15: Prevalent Cases of Netherton Syndrome in Spain (2018-2030)
Figure 16: Severity-specific cases of Netherton Syndrome in Spain (2018-2030)
Figure 17: Prevalent Cases of Netherton Syndrome in the United Kingdom (2018-2030)
Figure 18: Severity-specific cases of Netherton Syndrome in the United Kingdom (2018-2030)
Figure 19: Prevalent Cases of Netherton Syndrome in Japan (2018-2030)
Figure 20: Severity-specific cases of Netherton Syndrome in Japan (2018-2030)
Figure 21: Seven Major Market Size of Netherton Syndrome in USD Million (2018-2030)
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