Primary hereditary ichthyoses in the Eastern Province of Saudi Arabia

Al‐Zayir, Ahmad A.; Alakloby, Omar M.

doi:10.1111/j.1365-4632.2004.01687.x

Cited by 6 publications

(15 citation statements)

References 18 publications

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“…Male to female ratio in our study was 0.9 : 1. This was different from other studies, in which a male predominance was found [ 6 , 13 ].…”

Section: Discussioncontrasting

confidence: 99%

“…Our study shows that the occurrence rate of PHI was 25.7% of genodermatosis patients attending the genetics clinics and 1 per 2359 patients attending the Pediatric Hospital, Ain Shams University, Cairo, Egypt. The occurrence rate of PHI in the Eastern Province of Saudi Arabia study was 0.67% or 7 per 1000 new dermatology cases [ 6 ]. In addition, another study stated that the incidence of moderate to severe ichthyosis is 5 to 10 per 100,000 people every year in the United States [ 12 ].…”

Section: Discussionmentioning

confidence: 99%

“…Associated cutaneous and extracutaneous features, as well as disease onset and clinical course, provide important diagnostic clues. In all forms of Mendelian Disorders of Cornification (MeDOC), the clinical presentation may be variable in both severity and in the expression of associated features, and at times additional laboratory testing including microscopy and genetic analyses is required [ 1 , 6 – 8 ].…”

Section: Introductionmentioning

confidence: 99%

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High frequency of primary hereditary ichthyoses in the North-East region of Cairo, Egypt

Elsayed

Seifeldin

Gobrial

2018

pdia

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IntroductionInherited ichthyoses are caused by mutations in various genes important for keratinocyte differentiation and epidermal barrier function. Although ichthyoses are rare disorders, they require costly long-term medical management, and thus there is a need for efficient preventive and therapeutic strategies.AimWe performed a retrospective study to determine the frequency, types, clinical presentation and associated genomic errors of primary hereditary ichthyoses in Egyptian patients and their relatives consulting the Genetics Clinic, Pediatric Hospital, Ain Shams University.Material and methodsThe outpatient log books of patients between January 2000 and December 2014 were reviewed, and diagnosis of new patients was confirmed through examination by a dermatologist. All epidemiologic, demographic, and clinical data were extracted and recorded in especially designed data collection forms.ResultsThe occurrence rate of primary hereditary ichthyoses in our study was 25.7% of genodermatosis patients attending the genetics clinics and 1 per 2359 patients attending the Pediatric Hospital. The commonest type of ichthyosis in our study was Lamellar ichthyosis (38%), followed by congenital ichthyosiform erythroderma (26.8%). Consanguineous marriage was reported among the parents of 79% of patients and positive family history was reported in 72% of patients.ConclusionsTo the best of our knowledge, this preliminary study is the first report on the clinico-epidemiological features of primary hereditary ichthyoses in Egypt. The high rate of prenatal consanguinity among parents of our patients may account for the high frequency of these genodermatoses in Egypt. This highlights the importance of genetic counselling and prenatal diagnosis in Egypt.

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“…Male to female ratio in our study was 0.9 : 1. This was different from other studies, in which a male predominance was found [ 6 , 13 ].…”

Section: Discussioncontrasting

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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High frequency of primary hereditary ichthyoses in the North-East region of Cairo, Egypt

Elsayed

Seifeldin

Gobrial

2018

pdia

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“…Earliest studies were conducted outside Europe (Saudi Arabia and Tunisia) and concluded to prevalence which seems different than European one because of a high level of consanguinity [9-12]. In Europe, two recent Spanish studies have dealt with ichthyosis prevalence [13,14].…”

Section: Discussionmentioning

confidence: 99%

“…During the past few years, much progress has been achieved in describing the molecular basis of these disorders and in establishing genotype-phenotype correlations [8]. However, prevalence remains poorly described [9-16] and no patient registration exists. Understanding epidemiology of rare skin disorders such as ichthyosis is one of the main objectives advocated in the Plan Maladies Rares defined by the French Health ministry [17].…”

Section: Introductionmentioning

confidence: 99%

Prevalence of inherited ichthyosis in France: a study using capture-recapture method

Dreyfus

Chouquet

Ezzedine

et al. 2014

Orphanet J Rare Dis

117

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BackgroundInherited ichthyoses represent a group of rare skin disorders characterized by scaling, hyperkeratosis and inconstant erythema, involving most of the tegument. Epidemiology remains poorly described. This study aims to evaluate the prevalence of inherited ichthyosis (excluding very mild forms) and its different clinical forms in France.MethodsCapture – recapture method was used for this study. According to statistical requirements, 3 different lists (reference/competence centres, French association of patients with ichthyosis and internet network) were used to record such patients. The study was conducted in 5 areas during a closed period.ResultsThe prevalence was estimated at 13.3 per million people (/M) (CI95%, [10.9 – 17.6]). With regard to autosomal recessive congenital ichthyosis, the prevalence was estimated at 7/M (CI 95% [5.7 – 9.2]), with a prevalence of lamellar ichthyosis and congenital ichthyosiform erythroderma of 4.5/M (CI 95% [3.7 – 5.9]) and 1.9/M (CI 95% [1.6 – 2.6]), respectively. Prevalence of keratinopathic forms was estimated at 1.1/M (CI 95% [0.9 – 1.5]). Prevalence of syndromic forms (all clinical forms together) was estimated at 1.9/M (CI 95% [1.6 – 2.6]).ConclusionsOur results constitute a crucial basis to properly size the necessary health measures that are required to improve patient care and design further clinical studies.

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Disorders of epidermal maturation and keratinization

Weedon¹

2010

Weedon's Skin Pathology

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Primary hereditary ichthyoses in the Eastern Province of Saudi Arabia

Cited by 6 publications

References 18 publications

High frequency of primary hereditary ichthyoses in the North-East region of Cairo, Egypt

High frequency of primary hereditary ichthyoses in the North-East region of Cairo, Egypt

Prevalence of inherited ichthyosis in France: a study using capture-recapture method

Disorders of epidermal maturation and keratinization

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