Disease Information for Netherton's Syndrome

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Clinical Manifestations
Signs & Symptoms
Erythematous generalized rash
Rash
Scaling/Skin finding
Mental Deficiency Child
Growth/development delay
Clinical Presentation & Variations
Presentation/Otitis Media Recurrent
Disease Progression
Course/Improves with time/growth/development
Course/Prognosis good/usually
Demographics & Risk Factors
Sex & Age Groups
Population/Child
Population/Children/all
Population/Infant
Population/Neonate-newborn
Laboratory Tests
Abnormal Lab Findings - Increased
IGE/Immunoglobulin E (Lab)
Diagnostic Test Results
Pathology
PATH/Hair shaft/nodes frayed breakpoints
Associated Diseases & Rule outs
Associated Disease & Complications
Aminoaciduria
Atopic allergic disease
Bacterial infection, general
Cellular immunity defect/deficiency
Eczema/Dermatitis, atopic
Ichthyosis
Ichthyosis congenita
Immunodeficiency
Infections
Mental retardation
Neonatal Erythroderma
Netherton's Syndrome
Trichorrhexis nodosa
Urticaria/hives
Disease Mechanism & Classification
Class
CLASS/Pediatric disorders (ex)
CLASS/Hair involvement/disorder (ex)
Pathophysiology
Pathophysiology/Aminoaciduria Intermittant
Pathophysiology/Gene locus 5q32
Pathophysiology/Gene locus Chromosome 5
Pathophysiology/Gene locus Chromosome 5q
Pathophysiology/Gene SPINK5 defect/absent
Process
PROCESS/Autosomal recessive disorder (ex)
PROCESS/Hereditofamilial (category)
PROCESS/INCIDENCE/Extremely rare disease
PROCESS/INCIDENCE/Rare disease (ex)
Synonyms
Synonym
Synonym/Congenital Ichthyosiform Erythroderma, Synonym/Ichthyosis Linearis Circumscripta
Definition

Netherton's syndrome medical dictionary

<syndrome>

Congenital ichthyosiform erythroderma or ichthyosis linearis circumscripta associated with bamboo hair, and irregularly with atopy, urticaria, intermittent aminoaciduria, and mental retardation; probably an autosomal recessive trait that frequently resolves or improves in adolescence. medical dictionary monodata online

(05 Mar 2000)

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External Links Related to Netherton's Syndrome
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Wikipedia
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PubMed (National Library of Medicine)
NGC (National Guideline Clearinghouse)
Medscape (eMedicine)
Harrison's Online (accessmedicine)
NEJM (The New England Journal of Medicine)
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