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Disease Processes ▼
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Disease Information for Netherton's Syndrome
- 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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- Merck
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- NGC (National Guideline Clearinghouse)
- Medscape (eMedicine)
- Harrison's Online (accessmedicine)
- NEJM (The New England Journal of Medicine)