Disease Information for Incontinentia pigmenti/hereditary

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Clinical Manifestations
Signs & Symptoms
Birthmark
Decreased sweating/Absent sweating
Face skin lesions
Fissuring palms/soles
Hyperkeratosis/palms and soles
Increased pigmentation/tanning/hyperpigmented
Reticular pigmentations
Slate gray pigmentations
Small nails/congenital
Vesicobullous rash/lesions in Children
Vesicobullous rashes
Carious teeth/child
Carious teeth/sign
Dental/enamel erosion/discoloration
Teeth malformed/dysplastic
Tooth Enamel defects
Yellowing teeth
Autonomic instability/Variable BP/HR
Infant Seizures
Mental Deficiency Child
Seizures
Seizures/Children/recurrent
Macrocephaly/Large head
Megalocephaly
Blue Sclera
Disease Progression
Course/Chronic disorder
Course/Chronic only
Course/Progressive
Course/Progressive/slowly chronic illness
Onset/Infancy
Onset/insidious
Demographics & Risk Factors
Population Group
Child
Infant
Population/Pediatrics population
Sex & Age Groups
Population/Child
Population/Child-Infant Only
Population/Children/all
Population/Infant
Associated Diseases & Rule outs
Associated Disease & Complications
Anhydrosis/Absent sweating/diffuse
Dental caries
Dental deformities/anomalies
Fetal wastage
Hyperpigmentation
Incontenintia pigmenti/hereditary
Mental retardation
Microcephaly/oligophrenia
Teeth enamel hypoplastic
Vasomotor instability
Disease Mechanism & Classification
Class
CLASS/Pediatric disorders (ex)
CLASS/Dermatologic/Subcutaneous (category)
Pathophysiology
Pathophysiology/Genomic indentifiers (polymorphism/snip/mutations)
Pathophysiology/Homozygous/dominant lethal
Pathophysiology/X-linked dominant allele/Heterozygous
Pathophysiology/Lethal Mutation Homozygous
Process
PROCESS/Autosomal dominant hereditary disease (ex).
PROCESS/Hereditofamilial (category)
PROCESS/Phakomatosis/congenital (ex)
PROCESS/X-Linked dominant inheritance (ex)
PROCESS/Birthmark (ex)
Synonyms
Synonym
Incontinetia pigmenti hereditary, Synonym/Bloch-Sulzberger Syndrome, Synonym/Chromophore nevus/Naegeli, Synonym/Franceschetti-Jadassohn syndrome, Synonym/Naegeli incontinentia pigmenti, Synonym/Naegeli's syndrome/variant
Definition
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External Links Related to Incontinentia pigmenti/hereditary
Google
Wikipedia
Merck
Images
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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