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Disease Comparison Results (show all mode) for Incontinentia pigmenti/hereditary and Papillion-Lefevre syndrome

Clinical
Demographics
Lab
Diagnostic
Diseases
Classification
Synonyms
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Found: 72 | Differences: 53

Found: 65 | Differences: 46

Incontinentia pigmenti/hereditary and Papillion-Lefevre syndrome sharing 19 common findings. (show all, show common, show differences)

Clinical Manifestations
Signs & Symptoms: Dysplasia Toenails; Excess Sweating Children; Fissuring palms/soles; Hyperhidrosis/Chronic sweating excess; Hyperkeratosis/palms and soles; Hypertrophy nails/Onychogryposis; Palmar keratosis; Plantar hyperkeratosis/sign; Rash hand/foot areas; Sweating Excess Perspiration; Carious teeth/child; Carious teeth/sign; Eroded teeth; Loose teeth/falling out; Premature loss of teeth; Teeth malformed/dysplastic; Long thin fingers/toes (arachnodactyly)
Disease Progression: Course/Chronic disorder; Course/Chronic only; Onset/1-5 years old

Demographics & Risk Factors
Established Disease Population: Patient/Edentulous patient/status
Population Group: Child; Population/Pediatrics population
Sex & Age Groups: Population/Child; Population/Child-Infant Only; Population/Children/all

Laboratory Tests
Abnormal Lab Findings (Non Measured): Chromosomal abnormality (Lab)

Diagnostic Test Results
X-RAY: Xray/Dental Abnormal

Associated Diseases & Rule outs
Associated Disease & Complications: Acro-osteolysis; Dental caries; Dental deformities/anomalies; Flat feet/pes planus; Gingivitis; Impetigo/Pyoderm; Keratoderma palmaris; Nail dystrophy; Onycholysis; Papillion-Lefevre syndrome; Periodontitis in children; Periodontitis/periodontal disease; Plantar callous/palmar callous keratosis; Pyoderm; Skin infections; Teeth enamel hypoplastic; Hand anomalies

Disease Mechanism & Classification
Class: CLASS/Pediatric disorders (ex); CLASS/Dermatologic/Subcutaneous (category); CLASS/Skin disorder (ex); CLASS/Teeth/dental involvement/disorder (ex)
Pathophysiology: Pathophysiology/Deficit enzyme cathepsin C; Pathophysiology/Gene locus Chromosome 11; Pathophysiology/Gene locus Chromosome 11q; Pathophysiology/Gene Locus Identified/OMIM database; Pathophysiology/Gene locus/long arm chromosome 11 (11q14); Pathophysiology/Genomic indentifiers (polymorphism/snip/mutations); Pathophysiology/Gene Cathespin C Mutation; Pathophysiology/Maternal Chromosome mutation
Process: PROCESS/Autosomal recessive disorder (ex); PROCESS/Enzyme defect/Metabolic disorder (ex); PROCESS/Eponymic (category); PROCESS/Eponymic/Esoteric/Not yet integrated into database; PROCESS/Genetic disorder/Spontaneous mutations/sporadic; PROCESS/Hereditary developmental disorder (ex); PROCESS/Hereditofamilial (category); PROCESS/INCIDENCE/Esoteric disease (example); PROCESS/INCIDENCE/Extremely rare disease; PROCESS/Metabolic/storage disorder (category)

Synonyms
Synonym: Synonym/Cochin-Jewish Disease; Synonym/Haim-Munk syndrome; Synonym/Palmar-plantar hyperkeratosis periodontal destruction; Synonym/Palmoplantar keratoderma with periodontosis; Synonym/PLS

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: Synonym/Bloch-Sulzberger Syndrome; Synonym/Chromophore nevus/Naegeli; Synonym/Franceschetti-Jadassohn syndrome; Synonym/Naegeli incontinentia pigmenti; Synonym/Naegeli's syndrome/variant

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