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         Bloch-sulzberger Syndrome:     more detail

1. Bloch-Sulzberger Syndrome
Multiple Congenital Anomaly/Mental Retardation (MCA/MR) Syndromes. View the Full Record. Syndrome, blochsulzberger syndrome. Synonyms, Asboe-Hansen disease.
http://www.nlm.nih.gov/mesh/jablonski/syndromes/syndrome067.html
Multiple Congenital Anomaly/Mental Retardation (MCA/MR) Syndromes
View the Full Record
Syndrome Bloch-Sulzberger syndrome Synonyms Asboe-Hansen disease Bloch-Siemens syndrome Bloch-Sulzberger melanoblastoma Siemens-Bloch pigmented dermatosis incontinentia pigmenti (IP) incontinentia pigmenti 1 melanoblastosis cutis linearis sive systematisata melanosis corii degenerativa nevus pigmentosus systematicus Summary A skin pigmentation disorder with malformations of the eyes, teeth, bones, nails, heart, central nervous system, and hair. Mental deficiency is usually associated. The syndrome is divided into two forms: Incontinentia pigmenti type I and type II which lethal in males. Major Features Head and neck: Microcephaly. Eyes: Ocular abnormalities are present in about one-third of all cases. They include strabismus, cataracts, optic atrophy, retrolental fibroplasia, pseudoglioma, retinal detachment, myopia, blue sclerae, nystagmus, microphthalmos, retinal telangiectasia, ophthalmia, and pigmentation disorders of the conjunctiva, iris, retina, and oculomotor paralysis. Thorax: Supernumerary ribs, unilateral breast aplasia, and supernumerary nipples.

2. NINDS Incontinentia Pigmenti Information Page
Synonym(s) blochsulzberger syndrome. Reviewed 12-01-2003
http://www.ninds.nih.gov/health_and_medical/disorders/inconpig_doc.htm
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NINDS Incontinentia Pigmenti Information Page
Synonym(s):
Bloch-Sulzberger Syndrome
Reviewed 12-01-2003 Get Web page suited for printing
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Table of Contents (click to jump to sections) What is Incontinentia Pigmenti?
Is there any treatment?
What is the prognosis? What research is being done? ... Organizations What is Incontinentia Pigmenti? Is there any treatment? The skin abnormalities of IP usually disappear by adolescence or adulthood without treatment. Diminished vision may be treated with corrective lenses, medication, or, in severe cases, surgery. A specialist may treat dental problems. Neurological symptoms such as seizures, muscle spasms, or mild paralysis may be controlled with medication and/or medical devices and with the advice of a neurologist. What is the prognosis?

3. EMedicine - Incontinentia Pigmenti : Article By Celia H Chang, MD
Incontinentia Pigmenti Incontinentia pigmenti type 2, also known as bloch-sulzberger syndrome, is a rare, X-linked, dominantly inherited disorder of skin pigmentation that often is associated
http://www.emedicine.com/neuro/topic169.htm
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Incontinentia Pigmenti
Last Updated: February 8, 2002 Rate this Article Email to a Colleague Synonyms and related keywords: Bloch-Sulzberger syndrome AUTHOR INFORMATION Section 1 of 10 Author Information Introduction Clinical Differentials ... Bibliography
Author: Celia H Chang, MD , Assistant Professor, Department of Neurology, University of California at Davis Celia H Chang, MD, is a member of the following medical societies: American Academy of Neurology , and Child Neurology Society Editor(s): David Griesemer, MD , Chairman of Neurology, Associate Professor, Departments of Pediatrics and Neurology, Medical University of South Carolina; Francisco Talavera, PharmD, PhD , Senior Pharmacy Editor, Pharmacy, eMedicine; Kenneth J Mack, MD, PhD , Senior Associate Consultant, Department of Child and Adolescent Neurology, Mayo Clinic;

4. Bloch-Sulzberger Pigment Dermatosis (Bruno Bloch) (www.whonamedit.com)
BlochSulzberger pigment dermatosis (Bruno Bloch) A complex congenital disturbance characterized by bizarre, widespread pigmented macules of unusual shapes and defects of teeth, eyes, nails, Bloch) Bloch-Siemens syndrome (Bruno Bloch) Bloch-Sulzberger disease (Bruno Bloch) Bloch-Sulzberger melanoblastosis review of the bloch-sulzberger syndrome was provided by Landy
http://www.whonamedit.com/synd.cfm/1762.html

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Bloch-Sulzberger pigment dermatosis (Bruno Bloch) Also known as:
Asboe=Hansen's disease (Bruno Bloch)
Bloch-Siemens syndrome (Bruno Bloch) Bloch-Sulzberger disease (Bruno Bloch) Bloch-Sulzberger melanoblastosis (Bruno Bloch) Siemens-Bloch pigmented dermatosis (Bruno Bloch) Siemens-Bloch-Sulzberger disease (Bruno Bloch) Synonyms: Incontinentia pigmenti, melanoblastosis cutis linearis sive systematisata (Carol and Bour), melanosis corii degenerativa (Siemens), naevus pigmentosus systematicus. Associated persons: Gustav Asboe-Hansen Bruno Bloch Hermann Werner Siemens Marion Baldur Sulzberger Description: A complex congenital disturbance that occurs almost exclusively in newborn girls (95 %), characterized by bizarre, widespread pigmented macules of unusual shapes; and defects of teeth, eyes, nails, central nervous system and hair. Long lists of other symptoms includes retarded growth and mental retardation. Inheritance is X-linked dominant. It is usually lethal in males, who are probably the result of spontaneous mutations.

5. Syndrome DB - Table Of Contents
blepharophimosisepicanthus inversus-telecanthus syndrome Bloch-Siemens syndrome Bloch-Sulzberger melanoblastoma bloch-sulzberger syndrome Bohring syndrome
http://www.nlm.nih.gov/mesh/jablonski/syndrome_toc/toc_b.html
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6. LookSmart - Directory - Incontinentia Pigmenti Or Bloch-Sulzberger Syndrome
Incontinentia Pigmenti or blochsulzberger syndrome - Access resources, journal abstracts and support groups pertaining to incontinentia pigmenti or IP.
http://search.looksmart.com/p/browse/us1/us317837/us317920/us53948/us89134/us101
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Incontinentia Pigmenti or Bloch-Sulzberger Syndrome - Access resources, journal abstracts and support groups pertaining to incontinentia pigmenti or IP.
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  • allRefer Health - Incontinentia Pigmenti Achromians Information
    Uncover the symptoms, treatment, complications, and prevention of this condition, aka hypomelanosis of ito. Call a doctor if skin exhibits an unusual pigmentary pattern.
    allRefer Health - Incontinentia Pigmenti Syndrome (IPS)

    Profiles the symptoms, treatment, risks, tests, signs, prevention, prognosis, causes, and complications of this inherited disorder, aka Bloch-Sulzberger's disease.
    Dept. of Dermatology University of Iowa - Incontinentia Pigmenti

    Features several photos of incontinentia pigmenti and its distinguishable characteristics including blisters, rashes and discolorations.
    Dermatology Online Journal - Incontinentia Pigmenti Histology

    View slide samples depicting the cellular abnormalities in patients diagnosed with IP. Includes a brief description of the rare disorder.
    Features of Incontinentia Pigmenti
    View a brief text-based listing of related features that may be evident in patients diagnosed with IP, including anomalies of the hair and skin.
  • 7. Dictionary Definition Of BLOCH-SULZBERGER SYNDROME
    Dictionary definition of blochsulzberger syndrome. Medical dictionary. The ip sons die before birth. Ip is also known as bloch-sulzberger syndrome.
    http://www.dictionarybarn.com/BLOCHSULZBERGER-SYNDROME.php
    Dictionary definition of BLOCH-SULZBERGER SYNDROME
    Browse Dictionary by alphabet A B C D ... Z Top Words blab
    black

    black art

    black bass
    ...
    blasto-

    A genetic disease with blisters that develop soon after birth on the trunk and limbs, then heal, but leave dark (hyperpigmented) streaks and marble-like whorls on the skin. (the name came from the erroneous idea that the skin cells were incontinent of pigment and could not contain it normally.) other key features of ip include dental and nail abnormalities, bald patches, and (in about 1/3rd of cases) mental retardation. Ip is an x-linked dominant with male lethality. The ip gene is in band q28 on the x chromosome. Mothers with ip have an equal chance of having a normal or ip daughter or a normal son. The ip sons die before birth. Ip is also known as bloch-sulzberger syndrome.
    Please select first two letters of word you are looking for BA BB BC BD ... BZ fiSearchFormMaxSetId='AX006027'; Top Words blastocarpous
    blastocele

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    8. GASNet Anesthesiology: Bloch-Sulzberger Syndrome
    Bowen Syndrome (Cerebrohepatorenal Syndrome) symptoms and caveats bloch-sulzberger syndrome. - Cerebrohepatorenal Syndrome -
    http://gasnet.med.yale.edu/pediatric-syndromes/bowen.php

    9. Displaying Dictionary Terms Starting With Letter BL
    blistering blistering collodion blite bloat blob Bloch s reaction Bloch, Bruno Bloch, Marcel BlochSulzberger disease bloch-sulzberger syndrome block block
    http://www.dictionarybarn.com/dic-BL.php
    Browse Dictionary by alphabet A B C D ... Z Top Words bombastical
    basophil substance

    Bartley, Samuel

    Byzantine arch palate
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    biocytin

    Please select first two letters of word you are looking for BA BB BC BD ... BZ Displaying all terms starting with letter BL blab black black art black bass ... Blym Top Words belch
    blueberry

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    10. Incontinentia Pigmenti - Information / Diagnosis / Treatment / Prevention
    symptoms. ? National Library of Medicine blochsulzberger syndrome, the synonyms, a summary and major features. ? Readers Digest
    http://www.healthcyclopedia.com/genetic-disorders/incontinentia-pigmenti.html

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    Medical Definition: University of Newcastle-upon-Tyne Medical Dictionary: "incontinentia pigmenti"
    Health News: Search millions of published articles for news on Incontinentia Pigmenti Modern Medicine Aging The Ardell Wellness Report HealthFacts Medical Post Medical Update Men's Health and the National Women's Health Report Note: Subscription required to access the full text of articles. Web Directory: Incontinentia Pigmenti Informational sheet compiled by National Institute of Neurological Disorders and Stroke. Incontinentia Pigmenti International Foundation Details about the organization that is dedicated to research into the causes and treatment of this genetic disease. Includes information about the disease, genetics and genes. Incontinentia Pigmenti: All About Anna The authors' daughter was diagnosed with IP 6 weeks after birth. This is her story.

    11. Karger Publishers
    Background Incontinentia pigmenti (IP; blochsulzberger syndrome) is an inherited disorder of skin pigmentation that is associated with skin (100%), dental (90
    http://content.karger.com/ProdukteDB/produkte.asp?Aktion=ShowAbstract&ProduktNr=

    12. Incontinentia Pigmenti (Bloch-Sulzberger Syndrome)
    Incontinentia pigmenti (blochsulzberger syndrome) This disease is described in an article in GeneReviews. Copyright © 2002 SimulConsult®Inc. (- )
    http://www.simulconsult.com/resources/c0021171.html
    Incontinentia pigmenti (Bloch-Sulzberger syndrome)
    This disease is described in an article in GeneReviews SimulConsult Inc.

    13. EMedicine - Incontinentia Pigmenti : Article Excerpt By: Takuo Tsuji, MD
    Background Incontinentia pigmenti (IP), sometimes termed blochsulzberger syndrome, is an X-linked dominant single-gene disorder with neurologic
    http://www.emedicine.com/derm/byname/incontinentia-pigmenti.htm
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    Excerpt from Incontinentia Pigmenti
    Synonyms, Key Words, and Related Terms: IP, Bloch-Sulzberger syndrome, Bloch-Siemens syndrome, familial incontinentia pigmenti, IP2
    Please click here to view the full topic text: Incontinentia Pigmenti
    Background: Incontinentia pigmenti (IP), sometimes termed Bloch-Sulzberger syndrome, is an X-linked dominant single-gene disorder with neurologic, ophthalmologic, and dental involvement, as well as cutaneous findings. In 1906, Garrod probably reported the first case of IP and described it as a peculiar pigmentation of the skin in an infant. Subsequently, Bloch and Sulzberger further defined the condition as a clinical syndrome with a constellation of unique features, which include typical cutaneous lesions. Since then, a large number of small series and individual case reports have been published. Pathophysiology: IP is a disorder characterized by abnormalities of the tissues and organs derived from the ectoderm and neuroectoderm. The locus for IP is genetically linked to the factor VIII gene on chromosome band Xq28. Recently, Aradhya et al demonstrated that mutations in NEMO/IKK g , which encodes a critical component of the nuclear factor- k B (NF- k B) signaling pathway, are responsible for IP (Aradhya, 2001). Virtually all mutations eliminate the production of NF-

    14. Incontinentia Pigmenti
    pigmenti (IP2) Incontinentia pigmenti, also known as blochsulzberger syndrome, is a rare genodermatosis that usually appears at birth or shortly thereafter.
    http://www.edae.gr/incontinentia.html

    15. DERMATOLOGY DISEASES
    Behcet`s syndrome. blochsulzberger syndrome. Boils (Furuncles; Carbuncles). Incontinentia pigmenti (bloch-sulzberger syndrome). Juvenile xanthogranuloma.
    http://www.edae.gr/diseases.html
    HELLENIC ASSOCIATION DERMATOLOGY - VENEREOLOGY DERMATOLOGY DISEASES G. Karakatsanis, Dermatologist Acne Acrodermatitis Acrokeratosis Neoplastica (Bazex Syndrome) Adamantiades - Behcet`s syndrome ... Hyperhidrosis (excessive sweating) Ichthyosis Impetigo Impotence -Erectile Dysfuction Incontinentia pigmenti (Bloch-Sulzberger syndrome) ... Linear IgA dermatosis Livedo racemosa ( Sneddon`s syndrome Lupus Lyell`s Syndrome Lyme disease ... Skin Cancer ( Non Melanoma ) Sneddon`s syndrome Livedo racemosa) Sporotrichosis Squamous Cell Carcinoma Stevens Johnson Syndrome Syphilis ... Xeroderma Pigmentosum Miscellaneous

    16. BDJ Incontinentia Pigmenti. 6 Cases Of Bloch-Sulzberger Syndrome
    6, pages 195196. doi 10.1038/sj.bdj.4804779 Help. Incontinentia pigmenti. 6 cases of bloch-sulzberger syndrome. Burgess, MC. Abstract. No abstract available.
    http://www.nature.com/cgi-taf/dynapage.taf?file=/bdj/journal/v152/n6/abs/4804779

    17. Syndrome, Bloch-Sulzberger - Medical Dictionary Definitions Of Popular Medical T
    MedicineNet Home MedTerms medical dictionary AZ List Syndrome, BlochSulzberger. Advanced Search. IP is also known as bloch-sulzberger syndrome.
    http://www.medterms.com/script/main/art.asp?articlekey=5619

    18. Syndrome, Incontinentia Pigmenti - Medical Dictionary Definitions Of Popular Med
    inactivated. This phenomenon is termed nonrandom (or skewed) X-chromosome inactivation. IP is also known as bloch-sulzberger syndrome.
    http://www.medterms.com/script/main/art.asp?articlekey=5637

    19. Disease Directory : Genetic Disorders : Incontinentia Pigmenti
    Incontinentia Pigmenti Incontinentia pigmenti type 2, also known as bloch-sulzberger syndrome, is a rare, X-linked, dominantly inherited disorder of skin;
    http://www.diseasedirectory.net/Genetic_Disorders/Incontinentia_Pigmenti/default
    Wednesday, June 02, 2004 Genetic Disorders
    Aarskog Syndrome

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    Ablepharon-Macrostomia Syndrome
    ... Genetic Disorders : Incontinentia Pigmenti

    20. NINDS - Health And Medical
    Benign Focal Amyotrophy Benign Intracranial Hypertension BernhardtRoth Syndrome Binswanger s Disease Blepharospasm bloch-sulzberger syndrome Brachial Plexus
    http://www.ninds.nih.gov/health_and_medical/research.htm
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