1. NINDS Incontinentia Pigmenti Information Page Informational sheet compiled by National Institute of Neurological Disorders and Stroke.Category Health Conditions and Diseases incontinentia pigmenti......incontinentia pigmenti information sheet compiled by the National Institute ofNeurological Disorders and Stroke (NINDS). More about incontinentia pigmenti, http://www.ninds.nih.gov/health_and_medical/disorders/inconpig_doc.htm

National Institute of Neurological Disorders and Stroke Accessible version Science for the Brain The nation's leading supporter of biomedical research on disorders of the brain and nervous system Browse all disorders Browse all health organizations More about Incontinentia Pigmenti Studies with patients Research literature Press releases Search NINDS... (help) Contact us My privacy NINDS is part of the National Institutes of Health NINDS Incontinentia Pigmenti Information Page Synonym(s): Bloch-Sulzberger Syndrome Reviewed 11-02-2001 Get Web page suited for printing Email this to a friend or colleague 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. Dental and hair problems may be treated by a specialist. 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?

2. Incontinentia Pigmenti An article about this rare genetic disorder, its stages and symptoms. http://healthlink.mcw.edu/article/921770674.html

Search Articles: search tips Please Take the HealthLink Survey Email this article Print this article Find related articles: By topic: Neurology By keywords: Receive Health Link via email! Subscribe now >> Incontinentia Pigmenti Incontinentia pigmenti (IP) is a rare, genetic disorder characterized by unusual patterns of discolored skin. The disorder is caused by excessive deposits of melanin (normal skin pigment). IP is divided into 4 stages, which frequently overlap or appear together. During the first stage, which begins between birth and 6 months of age, there is inflammation accompanied by skin redness and spiral lines of small fluid-filled blisters. The second stage gradually develops with rough, warty skin growths which appear on the arms or legs and, sometimes, on the head or trunk. These growths, which are often arranged in the same spiral or linear pattern as in the first stage, usually resolve during infancy or early childhood. The third stage begins between 3 months and 2 years of age and is characterized by discolorations appearing in unusual patterns. The fourth stage consists of diminished pigmentation or atrophy in areas of previous discoloration. In rare cases of IP, hair loss with scarring and non-dermatological symptoms such as dental problems (delayed tooth growth or decay, missing or malformed teeth), diminished vision, seizures, muscle spasms, or slight paralysis may occur. Developmental abnormalities including dwarfism or short stature, club foot, spina bifida, skull and ear deformities, cleft lip or palate, atrophy on one side of the body, abnormal development of cartilage, congenital dislocation of hip, incomplete development of one side of the spinal bones, and extra ribs or webbed fingers may occur with the disorder but are not characteristic. In a few cases of IP, extremely wooly or kinky hair and an immune system dysfunction may also appear.

3. Nutrition - Incontinentia Pigmenti An article about incotinentia pigmenti.Category Health Conditions and Diseases incontinentia pigmenti......Nutrition. incontinentia pigmenti. Incontinentia Eisenhauer O'Brien J. IncontinentiaPigmenti A longitudinal study. AJDC 139 711712. Moss http://www.mc.vanderbilt.edu/peds/pidl/nutrit/incont.htm

PIDL Home/ Contents Development Nutrition Acute Illness ... Psychosocial Nutrition INCONTINENTIA PIGMENTI Incontinentia Pigmenti also called Bloch-Sulzberger syndrome or Bloch-Siemens syndrome is a complex hereditary disease with ectodermal, neurologic, ocular and musculoskeletal manifestations. It is an x-linked dominantly inherited disease, usually lethal in males. More than 95% of the reported cases are females, the few males being probably the result of spontaneous mutations. Chromosome studies have shown an increased frequency of aberrations and it has been suggested that this is a chromosomal breakage syndrome. Adolescents may have warty lesions around the nails with underlying lytic bone lesions. The pigmentation that was described as the third stage may be the only abnormality, it may be present first or may appear as the inflammatory lesions are subsiding, though not necessarily in the same sites. In addition, inflammatory lesions can develop in areas already pigmented. The pigmentation ranging in color from blue-grey or slate to brown is characteristic of the syndrome, and the bizarre "splashed" or "chinese figure" distribution is diagnostic. Occasionally, multiple linear and macular telangiectasia can be noted. The inflammatory lesions are uncommon after the age of 6 months but may be followed by atrophy or sclerosis. However, the pigmentation persists for many years, slowly fading until it is imperceptible by the second or third decade.

4. Incontinentia Pigmenti The authors' daughter was diagnosed with IP 6 weeks after birth. This is her story.Category Health Conditions and Diseases incontinentia pigmenti...... child. She was diagnosed with incontinentia pigmenti at 6 weeks ofage. This is her story, and the story of this condition. Please http://mom_2_three_ip.tripod.com/IP/

Get Five DVDs for $.49 each. Join now. Tell me when this page is updated Incontinentia Pigmenti Incontinentia Pigmenti... Other Effects of IP More Information Anna's Story ... Comments ALL ABOUT ANNA... This site is dedicated to my youngest child. She was diagnosed with Incontinentia Pigmenti at 6 weeks of age. This is her story, and the story of this condition. Please read on and when you are finished... feel free to contact me for questions, advice, etc. I am hoping that this site will help other families that are trying to deal with Incontinentia Pigmenti. I will try to cover most bases dealing with symptoms and give my thoughts as well. Like I said before, I am looking forward to hearing from MANY of you out there!!! What's New? December 22, 2001: Yes! I managed to upload all the pictures I currently have available to share with you. There are so many, I had to make 2 pages. Hope you take the time to look at them. Also, PLEASE take the time to sign the guestbook. It is located on the last page "CONTACT ME" in the gold/orange box that says "VIEW/SIGN GUESTBOOK" just give it a click!!! Thanks! January 3, 2003

5. Incontinentia Pigmenti - 1 incontinentia pigmenti is an Xlinked dominant disorder that presents in at or soon after birth. http://tray.dermatology.uiowa.edu/InconPig01.htm

Dept. of Dermatology - University of Iowa College of Medicine Incontinentia Pigmenti - Lower leg - 3 week old baby girl. To see representative histology, click Here Return to Image Index page. Return to Image Index page. Return to Dermatology's Home page. November, 1996

6. Incontinentia Pigmenti incontinentia pigmenti. Subject lncontinentia Pigmenti Titleincontinentia pigmenti Author(s) Journal Book Vol. http://med-aapos.bu.edu/PediRef/incont_pigment.html

Incontinentia Pigmenti Subject: lncontinentia Pigmenti Title:Incontinentia Pigmenti Author(s): Journal: Book: Vol.: Date: 1990 Pages: Organization: National Organization for Rare Disorders, Inc. Address: P.O. Box 8923; 100 Route 37 City: New Fairfield State: CT Zip: 06812-1783 Phone: (203) 746-6518 Type: Fact sheets You can send E-Mail to the AAPOS Web Server directly from here. Please send comments and suggestions. You may also attach electronic files to e-mail. Please send us feedback Last Modified by Rick Blocker on 3/10/95 Press This To Return Pedi Resource Home Page

7. Incontinentia Pigmenti : Sites Et Documents Francophones incontinentia pigmenti Menu général CISMeF Synonyme(s) bloch sulzberger, syndrome ; dermatose pigmentaire eclaboussures ; naevus chromatophore hereditaire ; syndrome bloch sulzberger . Ne pas confondre avec troubles pigmentation . Arborescence(s) du thesaurus MeSH contenant le motclé incontinentia pigmenti incontinentia pigmenti http://www.chu-rouen.fr/ssf/pathol/incontinentiapigmentia.html

Incontinentia Pigmenti Menu général CISMeF Synonyme(s) bloch sulzberger, syndrome ; dermatose pigmentaire eclaboussures ; naevus chromatophore hereditaire ; syndrome bloch sulzberger Ne pas confondre avec troubles pigmentation Arborescence(s) incontinentia pigmenti incontinentia pigmenti maladies et malformations congénitales, héréditaires et néonatales maladies peau et tissu conjonctif Position du mot-clé dans l' (les) arborescence(s) : Vous pouvez consulter toutes les ressources ou seulement les principales ou utiliser l' outil de recherche les recommandations les documents concernant l' enseignement les documents destinés aux patients Ou consulter ci-dessous une sélection des principales ressources : patient IPF - association Incontinentia Pigmenti France [Site éditeur Orphanet mots clés : France incontinentia pigmenti type(s) : association patients 20 janvier 2003 courriel Menu général CISMeF Haut de page © CHU de Rouen . Toute utilisation partielle ou totale de ce document doit mentionner la source.

8. Incontinentia Pigmenti International Foundation This is the official site of the incontinentia pigmenti International Foundation,a charitable foundation dedicated to research into the causes and treatment http://imgen.bcm.tmc.edu/IPIF/

Susanne Bross Emmerich, Executive Director 30 East 72nd St, New York, N.Y. 10021 U.S.A. Tel: 212 452-1231 Fax: 212 452-1406 E-Mail: ipif@ipif.org Web Site: http://imgen.bcm.tmc.edu/IPIF Foundation Links Incontinentia Pigmenti Links Genetics and IP Gene Discovery Links Letter from the Founder and Executive Director Description of Incontinentia Pigmenti What is a gene? What is important about the discovery of the gene called NEMO? ... Support Groups Participate in the IP Natural History Survey Project Where to be tested Translate: Francais , Deutsches, Italiano

9. NIPF Redirect Provides a mission statement and research goals. Describes common clinical features of the disorder. Foundation Links. incontinentia pigmenti Links. Genetics and IP http://www.medhelp.org/www/nipf.htm

Your Web Browser Should now take you to the new location for NIPF.Click on this link if it doesn't: http://imgen.bcm.tmc.edu/IPIF

10. IPF France Pr©sentation de la maladie, de lassociation et de ses actions, actualit©s et liens. http://www.orpha.net/nestasso/IPF/

11. 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. http://imgen.bcm.tmc.edu/ipif

Susanne Bross Emmerich, Executive Director 30 East 72nd St, New York, N.Y. 10021 U.S.A. Tel: 212 452-1231 Fax: 212 452-1406 E-Mail: ipif@ipif.org Web Site: http://imgen.bcm.tmc.edu/IPIF Foundation Links Incontinentia Pigmenti Links Genetics and IP Gene Discovery Links Letter from the Founder and Executive Director Description of Incontinentia Pigmenti What is a gene? What is important about the discovery of the gene called NEMO? ... Support Groups Participate in the IP Natural History Survey Project Where to be tested Translate: Francais , Deutsches, Italiano

12. Incontinentia Pigmenti... incontinentia pigmenti, This is the page where I will talk about thestatistics of incontinentia pigmenti (IP). Here are some important http://mom_2_three_ip.tripod.com/IP/id1.html

Get Five DVDs for $.49 each. Join now. Tell me when this page is updated Incontinentia Pigmenti... Home Incontinentia Pigmenti... Other Effects of IP More Information Anna's Story My Thoughts... ... Comments Incontinentia Pigmenti This is the page where I will talk about the statistics of Incontinentia Pigmenti (IP). Here are some important statistics before I get started: * Garrod, in 1906, may have been the first one to diagnose IP! * IP is a rare genetic disorder characterized by unusual patterns of discolored skin, a type of rash. * IP is catagorized in stages: 1st stage: Lasts from Birth to about 6 months: Inflammation, redness, spiral (or linear) patterns with small blisters (lesions, bullae, vesicles) filled with honey-colored, sticky fluid. They burst and ooze, then crust over. The crusts fall off and redness remains. The process repeats itself numerous times. 3rd stage: Three months to 2 years... Discolorations appear in odd patterns, (lighter or darker patches of skin). 4th stage: Diminished (loss of) pigmentation or atrophy in areas of previous discoloration. This is what one of the lesions looked like while it was in the 1st stage. I am in the process of getting some pictures that her doctor took. Will update when I get them.

13. Rd.com: A general discussion about incontinentia pigmenti, with alternate names and further resources. http://www.rd.com/common/nav/index.jhtml?articleId=8612393

14. Incontinentia Pigmenti - 3 Dept. of Dermatology University of Iowa College of Medicine. IncontinentiaPigmenti - Left upper leg - 1 week old baby girl. To http://tray.dermatology.uiowa.edu/InconPig03.htm

Dept. of Dermatology - University of Iowa College of Medicine Incontinentia Pigmenti - Left upper leg - 1 week old baby girl. To see representative histology, click Here Return to Image Index page. Return to Image Index page. Return to Dermatology's Home page. November, 1996

15. Incontinentia Pigmenti incontinentia pigmenti is an Xlinked dominant disorder that presentsin at or soon after birth. Although the condition involves http://dermatology.cdlib.org/DOJvol4num1/path/incont2.html

DOJ Index Spanish Portuguese Answer: Incontinentia Pigmenti Figure 3 Figure 4 Skin biopsy demonstrating marked edema of the upper epidermis Epidermal spongiosis and an eosinophil-rich cellular infiltrate Histologically, there is spongiosis manifest as epidermal intercellular edema with exocytosis of numerous eosinophils and mononuclear cells both within the epidermis as well as in spongiotic foci. Dyskeratotic keratinocytes are present adjacent to spongiotic microvesicles. An interstitial infiltrate of lymphocytes and eosinophils is present in the papillary dermis. Incontinentia pigmenti is an X-linked dominant disorder that presents in at or soon after birth. Although the condition involves the skin primarily, there are a number of associated disorders including dental defects, convulsive disorders, mental retardation, ocular abnormalities and childhood neoplasms. Because it is almost always lethal in males, is observed almost exclusively in female infants. Three successive stages of the cutaneous disease have been described. The first stage is characterized by a linear, erythematous, vesiculobullous eruption on the trunk and extremities within the first 2 weeks of life.[2] Histologically, spongiosis containing numerous eosoinophils with abundant dyskeratotic keratinocytes is noted.[1,3] The second stage, the so-called verrucous stage, usually develops within a few weeks and is manifest as verrucous hyperkeratotic papules and plaques. [2] Microscopically, there is acanthosis, papillomatosis, and hyperkeratosis with abundant dyskeratotic cells, often in small whorls in the epidermis.[3] Vacuolar change of the basal layer may be noted. Finally, as lesions resolve, the hyperpigmented stage develops in which there is a whorled slate-gray to brown hyperpigmentation that usually fades with time.[2] These areas contain many melanophages in the upper dermis.[3]

16. Incontinentia Pigmenti Translate this page incontinentia pigmenti, in Arndt KA, LeBoit PE, Robinson JK, Wintroub BU(eds)Cutaneous Medicine and Surgery An Integrated Program in Dermatology. http://dermatology.cdlib.org/DOJvol4num1/path/incont2-esp.html

DOJ Index Spanish Portuguese Respuesta: Incontinencia pigmenti Figura 3 Figura 4 Biopsia de piel mostrando marcado edema en epidermis superior. Espongiosis e infiltrado rico en eosinófilos. Histológicamente se observa espongiosis con edema intercelular y exocitosis de numerosos eosinófilos y células mononucleares, tanto en la epidermis como en los focos espongióticos. Se identifican queratinocitos disqueratósicos adyacentes a las microvesículas espongióticas. En la dermis papilar se observa un infiltrado intersticial de linfocitos y eosinófilos. La incontinencia pigmenti es una enfermedad que se transmite con carácter dominante ligado al cromosoma X, y que se manifiesta en el nacimiento o poco después. Aunque afecta primariamente a la piel, existen diferentes trastornos asociados, incluyendo defectos dentales, trastornos convulsivos, retraso mental, anomalías oculares y neoplasias infantiles. Dado que suele ser letal en varones, se observa casi exclusivamente en niñas. Han sido descritos tres estadios clínicos cutáneos. El primer estadio se caracteriza por una erupción lineal, eritematosa, vesículo-ampollosa en tronco y extremidades dentro de las dos primeras semanas de vida (2). Histológicamente se observa espongiosis con numerosos eosinófilos y queratinocitos disqueratósicos (1,3). El segundo estadio, denominado verrucoso, en general se desarrolla a las pocas semanas y se manifiesta con pápulas y placas verrugosas hiperqueratósicas (2). Microscópicamente se observa acantosis, papilomatosis e hiperqueratosis con abundantes células disqueratósicas, a menudo dispuestas en remolinos en la epidermis (3). Puede observarse alteración vacuolar de la basal. Finalmente y a medida que se resuelven estas lesiones, se desarrolla el estadio pigmentario con hiperpigmentación en espirales de tonalidad grisácea a marrón que generalmente se desvanece con el tiempo (2). Estas áreas contienen abundantes melanófagos en la dermis superior (3).

17. Incontinentia Pigmenti Image that's a link to Genetics Education Center Support Page IncontinentiaPigmenti National incontinentia pigmenti Foundation 30 East 72nd St. http://www.kumc.edu/gec/support/incontin.html

Incontinentia Pigmenti National Incontinentia Pigmenti Foundation 30 East 72nd St. New York, N.Y. 10021 U.S.A. Phone: 212.452.1231 Fax: 212.452.1406 E-mail: nipf@pipeline.com Web Site: http://imgen.bcm.tmc.edu/NIPF International Incontinentia Pigmenti Research Consortium Also See: National organizations , genetic conditions resources Incontinentia Pigmenti , National Institute of Neurological Disorders and Stroke Incontinentia Pigmenti , HealhLink National Eye Institute , NIH National Institute of Arthritis and Musculoskeletal and Skin Diseases , NIH Hypomelanosis of Ito Support Group , UK To locate a genetic counselor or clinical geneticist: Professional Genetics Societies Genetic clinics, centers, departments Revised March 15, 2001 Genetic Societies Clinical Resources Labs Clinics ... Search Genetics Education Center Debra Collins, M.S. CGC , Genetic Counselor, dcollins@kumc.edu This site subscribes to the principles of the HONcode (Health on the Net, Code of Conduct for Medical and Health Web Sites) of the Health On the Net Foundation

18. The Family Village / Incontinentia Pigmenti Library I J. incontinentia pigmenti. Synonym Block-Siemens-Suizerger Syndrome. http://www.familyvillage.wisc.edu/lib_inco.htm

Incontinentia Pigmenti Synonym: Block-Siemens-Suizerger Syndrome Who to Contact Where to Go to Chat with Others Learn More About It Web Sites ... Search AltaVista for "Incontinentia Pigmenti" or "Block-Siemens-Suizerger Syndrome" Who to Contact National Incontinentia Pigmenti Foundation 30 East 72nd Street 16 floor New York, NY 10021 212 452-1406 (fax) E-mail: nipf@pipeline.com Web: http://imgen.bcm.tmc.edu/NIPF/ Where to Go to Chat with Others UseNet News Group: alt.support.skin-diseases Learn More About It Incontinentia Pigmenti Incontinentia Pigmenti information from OMIM Incontinentia Pigmenti Type II information from OMIM Incontinentia Pigmenti Mini Information Sheet ... Incontinentia Pigmenti Vanderbilt Medical Center Web Sites National Incontinentia Pigmenti Foundation The International Incontinentia Pigmenti Research Consortium Back to [ I - J Family Village Home Library Coffee Shop ... Information Last Updated August-7-2001 by familyvillage@waisman.wisc.edu Document Source: http://www.familyvillage.wisc.edu/lib_inco.htm

19. INCONTINENTIA PIGMENTI Incontinentia http://digilander.libero.it/camdic/INCPIG.html

INCONTINENTIA PIGMENTI Prof. Camillo O. DI CICCO Member of " DNA REPAIR INTEREST GROUP " ( NIH ) National Insitutes of Health Bethesda, Maryland La sintomatologia cutanea, nella sua forma classica evolve in tre stadi: I° STADIO con presenza di lesioni di tipo eritemato-vescico-bolloso a gittate successive, disposizione lineare ed interessamento del tronco e degli arti; presente, inoltre, ipereosinofilia ematica. II° STADIO si evidenzia tra la seconda e la sesta settimana di vita con lesioni papulo lichenoidi, ipercheratosiche, verrucose, con aspetto di strie allungate nella parte distale degli arti (ginocchio, dorso del piede e della mano). III° STADIO al 3°- 6° mese di vita compaiono macule pigmentate brune a livello del tronco con disposizione a vortice, a turbine, a schizzi. Tale cronologia può non essere rispettata ed inoltre non è chiarito il rapporto tra il susseguirsi delle lesioni. - Anomalie neurologiche nel 30% dei casi, con interessamento motorio-spastico, convulsioni, deficit intellettivo. - Anomalie oculari nel 35% dei casi, con cecità nel 7,5% (cataratta, retinite, uveite, atrofia del nervo ottico).

20. INCONTINENTIA PIGMENTI Features Listed For incontinentia pigmenti. McKusick 308300. Abnormally shapedteeth; Absent or hypoplastic breasts; Aplasia or dysplasia of retina; http://www.hgmp.mrc.ac.uk/dhmhd-bin/hum-look-up?848

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