Infectious Diseases In Children Answer. This case turned out to be incontinentia pigmenti (IP), an Xlinked dominantdisorder with mortality in males, also known as bloch-sulzberger syndrome. http://idinchildren.com/200104/WYD.asp
Extractions: April 2001 Physical exam confirmed normal vital signs and revealed a mildly jaundiced infant with an erythematous, vesicular rash in whorl patterns over the parietal and occipital area of the scalp, the trunk and inner thighs, sparing the face (figures 1-6). The remainder of the exam was normal. The patient had viral, bacterial and fungal cultures and stains performed on the lesions, and was empirically treated with intravenous acyclovir for the possibility of neonatal HSV infection. Bacterial and fungal stains were negative. The patient was monitored in the NICU and otherwise treated normally. Dermatology obtained a biopsy of one of the lesions that showed numerous eosinophils within the superficial dermis and an area of hyperkeratosis. Answer This case turned out to be incontinentia pigmenti (IP), an X-linked dominant disorder with mortality in males, also known as Bloch-Sulzberger syndrome. Females with IP are mosaics. Variable presentation among patients is thought to be due to Lyonization. The term IP describes the histologic feature where there is incontinence of the melanocytes in the basal layer of the epidermis into the superficial dermis.
THE LIGHTNING HYPERTEXT OF DISEASE. Packet No. 16 27255 INCONTINENTIA PIGMENTI =ip blochsulzberger syndrome incontinentiapigmenti type i ip1 incontinentia pigmenti sporadic type key words http://www.pathinfo.com/cgi-bin/lh.cgi?tx=incontin
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Incontinentia Pigmenti Landy SJ, Donnai D. Incontinentia pigmenti (blochsulzberger syndrome). J MedGenet 1993;3053-9. Nelson-Adesokan P, Mallory SB. Incontinentia pigmenti. http://www.kfshrc.edu.sa/annals/205_206/00-070.htm
Extractions: Incontinentia Pigmenti Sultan Al-Khenaizan, MBBS, FRCPC Incontinentia pigmenti (IP) is an X-linked dominant (XLD) disorder that was first described in 1906 by Garrod, and subsequently named after Bloch and Sulzberger. The skin lesions in IP follow four well-characterized successive stages: vesicular, verrucous, whorl or streak-like hyperpigmentation, and hypopigmented scars. Skin appendages, including hair and teeth are commonly affected in IP, with scarring alopecia and peg-shaped teeth. IP also affects many other body systems, most commonly the central nervous system (CNS) and the eyes. Almost all patients with IP are females because of the lethality of the disease in males. Case Report From the Department of Medicine, King Fahad National Guard Hospital, Riyadh, Saudi Arabia. leaving no marks. The rest of the physical examination was unremarkable. Skin examination of the mother revealed multiple white, hypopigmented atrophic swirls and streaks along the Blaschko lines. Mouth examination revealed hypodontia and conical widely spaced teeth. The diagnosis of incontinentia pigmenti was made. CT scan and MRI of the brain revealed no abnormality. Electroencephalogram study was normal and there were no more seizures. The infant was discharged home in good condition on oral phenobarbitol. At three months' follow-up, the pigmentation had extended to most of the lower extremities and sides of the trunk (Figure 1), with no other skin changes. Neurological examination by the neurologist was normal.
WebMD - Siemens Incontinentia Pigmenti Melanoblastosis Cutis Linearis nord BlochSiemens-SulzbergerSyndrome nord bloch-sulzberger syndrome nord Blocked Artery http://my.webmd.com/content/healthwise/47/11580.htm
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Genetic KID syndrome keratitis, ichthyosis, and deafness. Incontinentia pigmenti(blochsulzberger syndrome) X linked dominant (97% females - males die); http://www.visioneyedoctor.com/genetic1.htm
INCONTINENTIA PIGMENTI (IP1); gene ? F M (201); lethal in males. PATHOGENESIS 1. Background. firstdescribed by M. Bardach in 1925; also called blochsulzberger syndrome or Disease; http://www.icondata.com/health/pedbase/files/INCONTIN.HTM
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