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Topic: Steatocystoma multiplex natal teeth

	Steatocystoma multiplex
		Steatocystoma multiplex is an uncommon, inherited disorder that is characterized by multiple, asymptomatic, variably-sized dermal cysts.
		Hybrid cysts showing alternate combination of eruptive vellus hair cyst, steatocystoma multiplex, and epidermoid cyst, and an association among the three conditions.
		Tomkova H; Fujimoto W; Arata J. Expression of keratins (K 10 and K 17) in steatocystoma multiplex, eruptive vellus hair cysts, and epidermoid and trichilemmal cysts.
	dermatology.cdlib.org /DOJvol6num1/NYUcases/steatocystoma/steatocystoma.html (571 words)

	Brazilian Dental Journal: Primo et al: Interruption of Breast Feeding Caused by the Presence of Neonatal Teeth (Site not responding. Last check: 2007-11-06)
		According to Massler and Savara (1950), teeth which are present in the oral cavity at birth are called natal teeth, and those which erupt during the first thirty days of life are called neonatal teeth.
		The preferred procedure is to maintain the teeth in place, and explain to the parents the importance of the teeth in the development and normal eruption of adjacent teeth (McDonald and Avery, 1994).
		Due to the long period between the appearance of the teeth and the parents' first visit to the clinic, the fragile structures of the teeth were already deformed causing pain, bleeding, and mobility.
	www.forp.usp.br /bdj/t1062.html (2016 words)

	White : Diseases of the Skin - Pediatric Dermatology - GENODERMATOSES
		One may suspect PC-2 in the newborn with natal teeth and milia-like cysts.
		The deciduous teeth develop normally, but then destruction of the periodontum, purulent gingivitis, and a fetid mouth odor accompany tooth loss, which is often complete by age 4–5 years.
		With the eruption of the permanent teeth, the process is repeated.
	www.merckmedicus.com /ppdocs/us/hcp/content/white/chapters/white-ch-015-s005.htm (4551 words)

	KRT17 - keratin 17
		Epidermoid cysts expressed K10 and eruptive vellus hair cysts expressed K17, whereas trichilemmal cysts and steatocystoma multiplex showed expression of both K10 and K17.
		Therefore, CRES is a new cross-class inhibitor that may regulate PC2 of PC2-like proteases and suggests a role for CRES in the regulation of prohormone and proprotein processing.
		We compared the patterns of keratin 10 (K10) and keratin 17 (K17) expression in epidermoid cysts, trichilemmal cysts, eruptive vellus hair cysts, and steatocystoma multiplex.
	www.ihop-net.org /UniPub/iHOP/gg/89742.html (5725 words)

	Human protein: Q04695 - Keratin, type I cytoskeletal 17 (Cytokeratin-17) (CK-17) (Keratin-17) (K17) (39.1). EMBL ...
		Missense mutations in keratin 17 cause either pachyonychia congenita type 2 or a phenotype resembling steatocystoma multiplex.
		Keratin 17 mutations cause either steatocystoma multiplex or pachyonychia congenita type 2.
		PC2 is characterized by onchyogryposis, limited plantar hyperkeratosis, multiple epidermal cysts, abnormal eyebrow and body hair and by the presence of natal teeth.
	harvester.embl.de /harvester/Q046/Q04695.htm (1269 words)

	HS-USA :: Abstract Viewer (Site not responding. Last check: 2007-11-06)
		(24,37) hidradenitis suppurativa has also been described in patients with pityriasis rubra pilaris, (46) acanthosis nigricans, (47) and steatocystoma multiplex, (48) associations emphasizing the potential importance of follicular occlusion in the pathogenesis of hidradenitis suppurativa.
		Un inflamed cysts, alone or as part of the steatocystoma multiplex syndrome, should present no diagnostic problems.
		McDonald RM, Reed WB: Natal teeth and steatocystoma multiplex complicated by hidradenitis suppurativa.
	www.hs-usa.org /abstracts/abstract_viewer.asp?ID=93 (3695 words)

	A.F.R.H., Hidrosadénite suppurée, maladie de Verneuil (Site not responding. Last check: 2007-11-06)
		We suggest an association between this type of pachyonychia congenita and hidradenitis suppurativa.
		Natal teeth and steatocystoma multiplex complicated by hidradenitis suppurativa.
		McDonald RM, Reed WB A new syndrome, consisting of natal or defective teeth, or both, steatocystomas of the skin, and epidermal cysts of the scalp, is described in several generations.
	www.afrh.fr /web-content/Pachyonychie.html (123 words)

	eMedicine - Keratosis Palmaris et Plantaris : Article by Robert Lee, MD, PhD (Site not responding. Last check: 2007-11-06)
		The second is keratodermas with associated features such as lesions of nonvolar skin, hair, teeth, nails, sweat glands, and/or with abnormalities of other organs.
		No significant correlation has been demonstrated between the level of periodontal infection and the severity of skin affections, which supports the concept that these major components of this syndrome are unrelated to each other.
		Natal or neonatal teeth and multiple pilosebaceous cysts (steatocystoma multiplex) are also present and help distinguish this type from type 1.
	www.emedicine.com /derm/topic589.htm (4732 words)

	Pachyonychia congenita, type II
		The mother reported that her daughter had natal teeth.
		The type II (Jackson-Lawler) syndrome involves mutations in keratin 17 and clinically resembles the type I syndrome with the additional findings of both natal teeth and steatocystoma multiplex.
		Additionally, the type II syndrome displays less severe palmoplantar keratoderma with milder or absent oral lesions.
	dermatology.cdlib.org /94/NYU/Jan2002/8.html (502 words)

	KRT16 - keratin 16 (focal non-epidermolytic palmoplantar keratoderma)
		By following the assembly of K6 and K16 in vitro and in cultured cells, we find that relative to K5 and K14, a well-characterized keratin pair that is constitutively expressed in epidermis, K6 and K16 polymerize into short 10-nm filaments that accumulate near the nucleus, a property arising from K16.
		We have shown that GCs repress the expression of the basal-cell-specific keratins K5 and K14 and disease-associated keratins K6, K16, and K17 but not the differentiation-specific keratins K3 and K10 or the simple epithelium-specific keratins K8, K18, and K19.
		These observations significantly extend the correlation between K6, K16, and K17 expression and epithelial wound closure, and provide direct evidence that expression of these keratins, K17 in particular, is important for the timeliness of this process.
	www.ihop-net.org /UniPub/iHOP/gg/89738.html (5795 words)

	eMedicine - Pachyonychia Congenita : Article by Aleksej Kansky, MD, PhD (Site not responding. Last check: 2007-11-06)
		It is differentiated from leukoplakia or cancer either by performing an oral biopsy or by recognizing its presence in patients with other expressions of PC.
		Steatocystoma multiplex is generally considered a hallmark for PC-2, but according to the author's observations and data from the literature (Ward, 2003), it may also appear later in life in patients with PC-1.
		The symptoms are similar to those in PC-1; however, PC-2 is characterized by natal teeth and the consistent presence of steatocystoma multiplex.
	www.emedicine.com /DERM/topic812.htm (3268 words)

	Faculty of Dentistry, University of Hong Kong.
		King N.M. and Lee A.M.P., An intranasal tooth in a patient with a cleft lip and palate: report of a case.
		King N.M. and Lee A.M.P., Natal teeth and steatocystoma multiplex: a newly recognized syndrome.
		Yuen S.W.H., Chan J.C.Y. and Wei S.H.Y., Double primary teeth and their relationship with the permanent successors -a radiographic study of 376 cases.
	www.facdenthk.org /genpub.asp?year=1987&type=journal (567 words)

	DermAtlas: Online Dermatology Image Library dermatology image,Pseudomonas aeruginosa infection,nail dystrophy,green ... (Site not responding. Last check: 2007-11-06)
		Type I (Jadassohn-Lewandowsky Syndrome) shows hyperkeratotic nails with frequent paronychia, folicular hyperkeratosis of the elbows and knees, oral leukokeratosis and palmar plantar hyperkeratosis with hyperhidrosis.
		Type II (Jackson-Lawler Syndrome) additionally displays natal teeth and steatocystoma multiplex.
		Type III (Schafer-Braunauer Syndrome) also resembles type I with the addition of leukokeratosis of the corneas.
	www.dermatlas.org /derm/result.cfm?Diagnosis=10 (939 words)

	UniProtKB/Swiss-Prot entry Q04695 [K1C17_HUMAN] Keratin, type I cytoskeletal 17
		Smith F.J.D. Corden L.D. Rugg E.L. Ratnavel R. Leigh I.M. Moss C. Tidman M.J. Hohl D. Huber M. Kunkeler L. Munro C.S. Lane E.B. McLean W.H.I. "Missense mutations in keratin 17 cause either pachyonychia congenita type 2 or a phenotype resembling steatocystoma multiplex.";
		Covello S.P. Smith F.J.D. Sillevis Smitt J.H. Paller A.S. Munro C.S. Jonkman M.F. Uitto J. McLean W.H.I. "Keratin 17 mutations cause either steatocystoma multiplex or pachyonychia congenita type 2.";
		Celebi J.T. Tanzi E.L. Yao Y.J. Michael E.J. Peacocke M. "Mutation report: identification of a germline mutation in keratin 17 in a family with pachyonychia congenita type 2.";
	www.expasy.org /uniprot/Q04695 (698 words)

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