Lipoid proteinosis in the eastern Mediterranean region of Turkey

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dc.contributor.authorDogramaci, Asena C.
dc.contributor.authorCelik, Murat M.
dc.contributor.authorCelik, Ebru
dc.contributor.authorBayarogullari, Hanifi
dc.date.accessioned2024-09-18T20:11:47Z
dc.date.available2024-09-18T20:11:47Z
dc.date.issued2012
dc.departmentHatay Mustafa Kemal Üniversitesien_US
dc.description.abstractBackground: Lipoid proteinosis (LP), also known as hyalinosis cutis et mucosae or Urbach-Wiethe disease, is a rare autosomal recessive genodermatosis. Hyalin-like material is deposited in multiple organs, including the skin, oral mucosa, larynx, and brain. Only about 300 cases have been reported in the literature so far. Occurrence of LP in siblings is also rare. The reasons for relatively large number of cases, the clinical features of the patients, and the association of LP with other clinical conditions are described in this article. Aims: The aim of this study is to show that LP is not rare in Turkey and consanguineous marriage is still a social problem. Methods: We included patients between the years of 2008-2011 with lesions resembling LP. Based on the findings of clinical and histological examination of all cases, a diagnosis of LP was made. Results: We presented six different families with multiple family members suffered from LP. In total, we determined eight children and six adult patients. Three of eight children were from the same family (sisters), the other three children belonged to another family (brothers), two children were from another family (brother and sister), and the adult patients were from three different families. Conclusions: Patients with LP are likely to present first to a dermatologist because of the appearance of their skin; therefore, it is important that the dermatologic diagnosis is not to be missed. We described patients with LP and discuss the salient features of this disease.en_US
dc.identifier.doi10.4103/0378-6323.95447
dc.identifier.endpage322en_US
dc.identifier.issn0378-6323
dc.identifier.issue3en_US
dc.identifier.pmid22565431en_US
dc.identifier.scopus2-s2.0-84862078276en_US
dc.identifier.scopusqualityQ3en_US
dc.identifier.startpage318en_US
dc.identifier.urihttps://doi.org/10.4103/0378-6323.95447
dc.identifier.urihttps://hdl.handle.net/20.500.12483/9073
dc.identifier.volume78en_US
dc.identifier.wosWOS:000304084200010en_US
dc.identifier.wosqualityQ3en_US
dc.indekslendigikaynakWeb of Scienceen_US
dc.indekslendigikaynakScopusen_US
dc.indekslendigikaynakPubMeden_US
dc.language.isoenen_US
dc.publisherMedknow Publicationsen_US
dc.relation.ispartofIndian Journal of Dermatology Venereology & Leprologyen_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/openAccessen_US
dc.subjectConsanguineous marriagesen_US
dc.subjectgenodermatosisen_US
dc.subjectlipoid proteinosisen_US
dc.subjectTurkeyen_US
dc.titleLipoid proteinosis in the eastern Mediterranean region of Turkeyen_US
dc.typeArticleen_US

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