Journal of cutaneous pathology, 2017-10, Vol.44 (10), p.887-891
2017
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- Autor(en) / Beteiligte
- Titel
- Lipoid proteinosis: A case with distinct histopathological and radiological findings
- Ist Teil von
- Journal of cutaneous pathology, 2017-10, Vol.44 (10), p.887-891
- Ort / Verlag
- Oxford, UK: Blackwell Publishing Ltd
- Erscheinungsjahr
- 2017
- Quelle
- MEDLINE
- Beschreibungen/Notizen
- Lipoid proteinosis is a rare inherited genodermatosis characterized by hyaline deposits in various tissues. Clinically, it manifests with cutaneous as well as extracutaneous features. Periodic acid‐Schiff (PAS)‐reactive hyaline deposits in the upper dermis, with localization around blood vessels and eccrine sweat glands, in particular, is the histopathological hallmark finding. On brain imaging, bilateral symmetrical temporal lobe calcifications are considered to be pathognomonic of this disorder. We report a case of lipoid proteinosis in which hyaline deposits were present in the papillary and reticular dermis, without being seen at the periphery of eccrine sweat glands, along with dystrophic calcification. Magnetic resonance imaging (MRI) of brain revealed hydrocephalus, subependymal heterotropia and absent splenium of corpus callosum with no evidence of temporal lobe calcification. Thus, our case highlights the inherent diverse nature of lipoid proteinosis.
- Sprache
- Englisch
- Identifikatoren
- ISSN: 0303-6987eISSN: 1600-0560DOI: 10.1111/cup.13002Titel-ID: cdi_proquest_miscellaneous_1917360909
- Format
- –
- Schlagworte
- Adult, Blood vessels, Calcification, Calcinosis - diagnostic imaging, Calcinosis - metabolism, Calcinosis - pathology, Case reports, Corpus callosum, Corpus Callosum - diagnostic imaging, Corpus Callosum - metabolism, Corpus Callosum - pathology, Deposits, Dermis, Dermis - metabolism, Dermis - pathology, dystrophic calcification, Eccrine Glands - metabolism, Eccrine Glands - pathology, eccrine sweat glands, Genodermatosis, Glands, Humans, Hyalin - metabolism, Hydrocephalus, Hydrocephalus - diagnostic imaging, Hydrocephalus - metabolism, Hydrocephalus - pathology, lipoid proteinosis, Lipoid Proteinosis of Urbach and Wiethe - diagnostic imaging, Lipoid Proteinosis of Urbach and Wiethe - metabolism, Lipoid Proteinosis of Urbach and Wiethe - pathology, Localization, Magnetic Resonance Imaging, Male, Neuroimaging, NMR, Nuclear magnetic resonance, Sweat, Temporal lobe, Temporal Lobe - diagnostic imaging, Temporal Lobe - metabolism, Temporal Lobe - physiology