Could “islets of sparing” be a clue for neutral lipid storage disease with ichthyosis in patients with congenital ichthyosiform erythroderma?
The prevalent form of ichthyosis in neutral lipid storage disease (NLSDI) is nonbullous congenital ichthyosiform erythroderma (CIE) characterized by fine, whitish scales on erythematous skin over the whole body. Here, we report a late-diagnosed, 25-year-old woman with NLSDI presenting with diffuse e...
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| Format: | Article |
| Language: | English |
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Wolters Kluwer Medknow Publications
2022-01-01
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| Series: | Indian Journal of Dermatology |
| Subjects: | |
| Online Access: | http://www.e-ijd.org/article.asp?issn=0019-5154;year=2022;volume=67;issue=5;spage=579;epage=582;aulast=Demir |
| _version_ | 1797954988478038016 |
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| author | Filiz Topaloglu Demir Baran Cayhan Cagla Karaoglan Zafer Turkoglu Nesimi Buyukbabani Mesut Ayer Bugrahan Duz Bulent Uyanık |
| author_facet | Filiz Topaloglu Demir Baran Cayhan Cagla Karaoglan Zafer Turkoglu Nesimi Buyukbabani Mesut Ayer Bugrahan Duz Bulent Uyanık |
| author_sort | Filiz Topaloglu Demir |
| collection | DOAJ |
| description | The prevalent form of ichthyosis in neutral lipid storage disease (NLSDI) is nonbullous congenital ichthyosiform erythroderma (CIE) characterized by fine, whitish scales on erythematous skin over the whole body. Here, we report a late-diagnosed, 25-year-old woman with NLSDI presenting with diffuse erythema and fine whitish scales throughout the body with patches of apparently normal skin, “islets of sparing” on her lower extremities. We observed that the size of the normal skin islets changed with time, and even the entire lower extremity was covered with erythema and desquamation like the rest of the body. Frozen section histopathological examinations were made from lesional skin and normal-looking skin; no difference was observed in terms of lipid accumulation. The only noticeable difference was the thickness of the keratin layer. In CIE patients, observation of patches of apparently normal skin or “islets of sparing” might be a clue for NLSDI to be distinguished from other CIE conditions. |
| first_indexed | 2024-04-10T23:27:15Z |
| format | Article |
| id | doaj.art-4bddeaeb96ac406e8c63378ff3bcb024 |
| institution | Directory Open Access Journal |
| issn | 0019-5154 1998-3611 |
| language | English |
| last_indexed | 2024-04-10T23:27:15Z |
| publishDate | 2022-01-01 |
| publisher | Wolters Kluwer Medknow Publications |
| record_format | Article |
| series | Indian Journal of Dermatology |
| spelling | doaj.art-4bddeaeb96ac406e8c63378ff3bcb0242023-01-12T11:31:44ZengWolters Kluwer Medknow PublicationsIndian Journal of Dermatology0019-51541998-36112022-01-0167557958210.4103/ijd.ijd_1154_20Could “islets of sparing” be a clue for neutral lipid storage disease with ichthyosis in patients with congenital ichthyosiform erythroderma?Filiz Topaloglu DemirBaran CayhanCagla KaraoglanZafer TurkogluNesimi BuyukbabaniMesut AyerBugrahan DuzBulent UyanıkThe prevalent form of ichthyosis in neutral lipid storage disease (NLSDI) is nonbullous congenital ichthyosiform erythroderma (CIE) characterized by fine, whitish scales on erythematous skin over the whole body. Here, we report a late-diagnosed, 25-year-old woman with NLSDI presenting with diffuse erythema and fine whitish scales throughout the body with patches of apparently normal skin, “islets of sparing” on her lower extremities. We observed that the size of the normal skin islets changed with time, and even the entire lower extremity was covered with erythema and desquamation like the rest of the body. Frozen section histopathological examinations were made from lesional skin and normal-looking skin; no difference was observed in terms of lipid accumulation. The only noticeable difference was the thickness of the keratin layer. In CIE patients, observation of patches of apparently normal skin or “islets of sparing” might be a clue for NLSDI to be distinguished from other CIE conditions.http://www.e-ijd.org/article.asp?issn=0019-5154;year=2022;volume=67;issue=5;spage=579;epage=582;aulast=Demirchanarin–dorfman syndromecongenital ichthyosisgenodermatosesneutral lipid storage disease with ichthyosis |
| spellingShingle | Filiz Topaloglu Demir Baran Cayhan Cagla Karaoglan Zafer Turkoglu Nesimi Buyukbabani Mesut Ayer Bugrahan Duz Bulent Uyanık Could “islets of sparing” be a clue for neutral lipid storage disease with ichthyosis in patients with congenital ichthyosiform erythroderma? Indian Journal of Dermatology chanarin–dorfman syndrome congenital ichthyosis genodermatoses neutral lipid storage disease with ichthyosis |
| title | Could “islets of sparing” be a clue for neutral lipid storage disease with ichthyosis in patients with congenital ichthyosiform erythroderma? |
| title_full | Could “islets of sparing” be a clue for neutral lipid storage disease with ichthyosis in patients with congenital ichthyosiform erythroderma? |
| title_fullStr | Could “islets of sparing” be a clue for neutral lipid storage disease with ichthyosis in patients with congenital ichthyosiform erythroderma? |
| title_full_unstemmed | Could “islets of sparing” be a clue for neutral lipid storage disease with ichthyosis in patients with congenital ichthyosiform erythroderma? |
| title_short | Could “islets of sparing” be a clue for neutral lipid storage disease with ichthyosis in patients with congenital ichthyosiform erythroderma? |
| title_sort | could islets of sparing be a clue for neutral lipid storage disease with ichthyosis in patients with congenital ichthyosiform erythroderma |
| topic | chanarin–dorfman syndrome congenital ichthyosis genodermatoses neutral lipid storage disease with ichthyosis |
| url | http://www.e-ijd.org/article.asp?issn=0019-5154;year=2022;volume=67;issue=5;spage=579;epage=582;aulast=Demir |
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