Familial reactive perforating collagenosis in two siblings
Reactive perforating collagenosis (RPC) is a rare form of transepithelial elimination disorder, in which genetically altered collagen is extruded through the epidermis. Of the acquired and inherited form, the latter is extremely rare. Here, we present two cases of inherited form of RPC in siblings a...
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Format: | Article |
Language: | English |
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Wolters Kluwer Medknow Publications
2018-01-01
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Series: | Indian Journal of Paediatric Dermatology |
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Online Access: | http://www.ijpd.in/article.asp?issn=2319-7250;year=2018;volume=19;issue=2;spage=161;epage=163;aulast=Rai |
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author | Abhijna K Rai Mamatha S Kusagur K S Chandan |
author_facet | Abhijna K Rai Mamatha S Kusagur K S Chandan |
author_sort | Abhijna K Rai |
collection | DOAJ |
description | Reactive perforating collagenosis (RPC) is a rare form of transepithelial elimination disorder, in which genetically altered collagen is extruded through the epidermis. Of the acquired and inherited form, the latter is extremely rare. Here, we present two cases of inherited form of RPC in siblings aged 4 and 7 years presented with a history of itchy raised lesions of 9 months and 3 years duration, respectively. Dermatological examination revealed multiple papules with a central keratotic plug distributed mainly over face and extensors of upper and lower extremities. Köebnerization was present. Skin biopsy revealed perforating collagen bundles in the upper dermis and epidermis. |
first_indexed | 2024-12-10T10:26:01Z |
format | Article |
id | doaj.art-ba9d86d714fa4fe19f03e8f7b05cc09a |
institution | Directory Open Access Journal |
issn | 2319-7250 |
language | English |
last_indexed | 2024-12-10T10:26:01Z |
publishDate | 2018-01-01 |
publisher | Wolters Kluwer Medknow Publications |
record_format | Article |
series | Indian Journal of Paediatric Dermatology |
spelling | doaj.art-ba9d86d714fa4fe19f03e8f7b05cc09a2022-12-22T01:52:44ZengWolters Kluwer Medknow PublicationsIndian Journal of Paediatric Dermatology2319-72502018-01-0119216116310.4103/ijpd.IJPD_129_16Familial reactive perforating collagenosis in two siblingsAbhijna K RaiMamatha S KusagurK S ChandanReactive perforating collagenosis (RPC) is a rare form of transepithelial elimination disorder, in which genetically altered collagen is extruded through the epidermis. Of the acquired and inherited form, the latter is extremely rare. Here, we present two cases of inherited form of RPC in siblings aged 4 and 7 years presented with a history of itchy raised lesions of 9 months and 3 years duration, respectively. Dermatological examination revealed multiple papules with a central keratotic plug distributed mainly over face and extensors of upper and lower extremities. Köebnerization was present. Skin biopsy revealed perforating collagen bundles in the upper dermis and epidermis.http://www.ijpd.in/article.asp?issn=2319-7250;year=2018;volume=19;issue=2;spage=161;epage=163;aulast=RaiFamilial reactive perforating collagenosisinheritedtransepithelial elimination |
spellingShingle | Abhijna K Rai Mamatha S Kusagur K S Chandan Familial reactive perforating collagenosis in two siblings Indian Journal of Paediatric Dermatology Familial reactive perforating collagenosis inherited transepithelial elimination |
title | Familial reactive perforating collagenosis in two siblings |
title_full | Familial reactive perforating collagenosis in two siblings |
title_fullStr | Familial reactive perforating collagenosis in two siblings |
title_full_unstemmed | Familial reactive perforating collagenosis in two siblings |
title_short | Familial reactive perforating collagenosis in two siblings |
title_sort | familial reactive perforating collagenosis in two siblings |
topic | Familial reactive perforating collagenosis inherited transepithelial elimination |
url | http://www.ijpd.in/article.asp?issn=2319-7250;year=2018;volume=19;issue=2;spage=161;epage=163;aulast=Rai |
work_keys_str_mv | AT abhijnakrai familialreactiveperforatingcollagenosisintwosiblings AT mamathaskusagur familialreactiveperforatingcollagenosisintwosiblings AT kschandan familialreactiveperforatingcollagenosisintwosiblings |