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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Main Authors: Abhijna K Rai, Mamatha S Kusagur, K S Chandan
Format: Article
Language:English
Published: Wolters Kluwer Medknow Publications 2018-01-01
Series:Indian Journal of Paediatric Dermatology
Subjects:
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.
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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