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 (Author), Mamatha S Kusagur (Author), K S Chandan (Author)
Format: Book
Published: Wolters Kluwer Medknow Publications, 2018-01-01T00:00:00Z.
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100 1 0 |a Abhijna K Rai  |e author 
700 1 0 |a Mamatha S Kusagur  |e author 
700 1 0 |a K S Chandan  |e author 
245 0 0 |a Familial reactive perforating collagenosis in two siblings 
260 |b Wolters Kluwer Medknow Publications,   |c 2018-01-01T00:00:00Z. 
500 |a 2319-7250 
500 |a 10.4103/ijpd.IJPD_129_16 
520 |a 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. 
546 |a EN 
690 |a Familial reactive perforating collagenosis 
690 |a inherited 
690 |a transepithelial elimination 
690 |a Dermatology 
690 |a RL1-803 
690 |a Pediatrics 
690 |a RJ1-570 
655 7 |a article  |2 local 
786 0 |n Indian Journal of Paediatric Dermatology, Vol 19, Iss 2, Pp 161-163 (2018) 
787 0 |n http://www.ijpd.in/article.asp?issn=2319-7250;year=2018;volume=19;issue=2;spage=161;epage=163;aulast=Rai 
787 0 |n https://doaj.org/toc/2319-7250 
856 4 1 |u https://doaj.org/article/ba9d86d714fa4fe19f03e8f7b05cc09a  |z Connect to this object online.