Rare presentation of Kyrle′s disease in siblings
<b>Background:</b> Kyrle′s disease is a rare variant of primary perforating dermatosis. Its occurrence in a familial setting, especially in children, is extremely uncommon. Similar appearing skin lesions have been described in adults, secondary to metabolic disorders, infecti...
| Main Authors: | , , , , |
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| Format: | Article |
| Language: | English |
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Wolters Kluwer Medknow Publications
2008-01-01
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| Series: | Indian Journal of Dermatology |
| Subjects: | |
| Online Access: | http://www.e-ijd.org/article.asp?issn=0019-5154;year=2008;volume=53;issue=2;spage=85;epage=87;aulast=Viswanathan |
| _version_ | 1818557313179451392 |
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| author | Viswanathan Seethalakshmi Narurkar Swati Rajpal Aruna Nagpur N Avasare S |
| author_facet | Viswanathan Seethalakshmi Narurkar Swati Rajpal Aruna Nagpur N Avasare S |
| author_sort | Viswanathan Seethalakshmi |
| collection | DOAJ |
| description | <b>Background:</b> Kyrle′s disease is a rare variant of primary perforating dermatosis. Its occurrence in a familial setting, especially in children, is extremely uncommon. Similar appearing skin lesions have been described in adults, secondary to metabolic disorders, infective agents as well as exposure to chemicals. We present a rare case of this genodermatosis in two siblings. <b> Materials and Methods:</b> Two siblings of a non-consanguineous marriage came with generalized discrete papular lesions with a central keratotic plug. All biochemical and serological investigations were within normal limits. Serial sections of the biopsy revealed typical epidermal invaginations filled with parakeratotic debris and perforation into the dermis with accompanying granulomatous reaction. <b> Results and Conclusions:</b> A careful history, detailed routine investigations and serial sections of the skin biopsy are required to demonstrate the typical morphology and stages of evolution of Kyrle′s disease. This helps to differentiate the rare primary Kyrle′s disease from other primary and secondary keratotic lesions. Due to the familial occurrence, screening of relatives of an index case is recommended. |
| first_indexed | 2024-12-13T23:57:52Z |
| format | Article |
| id | doaj.art-abe53eb15d6b4e3aba8896286cb4b3cd |
| institution | Directory Open Access Journal |
| issn | 0019-5154 1998-3611 |
| language | English |
| last_indexed | 2024-12-13T23:57:52Z |
| publishDate | 2008-01-01 |
| publisher | Wolters Kluwer Medknow Publications |
| record_format | Article |
| series | Indian Journal of Dermatology |
| spelling | doaj.art-abe53eb15d6b4e3aba8896286cb4b3cd2022-12-21T23:26:28ZengWolters Kluwer Medknow PublicationsIndian Journal of Dermatology0019-51541998-36112008-01-015328587Rare presentation of Kyrle′s disease in siblingsViswanathan SeethalakshmiNarurkar SwatiRajpal ArunaNagpur NAvasare S<b>Background:</b> Kyrle′s disease is a rare variant of primary perforating dermatosis. Its occurrence in a familial setting, especially in children, is extremely uncommon. Similar appearing skin lesions have been described in adults, secondary to metabolic disorders, infective agents as well as exposure to chemicals. We present a rare case of this genodermatosis in two siblings. <b> Materials and Methods:</b> Two siblings of a non-consanguineous marriage came with generalized discrete papular lesions with a central keratotic plug. All biochemical and serological investigations were within normal limits. Serial sections of the biopsy revealed typical epidermal invaginations filled with parakeratotic debris and perforation into the dermis with accompanying granulomatous reaction. <b> Results and Conclusions:</b> A careful history, detailed routine investigations and serial sections of the skin biopsy are required to demonstrate the typical morphology and stages of evolution of Kyrle′s disease. This helps to differentiate the rare primary Kyrle′s disease from other primary and secondary keratotic lesions. Due to the familial occurrence, screening of relatives of an index case is recommended.http://www.e-ijd.org/article.asp?issn=0019-5154;year=2008;volume=53;issue=2;spage=85;epage=87;aulast=ViswanathanFamilialKyrle′smetabolicperforating dermatosesprimary |
| spellingShingle | Viswanathan Seethalakshmi Narurkar Swati Rajpal Aruna Nagpur N Avasare S Rare presentation of Kyrle′s disease in siblings Indian Journal of Dermatology Familial Kyrle′s metabolic perforating dermatoses primary |
| title | Rare presentation of Kyrle′s disease in siblings |
| title_full | Rare presentation of Kyrle′s disease in siblings |
| title_fullStr | Rare presentation of Kyrle′s disease in siblings |
| title_full_unstemmed | Rare presentation of Kyrle′s disease in siblings |
| title_short | Rare presentation of Kyrle′s disease in siblings |
| title_sort | rare presentation of kyrle x2032 s disease in siblings |
| topic | Familial Kyrle′s metabolic perforating dermatoses primary |
| url | http://www.e-ijd.org/article.asp?issn=0019-5154;year=2008;volume=53;issue=2;spage=85;epage=87;aulast=Viswanathan |
| work_keys_str_mv | AT viswanathanseethalakshmi rarepresentationofkyrlex2032sdiseaseinsiblings AT narurkarswati rarepresentationofkyrlex2032sdiseaseinsiblings AT rajpalaruna rarepresentationofkyrlex2032sdiseaseinsiblings AT nagpurn rarepresentationofkyrlex2032sdiseaseinsiblings AT avasares rarepresentationofkyrlex2032sdiseaseinsiblings |