Suspect dyskeratotic neoformations in a 7-year-old child with keratitis-ichthyosis-deafness syndrome: diagnostic, surgical and wound care management
Keratitis-ichthyosis-deafness syndrome (KID) is a rare genetic disorder characterized by the triad of hyperkeratosis, ichthyosis, and congenital prelingual sensorineural deafness, with less than 100 cases described in the literature. In addition to many other extra-cutaneous manifestations, these p...
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| Format: | Article |
| Language: | English |
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PAGEPress Publications
2024-04-01
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| Series: | Dermatology Reports |
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| Online Access: | https://www.pagepress.org/journals/dr/article/view/9953 |
| _version_ | 1827281011370622976 |
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| author | Alvise Montanari Francesca Caroppo Antonio Amabile Anna Fortina Belloni Franco Bassetto |
| author_facet | Alvise Montanari Francesca Caroppo Antonio Amabile Anna Fortina Belloni Franco Bassetto |
| author_sort | Alvise Montanari |
| collection | DOAJ |
| description |
Keratitis-ichthyosis-deafness syndrome (KID) is a rare genetic disorder characterized by the triad of hyperkeratosis, ichthyosis, and congenital prelingual sensorineural deafness, with less than 100 cases described in the literature. In addition to many other extra-cutaneous manifestations, these patients are burdened by two principal increased risk factors involving the skin: the risk of developing infections and the risk of developing malignant skin tumors, especially Squamous Cell Carcinoma and Trichilemmal tumors. We present the case of a 7-year-old girl with a unique genetic variant described to date, who developed 4 dyskeratotic neoformation. Since the dermatoscopic examination was not sufficient to exclude malignancy, excisional and punch biopsies were used. Nonetheless, healing was challenging, with wound dehiscence and infection onset. Ad hoc wound care management resorting to negative pressure therapy and advanced medical honey-based dressings was necessary to achieve complete wound healing.
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| first_indexed | 2024-04-24T08:50:53Z |
| format | Article |
| id | doaj.art-4d3ccb2fd76049a1b9c4e4698dd467d0 |
| institution | Directory Open Access Journal |
| issn | 2036-7392 2036-7406 |
| language | English |
| last_indexed | 2024-04-24T08:50:53Z |
| publishDate | 2024-04-01 |
| publisher | PAGEPress Publications |
| record_format | Article |
| series | Dermatology Reports |
| spelling | doaj.art-4d3ccb2fd76049a1b9c4e4698dd467d02024-04-16T10:30:33ZengPAGEPress PublicationsDermatology Reports2036-73922036-74062024-04-0110.4081/dr.2024.9953Suspect dyskeratotic neoformations in a 7-year-old child with keratitis-ichthyosis-deafness syndrome: diagnostic, surgical and wound care managementAlvise Montanari0Francesca Caroppo1Antonio Amabile2Anna Fortina Belloni3Franco Bassetto4Plastic Surgery Unit, Department of Neuroscience, University of Padua; European Network for Rare Skin Disorders (ERN-Skin) PaduaEuropean Network for Rare Skin Disorders (ERN-Skin) Padua; Pediatric Dermatology Regional Center, Department of Women's and Children's Health (SDB), University of Padua; Dermatology Unit, Department of Medicine (DIMED), University of PaduaPlastic Surgery Unit, Department of Neuroscience, University of Padua; European Network for Rare Skin Disorders (ERN-Skin) PaduaEuropean Network for Rare Skin Disorders (ERN-Skin) Padua; Pediatric Dermatology Regional Center, Department of Women's and Children's Health (SDB), University of PaduaPlastic Surgery Unit, Department of Neuroscience, University of Padua; European Network for Rare Skin Disorders (ERN-Skin) Padua Keratitis-ichthyosis-deafness syndrome (KID) is a rare genetic disorder characterized by the triad of hyperkeratosis, ichthyosis, and congenital prelingual sensorineural deafness, with less than 100 cases described in the literature. In addition to many other extra-cutaneous manifestations, these patients are burdened by two principal increased risk factors involving the skin: the risk of developing infections and the risk of developing malignant skin tumors, especially Squamous Cell Carcinoma and Trichilemmal tumors. We present the case of a 7-year-old girl with a unique genetic variant described to date, who developed 4 dyskeratotic neoformation. Since the dermatoscopic examination was not sufficient to exclude malignancy, excisional and punch biopsies were used. Nonetheless, healing was challenging, with wound dehiscence and infection onset. Ad hoc wound care management resorting to negative pressure therapy and advanced medical honey-based dressings was necessary to achieve complete wound healing. https://www.pagepress.org/journals/dr/article/view/9953Kid syndromeskin neoformationsrare skin disorderspediatric wound caremedical honey |
| spellingShingle | Alvise Montanari Francesca Caroppo Antonio Amabile Anna Fortina Belloni Franco Bassetto Suspect dyskeratotic neoformations in a 7-year-old child with keratitis-ichthyosis-deafness syndrome: diagnostic, surgical and wound care management Dermatology Reports Kid syndrome skin neoformations rare skin disorders pediatric wound care medical honey |
| title | Suspect dyskeratotic neoformations in a 7-year-old child with keratitis-ichthyosis-deafness syndrome: diagnostic, surgical and wound care management |
| title_full | Suspect dyskeratotic neoformations in a 7-year-old child with keratitis-ichthyosis-deafness syndrome: diagnostic, surgical and wound care management |
| title_fullStr | Suspect dyskeratotic neoformations in a 7-year-old child with keratitis-ichthyosis-deafness syndrome: diagnostic, surgical and wound care management |
| title_full_unstemmed | Suspect dyskeratotic neoformations in a 7-year-old child with keratitis-ichthyosis-deafness syndrome: diagnostic, surgical and wound care management |
| title_short | Suspect dyskeratotic neoformations in a 7-year-old child with keratitis-ichthyosis-deafness syndrome: diagnostic, surgical and wound care management |
| title_sort | suspect dyskeratotic neoformations in a 7 year old child with keratitis ichthyosis deafness syndrome diagnostic surgical and wound care management |
| topic | Kid syndrome skin neoformations rare skin disorders pediatric wound care medical honey |
| url | https://www.pagepress.org/journals/dr/article/view/9953 |
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