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DOI 10.4081/dr.2024.9953

Медицинская библиотека · Dermatology reports · 2024

Suspect dyskeratotic neoformations in a 7-year-old child with keratitis-ichthyosis-deafness syndrome: diagnostic, surgical and wound care management.

Montanari Alvise, Caroppo Francesca, Amabile Antonio, Belloni Anna Fortina, Bassetto Franco

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Аннотация

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 honeybased dressings was necessary to achieve complete wound healing.

Для цитирования: Montanari Alvise, Caroppo Francesca, Amabile Antonio, Belloni Anna Fortina, Bassetto Franco. Suspect dyskeratotic neoformations in a 7-year-old child with keratitis-ichthyosis-deafness syndrome: diagnostic, surgical and wound care management.. Dermatology reports. 2024. DOI: 10.4081/dr.2024.9953.
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