Case Reports
Vol. 16 No. 4 (2024)
Suspect dyskeratotic neoformations in a 7-year-old child with keratitis-ichthyosis-deafness syndrome: diagnostic, surgical and wound care management
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All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.
Received: 3 February 2024
Accepted: 14 February 2024
Accepted: 14 February 2024
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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 four dyskeratotic neoformations. 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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Citations
Dey VK, Saxena A, Parikh S. KID Syndrome: A Rare Genodermatosis. Indian Dermatol Online J 2020;11:116-8. DOI: https://doi.org/10.4103/idoj.IDOJ_87_19
Cammarata-Scalisi F, Willoughby CE, Cárdenas Tadich A, et al. Clinical, etiopathogenic, and therapeutic aspects of KID syndrome. Dermatol Ther 2020;33:e13507. DOI: https://doi.org/10.1111/dth.13507
Coggshall K, Farsani T, Ruben B, et al. Keratitis, ichthyosis, and deafness syndrome: a review of infectious and neoplastic complications. J Am Acad Dermatol 2013;69:127-34. DOI: https://doi.org/10.1016/j.jaad.2012.12.965
Haruna K, Suga Y, Oizumi A, et al. Severe form of keratitis-ichthyosis-deafness (KID) syndrome associated with septic complications. J Dermatol 2010;37:680-2. DOI: https://doi.org/10.1111/j.1346-8138.2010.00839.x
Harms M, Gilardi S, Levy PM, Saurat JH. KID syndrome (keratitis, ichthyosis, and deafness) and chronic mucocutaneous candidiasis: case report and review of the literature. Pediatr Dermatol 1984;2:1-7. DOI: https://doi.org/10.1111/j.1525-1470.1984.tb00433.x
Hazen PG, Walker AE, Stewart JJ, et al. Keratitis, ichthyosis, and deafness (KID) syndrome: management with chronic oral ketoconazole therapy. Int J Dermatol 1992;31:58-9. DOI: https://doi.org/10.1111/j.1365-4362.1992.tb03524.x
Satyaprakash AK, Sheehan DJ, Sangüeza OP. Proliferating trichilemmal tumors: a review of the literature. Dermatol Surg 2007;33:1102-8. DOI: https://doi.org/10.1111/j.1524-4725.2007.33225.x
Ackerman AB, Reddy VB, Soyer HP. Neoplasms with follicular differentiation. 2nd ed. New York: Ardor Scribendi; 2001.
López-Ríos F, Rodríguez-Peralto JL, Aguilar A, et al. Proliferating trichilemmal cyst with focal invasion: report of a case and a review of the literature. Am J Dermatopathol 2000;22:183-7. DOI: https://doi.org/10.1097/00000372-200004000-00018
Nyquist GG, Mumm C, Grau R, et al. Malignant proliferating pilar tumors arising in KID syndrome: a report of two patients. Am J Med Genet A. 2007;143A:734-41. DOI: https://doi.org/10.1002/ajmg.a.31635
Werchau S, Toberer F, Enk A, Helmbold P. Keratitis-ichthyosis-deafness syndrome: response to alitretinoin and review of literature. Arch Dermatol 2011;147:993-5. DOI: https://doi.org/10.1001/archdermatol.2011.216
Lancaster L Jr, Fournet LF. Carcinoma of the tongue in a child: report of case. J Oral Maxillofac Surg 1969;27:269-70.
Rudkin GH, Carlsen BT, Chung CY, et al. Retinoids inhibit squamous cell carcinoma growth and intercellular communication. J Surg Res 2002;10:183-9. DOI: https://doi.org/10.1006/jsre.2001.6346
Caroppo F, Szekely S, Fortina AB. Novel Variant c.148G>T of GJB2 Gene in a 5-Year-Old Child with KID Syndrome. Indian Dermatol Online J 2020;11:1014-15. DOI: https://doi.org/10.4103/idoj.IDOJ_603_20
Santosa KB, Keane AM, Keller M, et al. Inpatient Versus Outpatient Management of Negative Pressure Wound Therapy in Pediatric Patients. J Surg Res 2020;254:197-205. DOI: https://doi.org/10.1016/j.jss.2020.04.025
McNamara SA, Hirt PA, Weigelt MA, et al. Traditional and advanced therapeutic modalities for wounds in the paediatric population: an evidence-based review. J Wound Care 2020;29:321-34. DOI: https://doi.org/10.12968/jowc.2020.29.6.321
Fowler JR, Perkins TA, Buttaro BA, Truant AL. Bacteria adhere less to barbed monofilament than braided sutures in a contaminated wound model. Clin Orthop Relat Res 2013;471:665-71. DOI: https://doi.org/10.1007/s11999-012-2593-z
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Montanari A, Caroppo F, Amabile A, Fortina Belloni A, Bassetto F. Suspect dyskeratotic neoformations in a 7-year-old child with keratitis-ichthyosis-deafness syndrome: diagnostic, surgical and wound care management. Dermatol Reports [Internet]. 2024 Apr. 16 [cited 2026 May 24];16(4). Available from: https://journals.pagepress.net/dr/article/view/9953
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