Keywords
clinical course
dermatology
children
How to Cite
Abstract
Introduction:Kindler Syndrome (KS) is a rare autosomal recessive genodermatosis, considered an uncommon subtype of epidermolysis bullosa, characterized by skin fragility, photosensitivity, trauma-induced blisters, and progressive poikiloderma. Objective: To identify the dermatological characteristics of KS in minors. Methodology: A qualitative methodology based on the PRISMA model was employed, with a comprehensive search conducted in databases such as PubMed and Scopus, considering articles between 2020 and 2024. Results: Fourteen cases in minors were analyzed, with symptoms ranging from birth to adolescence, showcasing dermatological manifestations such as skin fragility, poikiloderma, keratotic papules, pseudosyndactyly, as well as recurrent oral problems like angular cheilitis and erosive stomatitis. Manifestations from other systems included chronic diarrhea, constipation, urethral stenosis, vesico-ureteral complications, ocular defects like corneal erosions and amblyopia, and dental complications such as gingival hyperplasia and dental caries. Hematologically, anemia was observed in some cases. Conclusion: KS presents a complex set of dermatological and systemic symptoms from childhood. Early identification through a multidisciplinary approach can help manage complications and improve long-term prognosis.
References
Sari A, Celik S. Periodontal Manifestation in a Patient with Kindler Syndrome. Case Rep Dent. 8 de marzo de 2021;2021:6671229. Disponible en: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7963901/
Stefanescu BI, Radaschin DS, Mitrea G, Anghel L, Beznea A, Constantin GB, et al. Epidermolysis Bullosa—A Kindler Syndrome Case Report and Short Literature Review. Clin Pract. 30 de julio de 2023;13(4):873-80. Disponible en: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10453107/
Torres-Iberico R, Condori-Fernández Y, Apagüeño-Ruiz C, Andia-Ticona M, Pomar-Morante R. Síndrome de Kindler, manejo multidisciplinario. Actas Dermo-Sifiliográficas. 1 de noviembre de 2020;111(9):775-80. Disponible en: https://www.sciencedirect.com/science/article/pii/S0001731020302799
Zhang Q, Yang Q, Shen F, Wang L, Luo J. Identification of a novel FERMT1 variant causing kindler syndrome and a review of the clinical and molecular genetic features in Chinese patients. Front Pediatr. 6 de septiembre de 2024;12:1425030. Disponible en: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11415864/
Ahmed A, Zehra T, Moin A, Rehman Usmani SU. Battling a rarity: A case of kindler syndrome from a developing country. SAGE Open Med Case Rep. 16 de febrero de 2024;12:2050313X241231518. Disponible en: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10874135/
Chopkar AD, Malpani SS, Supekar BB, Mukhi JI. Kindler syndrome: A rare case report. 2021;
Shan Y, Zuo YG. [Clinical Characteristics and Gene Mutations in 186 Cases of Kindler Syndrome]. Zhongguo Yi Xue Ke Xue Yuan Xue Bao. abril de 2022;44(2):227-35.
Bhandary R, Venugopalan G, Hegde P. Unusual oral manifestation of Kindler syndrome: a case report and review of literature. Front Oral Health. 5 de septiembre de 2024;5:1430698. Disponible en: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11410757/
Ghorai R, Singh G, Mittal A, Panwar VK, Talwar H. Urological Manifestations of Kindler Syndrome: A Case Report. Cureus. 2022;14(5):e24758. Disponible en: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9167044/
Youssefian L, Vahidnezhad H, Uitto J. Kindler Syndrome. En: Adam MP, Feldman J, Mirzaa GM, Pagon RA, Wallace SE, Amemiya A, editores. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 2022. Disponible en: http://www.ncbi.nlm.nih.gov/books/NBK349072/
Maharana PK, Sahay P, Mandal S, Nagpal R, Sharma N. Ocular manifestations in Kindler syndrome. Indian J Ophthalmol. julio de 2022;70(7):2585-7. Disponible en: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9426131/
Srikumar A, Lava C, Udumudi A. Genetic Diagnosis of Kindler Syndrome, a Rare form of Genodermatosis in a Newborn with Identification of a Nonsense Mutation, c. 328C > T, in the FERMT1 Gene in the Indian Population. Indian J Dermatol. 2023;68(3):334-6. Disponible en: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10389160/
Idkaidak S, Albandak M, Alqarajeh F, Dukmak ON, Imhaimeed J, C. N. Khalil N. Kindler Syndrome Presenting as Colitis in an Infant. Cureus.;15(8):e43928. Disponible en: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10514736/
Neema S, Sinha P, Sandhu S, Mukherjee S, Radhakrishnan S. Dermoscopy of Kindler Syndrome. Dermatol Pract Concept. 3 de abril de 2020;10(2):e2020034. Disponible en: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7190576/
Edrees S, Jarkas N, Hraib M, Al-Yousef K, Baddour R. Kindler syndrome: a rare case report from Syria. Ann Med Surg (Lond). 6 de abril de 2023;85(5):2077-80. Disponible en: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10205305/
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