Kindler syndrome with intractable squamous cell carcinoma arising in the oral cavity: A case report and review of cases

Kindler syndrome (KS) is an extremely rare subtype of epidermolysis bullosa characterized by blistering of the extremities, progressive polymorphous skin atrophy, and mucosal inflammation. There is no curative therapy for KS, only symptomatic treatment. Patients with KS generally present with squamo...

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Veröffentlicht in:Current problems in cancer. Case reports Jg. 19; S. 100379
Hauptverfasser: Sugauchi, Akinari, Uchihashi, Toshihiro, Isomura, Emiko Tanaka, Seki, Soju, Kimura, Yuka, Kiyohara, Eiji, Fujimoto, Manabu, Tanaka, Susumu, Tanemura, Atsushi
Format: Journal Article
Sprache:Englisch
Veröffentlicht: Elsevier Inc 01.09.2025
Elsevier
Schlagworte:
Hematology, Oncology, and Palliative Medicine
Kindler syndrome
Oral cavity
Palliative care
Squamous cell carcinoma
Oral cavity
OSCC
Squamous cell carcinoma
RT
SCC
IMRT
PD
Kindler syndrome
Palliative care
KS
CR
progressive disease
complete response
oral squamous cell carcinoma
intensity-modulated radiation therapy
radiation therapy
ISSN:2666-6219, 2666-6219
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Zusammenfassung:Kindler syndrome (KS) is an extremely rare subtype of epidermolysis bullosa characterized by blistering of the extremities, progressive polymorphous skin atrophy, and mucosal inflammation. There is no curative therapy for KS, only symptomatic treatment. Patients with KS generally present with squamous cell carcinoma of the extremities and lips, which are more susceptible to irritation, with only a few cases involving the oral cavity. Herein, we report the case of a 53-year-old female patient with KS, who developed oral squamous cell carcinoma. While initial treatment with intensity-modulated radiation therapy was successful, shortly thereafter, she relapsed and was not responsive to multiple rounds of chemotherapy. Ultimately, palliative care at home was suggested. While the patient received radiation therapy and chemotherapy, she complained of difficulty breathing due to the tumor, especially during the last round of chemotherapy. As a result of aggressive weight reduction of tumor necrotic tissue, the patient's respiratory distress improved. Based on our experience in this case, we propose that the removal of oral tumor necrotic tissues, which is not often performed as part of conventional curative treatment approaches, can sometimes be efficacious for the maintenance of the quality of life, especially in a palliative context for OSCC patients with KS.
ISSN:2666-6219
2666-6219
DOI:10.1016/j.cpccr.2025.100379