Kindler syndrome complicated by invasive squamous cell carcinoma of the palate.

Autor: Souldi H; Service d'otorhinolaryngologie et de chirurgie cervico-faciale, hôpital 20 aout 1953, CHU Ibn Rochd, Casablanca, Morocco. Electronic address: hajarsouldi@gmail.com., Bajja MY; Service d'otorhinolaryngologie et de chirurgie cervico-faciale, hôpital 20 aout 1953, CHU Ibn Rochd, Casablanca, Morocco., Mahtar M; Service d'otorhinolaryngologie et de chirurgie cervico-faciale, hôpital 20 aout 1953, CHU Ibn Rochd, Casablanca, Morocco.
Jazyk: angličtina
Zdroj: European annals of otorhinolaryngology, head and neck diseases [Eur Ann Otorhinolaryngol Head Neck Dis] 2018 Feb; Vol. 135 (1), pp. 59-61. Date of Electronic Publication: 2017 Jun 20.
DOI: 10.1016/j.anorl.2017.05.003
Abstrakt: Introduction: Kindler syndrome is a very rare, autosomal recessive genodermatosis characterized by skin fragility and photosensitivity in infancy with progressive poikiloderma.
Case Report: We report the case of a young woman with a history of Kindler syndrome predominantly characterized by extensive involvement of the oropharyngeal mucosa. The patient presented with an ulcerative lesion of the palate. Computed tomography and biopsy concluded on unresectable invasive squamous cell carcinoma of the hard palate. Neoadjuvant chemotherapy was proposed, but the patient died after the first course of chemotherapy in a context of severe gastrointestinal mucositis and generalized sepsis.
Discussion: Mucosal manifestations of Kindler syndrome have been described in the literature, but very few cases of malignant transformation to squamous cell carcinoma have been reported, although it is a very well known, long-term complication of this disease. To our knowledge, this is the second reported case of Kindler syndrome complicated by invasive squamous cell carcinoma of the hard palate.
(Copyright © 2017 Elsevier Masson SAS. All rights reserved.)
Databáze: MEDLINE
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