RESUMO
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.
Assuntos
Antineoplásicos/efeitos adversos , Vesícula/complicações , Carcinoma de Células Escamosas/tratamento farmacológico , Epidermólise Bolhosa/complicações , Neoplasias Palatinas/tratamento farmacológico , Doenças Periodontais/complicações , Transtornos de Fotossensibilidade/complicações , Adulto , Antineoplásicos/uso terapêutico , Carcinoma de Células Escamosas/complicações , Carcinoma de Células Escamosas/diagnóstico , Quimioterapia Adjuvante/métodos , Evolução Fatal , Feminino , Humanos , Mucosite/induzido quimicamente , Neoplasias Palatinas/complicações , Neoplasias Palatinas/diagnóstico , Sepse/induzido quimicamente , Sepse/microbiologiaRESUMO
INTRODUCTION: The oral melanoma (OM) account for 1% of all melanomas. The prognosis is poor despite an adequate locoregional control of the disease. OBSERVATION: A 47 year old women consulted for a blackish lesion of the mandibular gingiva next to tooth 37. Intraoral examination showed a dark pigmented lesion on the lingual side of the left mandibular molar region, extending from tooth 37 to the trine retro left molar. The biopsy confirmed the diagnosis of mucosal nodular melanoma. A wide surgical excision without ipsilateral neck dissection was performed. The treatment was completed by 30 Gy external beam radiotherapy, the patient died 18 months after the first consultation. DISCUSSION: Oral melanomas have a poor prognosis probably because they are generally detected late. The most common sites for oral melanomas are the palate and maxillary gingival, malignant melanoma of the mandibular gingiva is extremely rare. About 30% of OM is preceded by areas of oral pigmentation for several months or years. The prognosis is poor with a 5% to 20% five-year survival rate.