Triplett James, Hee Geoffrey, McLean-Tooke Andrew, Lucas Michaela
Department of Immunology, Sir Charles Gairdner Hospital, Perth, Western Australia, Australia.
Department of Otolaryngology, Sir Charles Gairdner Hospital, Perth, Western Australia, Australia.
BMJ Case Rep. 2018 Jun 20;2018:bcr-2017-221333. doi: 10.1136/bcr-2017-221333.
Plasma cell mucositis (PCM) is a rare non-neoplastic plasma cell proliferative disorder of the mucous membranes, which typically presents as soft tissue lesions involving oral, upper airway or genital mucosa. Laryngeal involvement resulting in stridor has been reported in four other cases previously, with three requiring tracheostomy. We present a case of supraglottic stenosis in a 53-year-old woman presenting with dysphonia and stridor, requiring surgical resection on three occasions accompanied by tracheostomy on two occasions; biopsy was consistent with PCM. Due to relapsing disease activity, high-dose prednisolone and mycophenolate mofetil were commenced with prednisolone eventually being ceased. After 2 years of mycophenolate mofetil therapy, the patient's disease has been controlled without need for further surgical intervention. This is the first reported case of prolonged symptomatic improvement with the use of systemic immunosuppressive therapy with mycophenolate mofetil in PCM.
浆细胞性黏膜炎(PCM)是一种罕见的非肿瘤性浆细胞增殖性黏膜疾病,通常表现为累及口腔、上呼吸道或生殖器黏膜的软组织病变。此前另有4例报告了喉受累导致喘鸣,其中3例需要气管切开术。我们报告一例53岁女性声门上狭窄病例,该患者出现声音嘶哑和喘鸣,需3次手术切除,2次气管切开术;活检结果符合PCM。由于疾病活动复发,开始使用大剂量泼尼松龙和霉酚酸酯,最终停用泼尼松龙。经过2年的霉酚酸酯治疗,患者的疾病得到控制,无需进一步手术干预。这是首例报告使用霉酚酸酯进行全身免疫抑制治疗使PCM症状长期改善的病例。