OBJECTIVE: Discussion of a rare case of dermatomyositis associated with tonsillar neoplasm in an African American woman.
BACKGROUND: Dermatomyositis is a syndrome of inflamatory myopathy with multiorgan manifestations which has been linked to immune dysregulation and neoplasia.
INTRODUCTION: Many studies have shown five to seven fold increased risk of developing malignancy with dermatomyositis within two years of presentation. Most common cancers reported are adenocarcinomas of lung, breast, ovaries, stomach, pancreas and bladder. Dermatomyositis as a paraneoplastic manifestation of tonsillar squamous cell carcinoma has not previously been described.
DESIGN: This is a case report of a 52 year old woman who presented for the evaluation of weakness, facial rash and burning pains. Diagnosis of dermatomyositis was made clinically and corroborated by EMG and muscle biopsy. She was started on prednisone but did not improve. CT chest, abdomen and pelvis along with panendoscopy was done to evaluate for malignancy. She developed swallowing problems, laryngopharyngeal reflux disease and esophageal dysmotility syndrome within 1 year of diagnosis. Approximately 1.5 years after dermatomyositis diagnosis, she developed a right sided neck mass. Biopsy of the mass found metastatic squamous cell carcinoma. Further work up revealed an ulcerating cavity under tonsillolith containing abnormal tissue and this was thought to be the primary malignancy.
RESULTS: Patient underwent right radical neck dissection and tonsillectomy followed by chemotherapy and radiation and her muscle strength, facial rash, burning pains and swallowing difficulties improved.
CONCLUSION: To our knowledge, this is the first case of dermatomyositis in the setting of tonsillar squamous cell carcinoma. High suspicion of nasopharyngeal carcinoma should be maintained in dermatomyositis patients with otherwise negative routine malignancy screening who exhibit any pharyngeal or esophageal complaints
