Dermatomyositis as a paraneoplastic syndrome of tonsillar carcinoma

Dermatomyositis (DM) is characterized by weakness and pain of proximal muscles as well as skin manifestations (erythema). As a paraneoplastic syndrome, it is most frequently related to lung-, gynecological and gastrointestinal tumors. This work describes a case of tonsillar carcinoma associated with DM in consideration of the current literature. Initially the patient presented herself with pain of shoulders and hips, exanthema, pruritus and a positive ANA titer. 21 month later, the 56-year-old female was diagnosed with DM and tumor because of progressive symptoms, muscular weakness and cervical lymphadenopathy. A squamous cell carcinoma of the left tonsil (pT2 pN2b cM0 R0) was confirmed by computertomography, panendoscopy and lymph node (LN) biopsy. Because of rapid deterioration of her general condition tumor resection and neck dissection were performed only after conditioning with cortisone pulse therapy. Myasthenia was persistent after surgery, but skin manifestations were regressive, accompanied by sinking inflammatory parameters. Despite of postoperative cortisone pulse the patient developed autoimmune rhabdomyolysis affecting the heart muscle. Therefore the planned adjuvant radiochemotherapy was contraindicated. Immunosuppression was escalated with Rituximab. While rhabdomyolysis markers were decreasing it was possible by training to bring the before bedridden patient into a seating position 3 month after surgery. Seven month after surgery, she recovered to full strength level of all muscles groups.

Conclusion:

The occurence of DM and pathological lymphadenopathy of head and neck is a strong indication for performing panendoscopy and LN biopsy. Dependent on symptoms and laboratory values an intensified DM therapy is necessary concomitant to tumor treatment.

Publication History

Publication Date:
18 April 2018 (online)