Paraneoplastic dermatomyositis accompanying nasopharyngeal carcinoma: Diagnosis, treatment and prognosis

Abstract

Dermatomyositis (DM) is a multisystem inflammatory disorder primarily affecting the skin and muscles. Its pathophysiology is still very poorly understood, but humoral and cellular immune dysregulation is apparent. Diagnosis of DM is based on five criteria: proximal limb muscle weakness, serum muscle enzyme elevation, histopathologic muscle abnormalities on muscle biopsy, electromyographic abnormalities, and clinical inflammatory dermatological manifestations (heliotrope rash, poikiloderma, and inflammatory lesions on the hands and facing joints). DM is frequently associated with certain cancers, and may appear before, concurrent with, or after diagnosis of cancer. DM has been reported to be associated with approximately one per 1000 cases of nasopharyngeal carcinoma. Treatment is based on long-course nonselective immunosuppression, particularly corticosteroids, by general route, even when malignancy is present, but new-targeted therapies may modify the treatment strategy in the near future. Despite iatrogenic immunosuppression, the prognosis of nasopharyngeal cancer is not worse in patients with paraneoplastic DM. We report one case as an illustration of this paraneoplastic course (evolving in parallel with the cancer), and to make an update on the state of knowledge on paraneoplastic DM in such cancers.

Dermatomyositis and nasopharyngeal carcinoma in an Indian patient

“…[1] Although the association of dermatomyositis and nasopharyngeal carcinoma is well documented, [1][2][3] it is rare; the coexistence of dermatomyositis is seen in less than 1/1000 cases of nasopharyngeal carcinoma. [4] The association of dermatomyositis with nasopharyngeal carcinoma has been reported from Southern China, South-East Asia, North Africa, and the Alaskan Inuits, but to our knowledge this is the first report from India.…”

Paired study of 172 cases of nasopharyngeal carcinoma with or without dermatomyositis

“…DM is regarded as a paraneoplastic phenomenon with NPC [3], which is not much in the context of head and neck cancer. To date, there have been few studies on the association of NPC with DM [3], and the clinical differences between NPC with and without DM remain unknown. To investigate the clinical characteristics and prognosis of NPC with DM, we conducted a paired study to evaluate the long-term survival and prognostic factors.…”

“…The etiopathogenesis of DM is complex and remains poorly understood. EBV and immune factors such as HLA-DR3 and tumor necrosis factor may be involved in the pathophysiology of DM, but the exact mechanism remains unclear [3].…”

“…However, NPC is the most commonly occurring cancer with DM in Southern China, representing 51.3% (59/115) of the associated malignancies [5]. DM is regarded as a paraneoplastic phenomenon with NPC [3], which is not much in the context of head and neck cancer. To date, there have been few studies on the association of NPC with DM [3], and the clinical differences between NPC with and without DM remain unknown.…”

Dermatomyositis paraneoplastic syndrome before symptomatic tonsillar squamous cell carcinoma: A case report

“…Dermatomyositis is a myopathy characterized by muscle weakness and skin manifestations (including heliotrope rash and Gottron papules) . The pathogenesis of dermatomyositis is not entirely understood, but has been linked to immune dysregulation . Dermatomyositis has been linked to neoplastic processes (with a frequency of 2.5% to 29%); however, it has not been frequently reported to be associated with head and neck cancers .…”