Paraneoplastic dermatomyositis with pediatric Hodgkin lymphoma: A case report

Abstract

Dermatomyositis (DM) and polymyositis (PM) are autoimmune myopathies and their association with malignancies is well known in adults. However, DM/PM is an uncommon complication of Hodgkin lymphoma (HL), especially in children. An 11-year-old girl presented with deteriorating symmetrical muscle weakness, proximal limb myalgia, and persistent fever, followed by axillary and cervical lymphadenopathy for 2 months. She had no family history of autoimmune disease nor malignancy. Axillary lymph node biopsy revealed mixed cellularity classic HL (Fig. S1); her creatine kinase (CK) level was normal at the time of biopsy. On admission, she could not move due to severe pain with generalized non-pitting edema. She had developed a heliotrope rash but not Gottron papules. She did not complain of dysphasia nor dyspnea. Hematological findings showed elevated levels of CK (5250 IU/L), aldolase (31.9 U/L), lactate dehydrogenase (762 IU/L), C-reactive protein (11.4 mg/dL), and soluble interleukin-2 receptor (2407 U/mL), with leukocytosis (25 9 10 9 /L) and microcytic anemia (hemoglobin 8.9 g/dL). Epstein-Barr virus titer showed the presence of a nuclear antigen (1 : 10) and viral capsid antigen IgG (1 : 40), while the viral DNA load was undetectable. 18 F-fluorodeoxyglucose positron emission tomography (FDG-PET) (Fig.1a) and magnetic resonance imaging (Fig. 1b) showed abnormal diffuse accumulation in the muscles of all extremities, along with a finding of supradiaphragmatic lymphadenopathy, including infiltration of the mediastinal lymph nodes externally (Fig.1c). There was no comorbid interstitial lung disease and no evidence of metastasis to the bone marrow; muscle biopsy was difficult to perform. Clinical findings in addition to myopathic changes on electromyography confirmed a clinical diagnosis of DM, according to Bohan and Peter criteria. Serology was negative for anti-transcriptional intermediary factor 1 c (anti-TIF1c), anti-melanoma differentiation-associated gene 5 (anti-MDA5), anti-histidyl transfer RNA [t-RNA] synthetase (anti-JO-1),

Clinical Features of Cutaneous Paraneoplastic Syndromes in Hodgkin Lymphoma

“… 10 Paraneoplastic DM due to underlying HL is rare. 11 One patient in our study demonstrated clinical signs of DM, including proximal myopathy, elevated creatine kinase, positive antinuclear antibody, macular erythema on dorsal fingers (Gottron’s sign), hand xerosis, and nailfold abnormalities including periungual telangiectasias. These findings were present at the time of HL diagnosis and resolved with treatment of the underlying HL, suggesting a paraneoplastic etiology.…”