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Introduction
The macroglossia is observed in certain congenital muscle disease such us Becker’s and Duchenne’s dystrophies or Pompe’s disease. It rarely occurs in polymyositis. We report the case of a symptomatic macroglossia with myositis of the tongue associated with dermatomyositis.
Case Report
A 65 years old man was admitted in our service for macroglossia and macrochely (Fig. 1 [See PDF], 2) associated with a swallowing disorders with false routes and frequent tongue-biting during mastication lasting for a year. He had also an incomprehensible speech and complained of muscle weakness involving the shoulder and pelvic girdles and the limbs. The patient was in a good general condition. Blood pressure was 120/70 mmHg and cardiovascular examination was normal. On physical examination, a slight decrease in muscle strength in all 4 limbs and girdles was noted. He had a significant macroglossia with a falling of the lower lip and a nasal speeech. No skin abnormalities were observed and the neurological examination was strictly normal. The cell blood count was normal and there was no biological inflammatory syndrome. The creatinine phosphokinase (CPK) level was elevated at 606 IU/l and lactate dehydrogenase (LDH) at 416 IU/l. The hepatic and renal function was normal. Electromyography confirmed a diffuse typical myositic process. Muscle biopsy revealed necrotic muscle fibers, regenerating fibers, an endo- and perimysial inflammatory infiltrate and a perifascicular atrophy. The different etiologies of macroglossia have been eliminated by appropriate investigations. Thyroid balance was normal and biopsy of the tongue with congo red staining showed no amyloid deposits. Enzymatic assays were not made in the absence of clinical signs towards genetic myopathies. The biopsy of the tongue showed a phenotype T interstitial inflammatory infiltrate and perifascicular atrophy without neoplastic cells (Fig. 3 [See PDF]). The diagnosis of dermatomyositis sine dermatisis revealed by tongue myositis was certain according to the Bohan and Peter criteria. The search for possible neoplasia was negative (thoraco-abdominal scan and tumor markers). The diagnosis of tongue carcinoma has been ruled out by the tongue biopsy. The patient was treated by prednisone at a dose of 1 mg/kg/day associated with methotrexate (20 mg/week) for five years with no improvement of the symptoms. Imunoglobulin infusions...