Relapsing Polychondritis Associated with Myelodysplastic Syndrome

A 62-year-old man had arthritis and an infiltrated purpuric eruption of the legs. He presented then a cyanosis and aches of the left ear’s pinna with a voice disturbance and an inflammatory aspect of the nose. He had an anemia and a thrombopenia. The infectious and auto-immune research was negative. The laryngo-tracheal scan revealed calcifications of the larynx, the trachea and the initial portion of bronchi. Moreover, the biopsy from ear’s pinna cartilage confirmed the diagnosis of chondritis and peri-chondritis. He was thus considered to have the association with relapsing polychondritis and a myelodysplastic syndrome. The patient was treated with corticosteroids and iterative transfusions. The articular and skinny patient’s symptoms completely resolved. The anemia and the thrombopenia were partially improved. The relapsing polychondritis can be considered as paraneoplastic complication in the context of a myelodysplastic syndrome or as a fortuitous association. The mechanism of such an association remains unknown.

Autoimmune Hepatitis-Primary Biliary Cirrhosis: Overlap Syndrome Concomitant with Unexpected Myasthenia and Thymoma

The myasthenia gravis is an autoimmune disease which can be associated frequently with a thymoma and also with autoimmune pathologies. We report the case of a 64-year-old man affected an overlap syndrome corresponding to the association of autoimmune hepatitis (AIH) and primary biliary cirrhosis (PBC). He also presented concomitant myasthenia gravis and thymoma. The physical examination notes only a disturbed voice. The liver tests showed: alkaline phosphatase 600 U/l, alanine aminotransferase 1280 U/l, and aspartate aminotransferase 985 U/l. Viral serologies of hepatitis were all negative, as well as antinuclear, antimitochondrial and antismooth muscle antibodies. The diagnosis of an overlap syndrome was posed according to the biological and hepatic histhological results. An associated myasthenia was confirmed by the results of the EMG and the positivity of the anti-acetylcholine receptor antibody. A chest CT revealed a thymoma. The treatment consisted of ursodeoxycholic acid, prednisone, azathioprine and pyridostigmine. And the patient improved his voice and the liver function. Thymectomy was practiced without incidents.

Brown Tumor of the Maxilla Revealing Primary Hyperparathyroidism

Brown tumor or localized form of osteitis fibrosa cystic is a focal lesion complicating hyperpara-thyroidism. It’s rarely revelatory of the clinical feature and corresponds to a mass with partly cystic and partly solid areas. A 65-year-old man, followed up for hypertension, complained of a progressive swelling in the left paranasal part of the face lasting for 3 months. Physical examination revealed a left paranasal swelling of 4 cm diameter, with a healthy looking skin. CT facial bones found a round formation of 2 cm involving the left maxilla. He received tumor resection by vestibular pathway. Histological examination showed many giant cells. We then discussed the brown tumor diagnosis. The PTH dosage was high: 645 pg/mL (normal: 15 - 68 pg/mL). The serum calcium was in the superior normal limit (100 mg/dL). Cervical ultrasound revealed a bottom left parathyroid nodule. A lower left parathyroidectomy was performed. Histological results confirmed parathyroid adenoma. Monitoring was marked by a marked decrease of the PTH serum level then a return to normal values, a normocalcemia and no recurrence of the maxillary tumor with Recoil of 14 months. Brown tumor of the maxilla is rare and should be considered even in absence of hypercalcemia.