[Refractory Sweet syndrome complicated by monoclonal gammopathy of undetermined significance]. / Syndrome de Sweet récidivant compliqué de gammapathie monoclonale de signification indéterminée.

The association between Sweet syndrome and monoclonal gammopathy of undetermined significance (MGUS) is exceptional. We report the case of a 44 years-old woman in whom recurrent Sweet syndrome was complicated by monoclonal gammopathy of undetermined significance (MGUS) after 3 years of evolution.

L'association entre syndrome de Sweet et gammapathies monoclonales de signification indéterminée (MGUS) est exceptionnelle. Nous rapportons l'observation d'une patiente âgée de 44 ans chez qui un syndrome de Sweet d'évolution récurrente, s'est compliqué par l'apparition d'une gammapathie monoclonale de signification indéterminé après 3 ans d'évolution.

[Vertebral collapse revealing primary hyperparathyroidism in a 14-year-old girl]. / Tassement vertébral révélant une hyperparathyroïdie primaire chez une adolescente.

Primary hyperparathyroidism, widely prevalent in women after menopause, remains rare in children and adolescents. Sporadic forms are the most frequent. Clinical manifestations are general, renal, gastrointestinal, cardiac, or bony. Diagnosis is biological and radiological. The imaging modalities allow assessment of the disease impact and identification of the parathyroid adenoma.

[Secondary TFE3-associated renal cell carcinoma in a child treated for Ewing sarcoma]. / Carcinome à cellules claires du rein et sarcome d'Ewing pelvien : une association rare chez l'enfant en rapport avec la translocation TFE3 Xp11.2.

Renal cell carcinoma is a rare pediatric malignant tumor of the kidney. Unlike Wilms tumor, the efficacy of chemotherapy and radiation therapy in pediatric renal cell carcinoma remains uncertain. Surgery is the best treatment and prognosis is favorable when the tumor is localized and completely eradicated. We report an exceptional observation in a 7-year-old girl with renal cell carcinoma who had been treated 20 months previously for Ewing sarcoma with chemotherapy and radiotherapy. The renal tumor was revealed by abdominal pain without hematuria. She underwent a radical nephrectomy, and histopathology concluded in renal carcinoma associated with translocation Xp 11.2 grade 3 of Furhrman pT3a N1. No adjuvant therapy was given. After 3 years of follow-up, there is no evidence of local or metastatic recurrence. This observation is significant given the very young age of this patient, the occurrence after Ewing sarcoma with a short disease-free interval. It seems that translocation renal cell carcinoma is associated with previous exposure to chemotherapy, particularly topoisomerase II inhibitors or alkylating agents.

[Cryoglobulinemia in a Tunisian internal medicine department]. / Cryoglobulinémie : expérience dans un service de médecine interne tunisien.

AIM: Cryoglobulinemia is characterized by multiple organ involvement, mainly including the skin, liver, kidneys, and peripheral nerves. Our aim was to investigate the demographic, clinical, and serologic features, as well as survival in a group of 16 Tunisian patients with cryoglobulinemia. RESULTS: The study included 12 women and 4 men, and their mean age was 41 years. In all but two, the cryoglobulinemia was associated with another disease. These included lupus for 9, Sjögren syndrome for 2, and polyarteritis nodosa for one. They also included infectious diseases: 3 patients with hepatitis B virus infection, one with hepatitis C virus infection, one with parvovirus B19, and another with lymph node tuberculosis. Only one case of lymphoproliferative disease was noted. General symptoms were present in 81% of the patients, cutaneous vasculitis in 43%, peripheral vascular-Raynaud phenomenon in 37%, joint polyarthralgia or arthritis in 62%, renal involvement in 68%, neuropathy in 25%, lung involvement in 56%, gastrointestinal involvement in 37%, and finally cardiac involvement in 31%. In some cases it was difficult to determine if the clinical signs were attributable to cryoglobulinemia or the underlying pathology. The course was favorable under treatment for 5 patients, while 7 patients became sicker and 5 finally died. CONCLUSION: Cryoglobulinemia is underdiagnosed. Treatment depends on the severity of the lesions and the underlying disease.

[An unusual toothache!]. / Une douleur dentaire inhabituelle!

INTRODUCTION: Dental pain is a frequent reason for consulting. It may have non-odontogenic causes such as lesions of vascular, neurologic, muscular, or bone structures. The diagnosis and management of this acute or chronic pain syndrome may be difficult. We report a case of atypical dental pain leading to the diagnosis of a plasmocytic mandibular tumor revealing a multiple myeloma. CASE REPORT: A 50-year-old female patient consulted for dental pain during the 3 previous months. Bilateral mandibular swelling was noted during the clinical examination. Radiological examinations revealed a tumoral process associated with osteolytic lesions. The pathological examinations of biopsy samples revealed plasmocytic proliferation. A myelogram and immunoglobulin electrophoresis supported a diagnosis of multiple myeloma with kappa light chains. DISCUSSION: Discovering a mandibular tumor with lytic lesions is an indication for an etiological assessment and screening for a blood disease. A mandibular plasmacytoma may be isolated or present as a multiple myeloma, justifying a complete initial assessment. The bone localization of a plasmacytoma is a bad prognostic factor for survival for patients presenting with multiple myeloma.

[Lupus and pregnancy: study of 26 cases, an internal medicine department experience and review of the literature]. / Lupus et grossesse : expérience d'un service de médecine interne à propos de 26 cas et revue de la littérature.

OBJECTIVE: To study the maternal and fetal outcomes in women with systemic lupus erythematosus. PATIENTS AND METHODS: A retrospective study of 26 pregnancies in 15 systemic erythematosus patients diagnosed before or during pregnancy regarding to American College of Rheumatology criteria in a single reference center. RESULTS: The mean patient age was 31.52 years (24-39 years). The mean interval from the diagnosis of the systemic lupus erythematosus to pregnancy was 4.2 years. Eight pregnancies were planned. The flare rate of lupus during pregnancy was 31%, life birth rate was 65% and fetal loss rate was 35%. DISCUSSION AND CONCLUSION: As an increase in disease activity can occur during pregnancy and because of a higher rate of obstetrical complications in patients with lupus, it is important to carefully plan pregnancy. Pregnancy in lupus patients must be closely monitored by a multispeciality care of the patients.

[Wegener's granulomatosis diagnosed by orbital-meningeal presentation: a case report]. / Granulomatose de Wegener révélée par une localisation orbito-méningée : à propos d'un cas.

Wegener's granulomatosis is a necrotizing granulomatous vasculitis with a strong affinity for the upper respiratory tract, lung and kidney. The ophthalmologic manifestation most often presents as inflammatory orbital pseudotumor or scleritis. We report a case of a 27-year-old woman with an orbital-meningeal presentation leading to a diagnosis of Wegener's granulomatosis.

[Spontaneous orbital hematoma: two case reports]. / Hématome orbitaire spontané: à propos de deux cas.

Although rare, spontaneous intra-orbital hematoma can quickly jeopardize vision. It usually presents with painful proptosis. It can result from multiple etiologies, and the diagnosis is based on imaging studies in the absence of known causes. We describe two cases of spontaneous intraorbital hematoma. The first, of unknown etiology, required needle drainage. The second was associated with a subperiosteal hematoma of the orbital roof complicating a periorbital bone infarction in a patient with sickle-thalassemia.