[Muscular sarcoidosis: a case report of muscle and fascia involvement and literature]. / Sarcoïdose musculaire : à propos d'un cas avec atteinte des muscles et des fascias et revue de la littérature.

INTRODUCTION: Muscular involvement in sarcoidosis is noted in 50 to 80% of the patients when systematic muscle biopsies are carried out. Nevertheless, symptomatic forms are rare and three different types can be distinguished: nodular, chronic myopathy and acute myositis. CASE REPORT: We report a 43-year-old man who presented with sarcoidosis involving in muscles, fascia and articulations. The diagnosis of sarcoidosis was supported by magnetic resonance imaging and positron emission tomography imaging and confirmed by a muscle biopsy. Clinical manifestations resolved with corticosteroids and methotrexate. CONCLUSION: We review 23 cases of sarcoidosis myosistis most similar to ours. The commonest presentation is the involvement of the proximal limb muscles in association with elevated serum muscle enzymes.

[Multiple pseudo-cellulitis plaques and Koplick's sign: a particular form of parvovirus B19 primo-infection in adults]. / Placards pseudocellulitiques multiples et signe de Koplick: une forme originale de primo-infection à parvovirus B19 de l'adulte.

INTRODUCTION: clinical presentation which seems to be more specific of this infection. EXEGESIS: A 35-year-old woman, 12 weeks pregnant, presented with a primary infection of parvovirus B19. The clinical presentation was characterized by pseudo-cellulitis plaques of the buttocks and the vulva, buccal enanthema with ulcerations and Koplick spot. CONCLUSION: This is the second observation which describes such cutaneous and mucosal manifestations associated with parvovirus B19 infection. This kind of clinical presentation should be systematically reported to become well known by the physicians as erythema infectiosum of fifth disease or "gloves and socks" syndrome.

Follow-up of complete cytogenetic remission in patients with chronic myeloid leukemia after cessation of interferon alfa.

PURPOSE: A small proportion of patients with chronic myeloid leukemia (CML) achieve a complete cytogenetic response (CCR), defined as the disappearance of Philadelphia (Ph) chromosome-positive metaphases, after treatment with interferon alfa (IFN). In this population of patients, the question of whether treatment should then be withdrawn is not yet resolved. PATIENTS AND METHODS: In the present study, we followed 15 patients who stopped IFN after achieving CCR. In nine patients IFN was stopped in view of adverse reactions (n = 8) or patient's choice (n = 1). For the remaining six patients, the treatment was stopped because no BCR/ABL rearrangement could be detected by reverse transcriptase polymerase chain reaction (RT-PCR) in four successive analyses using peripheral-blood samples. RESULTS: Loss of CCR and survival were not statistically different (P =.48; P =.7) for the 15 patients who stopped IFN compared with 41 other CCR patients who continued IFN therapy in our institution. The median follow-up after discontinuation of IFN treatment was 36 months (range, 6 to 108 months). Seven patients (47%) (females, or CCR > 24 months and RT-PCR negative before IFN cessation; P <.0001) did not relapse. Eight other patients (53%) relapsed (lost CCR) within 3 to 33 months of treatment discontinuation. One of them relapsed in major cytogenetic remission (MCR) and was still in MCR 87 months after stopping therapy without any treatment. CONCLUSION: It is possible to stop IFN treatment at least in some patients with CML who achieve a prolonged period of CCR. This study also illustrates the hypothesis that persistence of low numbers of Ph-positive cells does not necessarily imply hematologic relapse.

[Sarcoidosis and comorbidity: retrospective study of 32 cases]. / Sarcoïdose et comorbidité: étude rétrospective de 32 observations.

PURPOSE: A retrospective study was set up to investigate active pathologic processes associated with sarcoidosis diagnosed in 32 patients. METHODS: Eighteen patients had two identified granulomatous localizations (56%) and 14 patients had three localizations or more (27%). Comorbidity was noticed in nine patients (28% of cases). Sarcoidosis was associated with an infectious disease five times (hepatitis C virus [HCV] infection three times, including one case after recombinant interferon alpha therapy, and HIV and HCV co-infection two times). The association of sarcoidosis with a chronic immunologic inflammatory disease was noticed four times (lupus erythematosus two times, myasthenia and primary biliary cirrhosis). Finally, in two cases sarcoidosis was associated with a neoplasia (non-Hodgkin's lymphoma in a co-infected HIV-HCV patient, ovarian cystadenocarcinoma in another patient). Sarcoidosis preceded or revealed the comorbidity four times.

Arthropathy associated with anti-Jo-1 antibody.

Anti-Jo-1 antibody is associated with an overlap syndrome usually described as the association of idiopathic inflammatory myopathy, pulmonary fibrosis and polyarthritis. We report three observations illustrating different aspects of arthropathy associated with anti-Jo-1 antibody. Two patients presented with a deforming and erosive arthritis affecting the hands, periarticular calcifications and dislocation of the interphalangeal (IP) joint of the thumb. The third patient, who had a short disease course, presented only with a mild non-erosive polyarthritis of both hands, metacarpophalangeal joint narrowing and periarticular calcifications. All the patients had interstitial pulmonary syndrome. Only two of them had myositis. An arthropathy characterized by erosive arthritis of the fingers, with dislocation of the IP joint of the thumb and periarticular calcifications, seems to be specifically associated with anti-Jo-1 antibody.

[Cutaneous pyoderma gangrenosum with hepatosplenic localization and monoclonal gammapathy. A case report]. / Pyoderma gangrenosum cutané avec localisation hépatosplénique et dysglobulinémie monoclonale.

Pyoderma gangrenosum is an ulcerative disease of the skin. The histopathological lesions are nonspecific, characterized by a diffuse neutrophilic infiltrate in the dermis. Pyoderma gangrenosum is associated with inflammatory, digestive or articular disease, or acute or chronic hemotology disorders in 50% of the cases, more rarely with monoclonal gammapathy. A visceral localization of pyoderma gangrenosum is rare, simulating a systemic disease or an underlying neoplasia. We report a case of cutaneous pyoderma gangrenosum with splenic and hepatic localizatios associated with an IgG monoclonal gammapathy. We emphasize the efficacy of immunosuppressor treatment and the importance of long-term monitoring of these patients.

[Familial Waldmann's disease]. / Maladie de Waldmann familiale.

We report the observation of a mother and her daughter who presented edema, hypoprotidemia and lymphopenia due to protein-losing enteropathy. Radiological, endoscopic and histological investigations revealed the diagnosis of primary intestinal lymphangiectasis or Waldmann's disease. Dietary treatment with middle chained triglycerides was effective. Familial cases are rarely described.

[Schnitzler syndrome: a rare cause of systemic urticaria]. / Syndrome de Schnitzler: une cause rare d'urticaire systémique.

INTRODUCTION: The Schnitzler's syndrome first described in 1972, associates urticaria, bone pain, and monoclonal IgM gammapathy. EXEGESIS: A 50-year-old man presented symptoms of urticaria restricted to the trunk and lower members, with episodes of fever accompanied by inflammatory pain in the knees and legs. Slight deterioration of his general condition was also observed. Biological findings showed the existence of an inflammatory syndrome. Electrophoresis and immunoelectrophoresis provided evidence for the existence of underlying IgM gammapathy. Bone X-ray demonstrated the presence of tibial and peroneal metaphysis thickening, with hyperfixation on bone scintigraphy. The patient's condition improved after cortisone and colchicine treatment, allowing decrease in coricosteroid doses. Two years later, except for urticaria, clinical features have disappeared and no hematological disorder has been observed.

[Langerhans-cell histiocytosis and Erdheim-Chester disease: probably not a fortuitous association]. / Histiocytose langerhansienne et maladie d'Erdheim-Chester: une association probablement non fortuite.

BACKGROUND: Erdheim Chester disease (MEC) is a rare non-Langerhans cell histiocytosis characterized by multi-visceral involvement. We report a case of MEC associated with Langerhans cell histiocytosis (HCL). CASE REPORT: A 46-year-old women presented skin and vulvar localization of HCL associated with typical MEC bone involvement. Despite chemotherapy (vinblastine) and prednisone, the disease progressed to involve the central nervous system, leading to fatal outcome. Post-mortem examination showed HCL in skin, MEC in bones and central nervous system, and intermediate histiocytic proliferation in the encephalon. DISCUSSION: Usually, MEC and HCL are considered as distinct entities. MEC is characterized by a xanthogranulomatous proliferation of CD 68+ foamy histiocytes nested in fibrosis, and HCL by a proliferation of PS 100+ and CD1a+ Langerhans cells. However, our observation, as well as previous reports, suggests that MEC is part of the HCL spectrum.