[Malakoplakia of the submandibular gland in a renal transplant patient]. / Malakoplakie de la glande sous-mandibulaire chez un patient transplanté rénal.

INTRODUCTION: Malakoplakia (MP) is a rare granulomatous disease, usually occurring in immunocompromised patients, linked to Escherichia coli infection. The lesions are usually located in the genitourinary tract, but there is a great variability in the topography and the clinical presentation. CASE REPORT: A 70-year-old diabetic kidney transplant patient under immunosuppressive treatment presented with a voluminous submandibular chronic lesion, involving the skin, associated with a burgeoning lesion of the oral mucosa. Histological examination of biopsies concluded to MP and bacteriological samples were positive for E. coli. Antibiotic treatment allowed for the regression of the lesion before surgical removal. Histological examination of resected material confirmed the diagnosis of invasive MP of the submandibular gland. DISCUSSION: The diagnosis of MP relies on histological examination, showing the presence of von Hansemann's cells and Michaelis- Gutmann bodies. The treatment is based on active antibiotics targeted against intracellular bacteria, possibly associated with surgery. We report the first case of MP involving the submandibular gland.

Diffuse dermal angiomatosis associated with severe atherosclerosis: two cases and review of the literature.

Diffuse dermal angiomatosis (DDA) is a rare condition characterized by endothelial proliferation in the reticular dermis. Several diseases have been associated with DDA, including peripheral arterial disease (PAD). We report two cases of DDA associated with PAD. Patient 1 was a 71-year-old woman, who presented with painful necrotic ulcerations on her trunk and a medical history of PAD. Skin biopsy revealed a dermal proliferation of endothelial cells, and despite medical treatment, she died 1 month later. Patient 2 was an 81-year-old man, who presented with an erythematous, bluish plaque of the shoulder. He was a heavy smoker, with severe PAD. Biopsy showed dermal capillary hyperplasia, with a few fibrin thrombi, and follow-up only was recommended. In both cases, laboratory tests and Doppler ultrasonography ruled out other thrombotic conditions and vascularitis. DDA is a rare complication of PAD, and the optimum medical treatment remains to be clarified, especially when revascularization has failed or is not possible, as in our cases.

Clinical efficacy of intravenous immunoglobulins for the treatment of dermatomyositis skin lesions without muscle disease.

BACKGROUND: Treating dermatomyositis (DM) with isolated skin involvement is difficult and inconsistently performed. Intravenous immunoglobulins (IVIg) are recommended for corticoresistant or corticodependant DM, but only a few cases of IVIg use in DM with isolated skin involvement have been reported. DESIGN: We performed a retrospective monocentric study of 27 patients who were treated with IVIg for severe DM skin lesions (no or minor muscle involvement) after failure of photoprotection and at least one line of treatment. RESULTS: Nineteen patients (70%) exhibited a major response, four patients exhibited a partial response and four patients exhibited no response, including two patients with grade 3 side effects (headaches). The mean number of IVIg courses was 4.8 (range 1-15). Ten patients (53%) relapsed, with a median time of 6.2 months after the last IVIg course. Six of these patients were successfully treated with a new IVIg course. Muscle disease developed in six patients. CONCLUSION: IVIg may be an effective and safe treatment for DM with isolated skin involvement. Relapse occurred frequently, but treatment with a new course of IVIg was successful. Controlled studies are required to confirm these results.

Disseminated cutaneous infection due to Mycobacterium chelonae following hematopoietic stem cell transplantation.

This report describes two cases of disseminated cutaneous Mycobacterium chelonae after hematopoietic stem cell transplantation (HSCT).

IL-10 producing regulatory B cells in mice and humans: state of the art.

IL-10-producing regulatory B cells have been undoubtedly identified in mice and shown to downregulate inflammation, making them potentially important for maintenance of tolerance. Several recent works have also identified IL-10 producing regulatory B cells in humans and have begun to unravel their phenotype and mode of suppression. Cell surface phenotype of human Bregs includes CD38, CD27, CD24 and CD5. Mechanisms of suppression may imply inhibition of CD4+ T proliferation, inhibition of Th1 differentiation, induction of regulatory T cells and suppression of monocytes activation. These recent findings imply that manipulating IL-10 production by human B cells could be a useful therapeutic strategy for modulating immune responses in humans.

[Chloroquine cardiotoxicity in long-term lupus therapy in two patients]. / Cardiotoxicité après un traitement au long cours par chloroquine chez deux patientes lupiques.

BACKGROUND: The antimalarial compounds chloroquine and hydroxychloroquine are widely used in the treatment of connective tissue diseases and are usually well tolerated. We report two cases of chloroquine cardiotoxicity. PATIENTS AND METHODS: Two women (aged 43 and 48 years) were treated for 5 years for lupus. They developed severe conduction disturbances requiring a pacemaker. Plasma chloroquine concentrations were abnormally high in both cases. In one case, a genetic polymorphism modulating the activity of a cytochrome involved in chloroquine metabolism (CYP2C8) was identified. DISCUSSION: Since 1965, 60 cases of occasionally severe cardiotoxicity have been reported following long-term treatment with chloroquine in most cases, but also with hydroxychloroquine. This toxicity must be detected early and close cardiac assessment is required.