Sirolimus as combination rescue therapy with tumor necrosis alpha inhibitors for severe, refractory hidradenitis suppurativa

International audienceNo abstract availabl

Systemic inflammatory trunk recurrent acute macular eruption ( SITRAME ): A new auto‐inflammatory syndrome in adult?

International audienceWe report here a series of 16 adult patients, equally male and female (50%), with recurrent nonpruriginous stereotyped macular, and sometimes papular, eruption involving always the trunk, sparing some part of the skin with sharp demarcation associated in 100% of cases with systemic inflammation. The median age at the diagnosis was 53 years-old [range 28–68]. All cases were sporadic and occurred among adults from cosmopolitan origin. Thefirst attacks of the disease occurred on a mean age of 35 years [range 18–52], never during childhood

Clinical Spectrum, Quality of Life, BRAF Mutation Status and Treatment of Skin Involvement in Adult Langerhans Cell Histiocytosis

Langerhans cell histiocytosis is a rare histiocytic disorder for which skin involvement and management are poorly described in adults. The aim of this retrospective monocentric study in a national reference centre is to describe the clinical characteristics, quality of life, BRAF mutation status and outcomes of skin involvement in adult patients with Langerhans cell histiocytosis. Twenty-five patients (14 females, mean age 47 years) were included, with a median follow-up of 33 months (range 4–420 months). Patients experienced poor dermatological quality of life despite low body surface involvement. BRAFV600 mutations were detected in 8 of the 18 patients analysed (45%). Eight patients had an associated malignancy. Several treatment options were used and consisted of surgery, topical steroids and carmustine, thalidomide, methotrexate, vinblastine and steroids and cladribine. This study highlights the need to evaluate quality of life and to screen for associated malignancy in adult patients with Langerhans cell histiocytosis

Dominance of an UBA1 mutant clone over a CALR mutant clone: from essential thrombocytemia to VEXAS.

International audienc

Efficacy and safety of treatments in cutaneous polyarteritis nodosa: A French observational retrospective study

International audienceCutaneous polyarteritis nodosa is a form of medium-sized vessel vasculitis. Despite a disabling and prolonged course, data on treatment efficacy and safety remain scarce.Retrospective study

Clinical aspects and management of cutaneous leishmaniasis in rheumatoid patients treated with TNF-α antagonists

Patients under immunosuppressive therapy with tumor necrosis factor alpha (TNF-α) antagonists are vulnerable to various opportunistic infections including leishmaniasis. We present a case series of 8 travellers developing cutaneous leishmaniasis whilst on TNF-α antagonist treatment and review the literature on aspects of cutaneous leishmaniasis developing in patients treated with TNF-α antagonists. We make interim recommendations regarding the drug therapy used to maintain remission in travellers with rheumatoid disease travelling to leishmania prone areas. Despite having a medical condition requiring continued rheumatological review the interval to diagnosis appears not to be reduced compared to that described in non-rheumatoid patients. Rheumatologists and family doctors should be aware of the need for post-travel surveillance for leishmaniasis in rheumatoid patients on TNF-alpha antagonist treatment

Increased CD8+CD28- circulating T cells and high blood interferon score characterize the systemic inflammation of amyopathic dermatomyositis

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Increased CD8CD28 circulating T cells and high blood interferon score characterize the systemic inflammation of amyopathic dermatomyositis.

Amyopathic dermatomyositis (ADM) is a subtype of DM defined by the presence of cutaneous signs of DM with no evidence of muscle weakness or abnormal muscle enzymes for ≥ 6 months.1 Classical DM (CDM) is characterized by modification of circulating lymphocytes 4, type I interferon (IFN) signature 2, elevation of serum pro-inflammatory cytokines 3. Pathophysiology of ADM is less studied. We analysed circulating T cells by flowcytometry (using specific monoclonal antibodies), peripheral blood mononuclear cells type I IFN signature by PCR and serum cytokine levels by ELISA in a series of 17 ADM and 15 CDM patients. [...

Increased CD8+CD28- circulating T cells and high blood interferon score characterize the systemic inflammation of amyopathic dermatomyositis

International audienceNo abstract availabl