Anti-p200 pemphigoid responding to dapsone

Anti-p200 pemphigoid is a rare autoimmune subepidermal blistering disease. Clinical presentation is similar to standard bullous pemphigoid (BP) but mucous membranes and cephalic lesions are more frequent. Histology and direct immunofluorescence (IF) are identical to BP but indirect IF discloses linear deposits of immunoglobulin G (IgG) on the dermal side of artificial salt-split skin. Specific diagnosis is based on western immunoblotting that shows circulating IgG recognizing a 200-kDa protein localized on the dermal extract. The 200-kDa antigen was recently identified as laminin γ1. Anti-p200 pemphigoid should be considered before all atypical or topical steroid-resistant bullous disease, as well as mucous membranes pemphigoid or epidermolysis bullosa acquisita. Dapsone appears to be the most effective treatment and should be used as the first option in combination with topical steroids. In this report, we describe the case of a patient with a typical clinical and immunopathological anti-p200 pemphigoid, responding to a combination of topical steroids and dapsone

Autoimmune Myopathies: Where Do We Stand?

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murin model of anti-TIF1gamma dermatomyositis: preliminary results

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Outcome of anti-PL12 positive patients with antisynthetase syndrome.[Profil évolutif du syndrome des antisynthétases avec anticorps anti-PL12]

International audienceOBJECTIVES: The aim of the present study was to assess the outcome in anti-PL12 patients with antisynthetase syndrome (ASS). METHODS: The medical records of anti-PL12 (n=5) patients with ASS were retrospectively analyzed without prior selection. To exclude false-positive patients, we included patients who were successively tested positive for anti-PL12 antibody at least twice by immunodot and/or Western blot. RESULTS: Anti-PL12 patients experienced: myositis (n=2), Raynaud's phenomenon (n=2), mechanic's hands (n=1), joint impairment (n=4), digestive involvement (n=2), and interstitial lung disease (ILD) (n=4). The two patients with myositis exhibited deterioration of muscle manifestations despite therapy. As regards outcome of ILD, patients developed resolution (n=1), stabilization (n=1) or deterioration (n=2) of pulmonary status. One patient died of pyogenic pneumonia. CONCLUSION: Our series underscores that the presence of anti-PL12 antibody is associated with a particular phenotype of ASS characterized by: (1) less frequent although severe/steroid refractory myositis; (2) less common mechanic's hands and calcinosis cutis; (3) both frequent and severe ILD. Taken together, our findings suggest that PM/DM patients should routinely undergo the search for anti-PL12 antibody as this autoantibody appears to impact patients' prognosis. Furthermore, ILD patients with anti-PL12 antibody should routinely undergo clinical screening for underlying ASS

The IgG2 isotype of anti-transcription intermediary factor 1-gamma autoantibodies is a biomarker of mortality and cancer in adult dermatomyositis

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Mixed muco-cutaneous pemphigoid: Clinical and immunological features of 15 cases

IntroductionWe describe a series of patients whose auto-immune bullous skin disease (AIBD) of the dermal-epidermal junction (DEJ) was characterized by clinical, immunological and ultrastructural features intermediate between bullous pemphigoid (BP) and mucous membrane pemphigoid (MMP), and a recalcitrant course.Patients and MethodsFrom the database of the French reference centre for AIBD, we screened all the patients who were referred for an AIBD of the DEJ with a mucosal involvement, who neither met the diagnostic criteria for the diagnosis of BP, nor were typical of MMP. Sera were analysed by NC16A-ELISA and immunobloting against the C-terminal and LAD-1 parts of BP180. Skin biopsies were studied by direct immunoelectron microscopy (IEM).ResultsFifteen patients (4 males, 11 females) of mean age 70.8 ± 11.8 years were included. The mucosal involvement was localized in oral cavity in all cases and in pharyngeal/laryngeal or genital area in 8 (53%), and 6 patients (40%), respectively. No patient had ocular involvement, nor atrophic or fibrosing scars. All patients had extensive skin lesions (mean BPDAI score =65.9 ± 24.4), which predominated on the upper body part. Direct IEM performed on 8 patients showed IgG deposits on the lamina lucida in all cases, and the lamina densa in 5 cases. All sera recognized NC16A, while none recognized BP-230 in ELISA. 10 out of the 13 tested sera (76.9%) contained IgG which recognized the C-terminal domain of BP180 and 10 sera (76.9%) the LAD-1 domain of BP180. Patients poorly responded to super potent topical corticosteroids and were treated with oral corticosteroids ± immunosuppressant in 13 cases (86.6%).ConclusionThis mixed muco-cutaneous pemphigoid differs from BP by the younger age of patients, multiple mucosae involvement, circulating antibodies against both the C- and N-terminal part of BP180, and very poor response to topical CS. It differs from MMP by extensive inflammatory skin lesions, absence of ocular involvement and atrophic/fibrosing scars

Functional outcome and prognostic factors in anti-Jo1 patients with antisynthetase syndrome

International audienceIntroductionThe aims of this present study were firstly to assess the outcome, including functional course, in anti-Jo1 positive patients with antisynthetase syndrome (ASS), and secondly to determine predictive parameters of poor outcome in these patients.MethodsThe medical records of 86 consecutive anti-Jo1 patients with ASS were reviewed in 4 academic centers.Results13 patients (15.1%) achieved remission of ASS, whereas 55 (63.9%) improved and 18 (20.9%) deteriorated in their clinical status. Both steroid and cytotoxic drugs could be discontinued in only 4.7% of patients. ASS was associated with decreased quality of life at long-term follow-up: only 69.2% of patients considered to be in remission experienced a return to previous normal activities; and 24.7% of other patients with non-remitting ASS still had a marked reduction of activities (as shown by the disability scale of the Health Assessment Questionnaire). Decreased quality of life was further due to calcinosis cutis (8.1%) and adverse effects of steroid therapy (36%). Factors associated with ASS deterioration were older age, pulmonary and esophageal involvement, calcinosis cutis and cancer. Higher anti-Jo1 levels were further associated with disease severity in ASS patients.ConclusionsThe present study shows high morbidity related to ASS. Furthermore, we suggest that patients with predictive factors of ASS deterioration may require more aggressive therapy. Our findings also suggest that in anti-Jo1 patients with severe esophageal manifestations, combined high dose steroids and intravenous immunoglobulins might be proposed as the first line therapy. Finally, as cancer occurred in 14% of anti-Jo1 patients, our findings underscore that the search for cancer should be performed in these patients