Oesophageal involvement in 26 consecutive patients with mucous membrane pemphigoid.

BACKGROUND: Oesophageal involvement of mucous membrane pemphigoid (MMP) has not yet been thoroughly described. OBJECTIVES: To characterize systematically the endoscopic lesions of a series of patients with oesophageal symptoms seen at a referral centre for autoimmune bullous diseases. METHODS: Clinical, endoscopic and immunological findings of consecutively referred patients with MMP with oesophageal involvement, systemic and endoscopic treatments, and follow-up are described. RESULTS: Of 477 consecutive patients with MMP consulting between 2002 and 2012, 26 (5·4%) had symptomatic oesophageal involvement. Dysphagia, observed in 23 (88%) patients, was the most frequent symptom. Oesophageal symptoms could be the first sign of MMP. Patients with oesophageal involvement had a mean of three other involved sites. At initial oesophageal endoscopy, 17 of 26 patients had active lesions (intact bullae, erosions and/or erythema), 15 had stricture(s) and 12 had other cicatricial lesions. Systemic therapy alone achieved oesophageal symptom relief for five patients. Dilatation was combined with systemic therapy for 12 patients and was successful in nine; one perforation occurred. CONCLUSIONS: Symptomatic oesophageal involvement affected 5·4% of patients with MMP. Dermatologists and gastroenterologists should be aware of these mucocutaneous diseases and their oesophageal involvement, as it could lead to earlier diagnosis and better care. Oesophageal dilatation could be a therapeutic option for symptomatic stricture not relieved by optimized systemic therapy alone.

Oral cyclophosphamide without corticosteroids to treat mucous membrane pemphigoid.

BACKGROUND: Mucous membrane pemphigoid (MMP) still represents a potentially life- and sight-threatening disease. Immunosuppressants, such as cyclophosphamide (CYC), are indicated for patients with severe and/or refractory MMP. OBJECTIVES: To evaluate the efficacy and safety of daily oral CYC without corticosteroids as therapy for severe MMP. METHODS: Thirteen patients with severe refractory MMP, who received oral CYC at an initial dose of 2 mg kg(-1) without corticosteroids, were retained. Previous treatments, for example dapsone, sulfasalazine or topical agents, were maintained during CYC treatment. Initial clinical severity and response to treatment were assessed by scoring. CYC was stopped after complete remission (CR), or when MMP progressed or lymphopenia (< 0·7 × 10(9) cells L(-1) ) occurred. RESULTS: After 52 weeks of CYC treatment, the overall response rate was 69% (9/13 patients) with a median time to disease control of 8 weeks (range 4-52 weeks). Seven patients (54%) entered CR with a median time to CR of 24 weeks (range 16-52 weeks), all remaining in CR at week 52. The mean duration of CYC administration was 12 weeks (range 2-52 weeks). The most common side effect was lymphopenia (10/13 patients), which led to CYC withdrawal for six patients. No sepsis was observed. CONCLUSIONS: CYC without corticosteroids had rapid efficacy in patients with severe refractory MMP and was safe.

[Epidermolysis bullosa acquisita: literature review]. / L'épidermolyse bulleuse acquise: revue de la littérature.

INTRODUCTION: Epidermolysis bullosa acquisita (EBA) is the rarest of the autoimmune bullous diseases (AIBD). It is defined as an AIBD secondary to production of antibodies directed against type VII collagen and then binding to anchoring fibrils in the basal membrane zone (BMZ) of the skin and the Malpighian mucosa. AIMS: To evaluate risk factors, different clinical forms and diagnostic methods, and the efficacy of treatments. METHODS: The articles were identified by a search of PubMed and Embase from the initial creation of these databases through to March 2009. We selected generalised reviews and meta-analyses, cases involving unusual and/or serious clinical presentations, studies of immunological tests and homogeneous retrospective series regarding therapy. RESULTS: Of the 206 articles analysed, only two were of an adequate level of proof, with four of intermediate level, and all the others of only low level. EBA affects all age groups (from newborn infants to the very elderly) with a slight predominance in female subjects. Diagnosis must be considered in subjects with black skin of African origin. A drug-induced origin of the disease was reported in 11% of cases of IgA-EBA. Classical EBA (30 to 50% of cases), resembles epidermolysis bullosa hereditaria (EBH), with fragile skin, non-inflammatory bullae, dystrophic scars and milia. Numerous atypical and misleading forms exist. Evocative signs are the presence of mucosal lesions and/or scars. The severity of EBA is determined by the extent of cutaneous lesions, and ophthalmological, ENT and/or oesophageal involvement. Crohn's disease is associated in 25% of cases of EBA. Unequivocal diagnosis is provided by direct immunoelectron microscopy (IEM). Therapeutic efficacy has been reported for dapsone, sulphapyridine and colchicine in milder forms, and for cyclosporine, mycophenolate mofetil, rituximab, intravenous immunoglobulins and extracorporeal photochemotherapy in resistant and severe forms. A number of authors have reported inefficacy of systemic corticosteroids, even in high-dose regimens, with the development of corticosteroid dependence in certain cases. CONCLUSIONS: In the absence of any therapeutic trials, it is difficult to select optimal treatment; however, the benefit/risk ratio of systemic corticosteroid treatment is unfavourable.

[Factors associated with delayed diagnosis of bullous pemphigoid]. / Facteurs associés au retard diagnostique dans la pemphigoïde bulleuse.

BACKGROUND: Bullous pemphigoid (BP) is the most common form of autoimmune bullous dermatosis. The first signs of the disease comprise non-specific lesions. We conducted a retrospective study of two large series of cases of BP to identify parameters related to patients or to the disease affecting time to diagnosis (TD). METHODS: We used files from the "BP1" series retrospectively (comparison of topical and oral corticosteroids) and from the "BP2" series (comparison of two regimens of topical corticosteroids) in order to determine mean and median TD. In each series, patients were divided into two equivalent groups, i.e. patients with short TD (STD), i.e. less or equal to median, and patients with long TD (LTD), i.e. greater than median. Patient-related parameters (age, Karnofsky score, associated neurological diseases) and disease-related parameters (number of blisters and eosinophil count at diagnosis) were compared between these groups. We also investigated for any influence on TD of dermatological demography. RESULTS: Mean TD in the BP2 series was significantly shorter than in the BP1 series (61 versus 91 days, p=0.04) but the median values were similar (31 versus 36 days). There was no difference in patient age, associated neurological diseases or numbers of bullae and eosinophils between the two groups. In contrast, in the BP2 series alone, Karnofsky score was significantly lower in patients with STD (60.25%) than in patients with LTD (66%), p=0.02. Dermatological demographics had no effect on TD. CONCLUSION: Karnofsky score is the only parameter that appears to be associated with TD, with patients with worse scores having a shorter TD, probably due to better medical follow-up. Improved knowledge among general practitioners concerning the initial signs of the disease should shorten TD and accelerate the institution of appropriate therapy.

[Relapsing annular erythema following streptococcal throat infection in an adult patient: Adult erythema marginatum associated with rheumatic fever]. / Erythème annulaire récidivant après angine streptococcique : érythème marginé rhumatismal de l'adulte.

BACKGROUND: Erythema marginatum is one of the main Jones diagnostic criteria for rheumatic fever. However, since it rarely occurs in industrialized countries, this diagnosis is seldom suspected, especially in adult patients. CASE REPORT: We report a case of an annular facial eruption associated with fever and polyarthralgia seen twice in a 30-year-old woman following episodes of streptococcal throat infection. DISCUSSION: This case report underlines the fact that the rheumatic fever has not completely disappeared in the highly developed countries. Dermatologists should be careful not to overlook its clinical manifestations and should be vigilant about potential cardiac complications.