Objective measures of disease activity have attracted considerable interest within dermatology because of their capacity to evaluate disease severity through a validated means, to monitor the trajectory of a patient’s disease and as standardised outcome measures in clinical trials (1). Two disease-specific instruments exist for measuring the extent of autoimmune bullous diseases: the Autoimmune...

Pemphigus vulgaris (PV) is a rare, potentially life threatening, autoimmune blistering skin disease. The International Pemphigus and Pemphigoid Foundation (IPPF) has recently developed a disease registry with the aim to enhance our understanding of autoimmune bullous diseases with the long-term goal of acquiring information to improve patient care. Patients were recruited to the IPPF disease re...

BACKGROUND Bullous pemphigoid is the most common autoimmune blistering skin disease of the elderly. Because elderly people have low tolerance for standard regimens of oral corticosteroids, we studied whether highly potent topical corticosteroids could decrease mortality while controlling disease. METHODS A total of 341 patients with bullous pemphigoid were enrolled in a randomized, multicente...

Bullous pemphigoid is an autoimmune sub epidermal blistering dermatosis that is uncommon in childhood. We report two cases of children with bullous pemphigoid. The first one was a case of a 4-month-old female infant, whereas the second is of a 3-month-old male infant. The two cases presented clinical and laboratory data for the confirmatory diagnosis of bullous pemphigoid. Opportunely we review...

INTRODUCTION Anti-p200 pemphigoid is a rare autoimmune subepidermal blistering disease, first described in 1996. This disorder was initially considered as a more benign condition compared with bullous pemphigoid and epidermolysis bullosa acquisita, but in a recent series of patients, heterogeneous clinical presentations have been described, including cases with a more severe course than previou...

BACKGROUND Autoimmune polyendocrinopathy candidiasis ectodermal dystrophy (APECED) is a rare autosomal recessive disorder caused by mutations of the autoimmune regulator (AIRE) gene, whose loss of function leads to the escape of self-reactive T cells from the thymus and autoimmunity. APECED patients typically develop tissue-specific autoantibodies and anti-cytokine antibodies. Consequently, var...

INTRODUCTION  Bullous systemic lupus erythematosus (BSLE) is an autoantibody-mediated disease with subepidermal blisters. It is a rare form of presentation of SLE that occurs in less than 5% of cases of lupus. CASE REPORT  A 27-year-old, female, FRS patient reported the appearance of painful bullous lesions in the left nasal wing and left buccal mucosa that displayed sudden and rapid growth. ...