Bullous pemphigoid (BP) is the most common autoimmune subepidermal blistering disease in Western countries, and typically affects the elderly. BP is immunologically characterized by tissue-bound and circulating autoantibodies directed against either the BP antigen 180 (BP180, or BPAG2) or the BP antigen 230 (BP230, or BPAG1e), or even both, which are components of hemidesmosomes involved in the dermal-epidermal cohesion. Risk factors for BP include old age, neurologic diseases (dementia, Parkinson's disease, cerebrovascular disease), and some particular drugs, including loop diuretics, spironolactone and neuroleptics. The spectrum of clinical presentations is extremely broad. Clinically, BP is an intensely pruritic erythematous eruption with widespread blister formation. In the early stages, or in atypical, non-bullous variants of the disease, only excoriated, eczematous or urticarial lesions (either localized or generalized) are present. The diagnosis of BP relies on immunopathologic findings, especially based on both direct and indirect immunofluorescence microscopy observations, as well as on anti-BP180/BP230 enzyme-linked immunosorbent assays (ELISAs). BP is usually a chronic disease, with spontaneous exacerbations and remissions, which may be accompanied by significant morbidity. In the past decade, potent topical corticosteroids have emerged as an effective and safe first-line treatment for BP, but their long-term feasibility is still controversial. Newer therapeutic agents targeting molecules involved in the inflammatory cascade associated with BP represent future alternatives to classical immunosuppressant drugs for maintenance therapy.

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Connective tissue diseases (CTDs) such as systemic lupus erythematosus, systemic sclerosis, myositis, Sjögren’s syndrome, and rheumatoid arthritis are systemic diseases which are often associated with a challenge in diagnosis. Autoantibodies (AAbs) can be detected in these diseases and help clinicians in their diagnosis. Actually, pathophysiology of these diseases is associated with the presence of antinuclear antibodies. In the last decades, many new antibodies were discovered, but their implication in pathogenesis of CTDs remains unclear. Furthermore, the classification of these AAbs is nowadays misused, as their targets can be localized outside of the nuclear compartment. Interestingly, in most cases, each antibody is associated with a specific phenotype in CTDs and therefore help in better defining either the disease subtypes or diseases activity and outcome. Because of recent progresses in their detection and in the comprehension of their pathogenesis implication in CTD-associated antibodies, clinicians should pay attention to the presence of these different AAbs to improve patient’s management. In this review, we propose to focus on the different phenotypes and features associated with each autoantibody used in clinical practice in those CTDs.