Bullous autoimmune dermatoses

Abstract

SummaryPathophysiologically, bullous autoimmune dermatoses are caused by autoantibodies directed against adhesion molecules or structural proteins of the skin and mucous membranes, clinically resulting in blister formation. Depending on the respective target proteins of the autoimmune response and their location in the skin, a distinction is made between intraepidermal (pemphigus disorders), junctional (pemphigoid disorders), and subepidermal (epidermolysis bullosa acquisita, dermatitis herpetiformis) autoimmune blistering diseases.The most common bullous autoimmune dermatosis, bullous pemphigoid is characterized by marked clinical variability and intense pruritus. Predominantly affecting elderly individuals, there has been a significant increase in its incidence in recent years. While mucosal lesions occur in less than 30 % of bullous pemphigoid patients, the second most common bullous autoimmune dermatosis, pemphigus vulgaris, typically presents with oral erosions as the predominant and – frequently – initial symptom. Its onset is usually in the 4th to 6th decade of life. Scarring is typically found in subepidermal blistering disorders such as epidermolysis bullosa acquisita or mucous membrane pemphigoid.Diagnosis is based on clinical and histological findings as well as direct and indirect immunofluorescence and detection of circulating autoantibodies. Although a number of controlled clinical trials have been conducted in recent years, treatment of bullous autoimmune disorders is still primarily based on clinical experience. Therapeutic options include topical and systemic corticosteroids as well as adjuvant immunosuppressants. Recalcitrant cases may require treatment with immunoadsorption, intravenous immunoglobulins, or the monoclonal anti‐CD20 antibody rituximab.

Blistering and scarring confined to the head

“…Epidermolysis bullosa acquisita (EBA) is a rare acquired chronic subepidermal bullous disease characterized by tense blisters, erythematous plaques, erosions and subsequent scarring [1,2]. First described in 1957 [3], the Brunsting-Perry type is a disease variant that presents with lesions typically localized to the head and neck.…”

“…A u-serrated pattern of antibody deposition is characteristic of EBA [2,6], whereas an n-serrated pattern is typically seen in bullous pemphigoid. While type VII collagen is the main component of the anchoring fibrils in the papillary dermis [1], it is also found in mucous membranes (MM-EBA) ( Table 1). Thus, EBA may be associated with chronic inflammatory bowel disease.…”

Invasive pulmonary aspergillosis associated with bullous pemphigoid

“…Most cases have been reported in patients with hematological malignancy, especially those undergoing hematopoietic stem cell transplantation (HSCT), and in patients with chronic granulomatous diseases, chronic obstructive pulmonary disease, or those undergoing solid organ transplantation. Thus far, there is only one report of IPA in a patient with bullous pemphigoid (BP) [1,2], the most common autoimmune blistering skin disorder [3,4]. Here we report a case of IPA associated with BP that was treated with a moderate dose of oral prednisolone for just three weeks.…”

Bullöse Autoimmundermatosen

“…Bullöse Autoimmundermatosen sind eine bezüglich Altersverteilung, klinischer Präsentation und Immunpathologie heterogene Gruppe von seltenen Erkrankungen, die durch Ablagerung spezifischer Autoantikörper in der Haut und/ oder oberflächennaher Schleimhäute charakterisiert sind [1,2]. Bullöse Autoimmundermatosen können in 3…”