Bullous pemphigoid

Abstract

Bullous pemphigoid is the most frequent autoimmune bullous disease and mainly affects elderly individuals. Increase in incidence rates in the past decades has been attributed to population aging, drug-induced cases and improvement in the diagnosis of the nonbullous presentations of the disease. A dysregulated T cell immune response and synthesis of IgG and IgE autoantibodies against hemidesmosomal proteins (BP180 and BP230) lead to neutrophil chemotaxis and degradation of the basement membrane zone. Bullous pemphigoid classically manifests with tense blisters over urticarial plaques on the trunk and extremities accompanied by intense pruritus. Mucosal involvement is rarely reported. Diagnosis relies on (1) the histopathological evaluation demonstrating eosinophilic spongiosis or a subepidermal detachment with eosinophils; (2) the detection of IgG and/or C3 deposition at the basement membrane zone using direct or indirect immunofluorescence assays; and (3) quantification of circulating autoantibodies against BP180 and/or BP230 using ELISA. Bullous pemphigoid is often associated with multiple comorbidities in elderly individuals, especially neurological disorders and increased thrombotic risk, reaching a 1-year mortality rate of 23%. Treatment has to be tailored according to the patient's clinical conditions and disease severity. High potency topical steroids and systemic steroids are the current mainstay of therapy. Recent randomized controlled studies have demonstrated the benefit and safety of adjuvant treatment with doxycycline, dapsone and immunosuppressants aiming a reduction in the cumulative steroid dose and mortality.

Dyshidrosiform Bullous Pemphigoid: Case Reports and Review

“…An association between bullous pemphigoid and neurologic or psychiatric disorders has been identified [1][2][3]. Both of the men in this report had neurologic disorders, either epilepsy or Parkinsonism.…”

“…Bullous pemphigoid is an autoimmune blistering condition that usually occurs in elderly individuals. The immunopathogenesis of the disease is attributed to circulating and tissuebound autoantibodies directed against bullous pemphigoid antigen 230 (bullous pemphigoid antigen 1) or bullous pemphigoid antigen 180 (bullous pemphigoid antigen 2) or both [1][2][3]. The condition typically presents as pruritic tense subepidermal blisters.…”

“…In addition to the lower trunk, the lesions frequently appear on the proximal flexural aspects of the arms (near the axilla) and legs (near the groin). The lesions can be localized or widespread [1][2][3].…”

“…Unusual clinical variants of bullous pemphigoid have been observed in literature [1][2][3]. Dyshidrosiform bullous pemphigoid refers to the condition when the blisters are initially or persistently localized to the palms and soles [4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19].…”

Should SARS‐CoV‐2 influence immunosuppressive therapy for autoimmune blistering diseases?

“…In particular, we asked whether patients in immunosuppressive therapy Among possible trigger factors of autoimmune blistering diseases, immunization and viral infections are mentioned in the literature, although the underlying immunological mechanism is still unclear. [7][8][9] The most acceptable hypothesis involves the possible molecular mimicry existing between viral and epidermal proteins, and over activation of the immune system as a consequence of the viral attack. 9 Indeed, in autoimmune blistering disorders, once the autoantibodies bind to…”