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Anais Brasileiros de Dermatologia 2019Bullous pemphigoid is the most frequent autoimmune bullous disease and mainly affects elderly individuals. Increase in incidence rates in the past decades has been... (Review)
Review
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.
Topics: Aged; Autoimmunity; Diagnosis, Differential; Fluorescent Antibody Technique; Humans; Pemphigoid, Bullous; Steroids
PubMed: 31090818
DOI: 10.1590/abd1806-4841.20199007 -
Medicina (Kaunas, Lithuania) Oct 2021The pemphigoid family of dermatoses is characterized by autoimmune subepidermal blistering. The classic paradigm for pemphigoid, and the most common member, is bullous... (Review)
Review
The pemphigoid family of dermatoses is characterized by autoimmune subepidermal blistering. The classic paradigm for pemphigoid, and the most common member, is bullous pemphigoid. Its variable clinical presentation, with or without frank bullae, is linked by significant pruritus afflicting the elderly. Mucous membrane pemphigoid is an umbrella term for a group of subepidermal blistering dermatoses that favor the mucosal membranes and can scar. Epidermolysis bullosa acquisita is a chronic blistering disorder characterized by skin fragility, sensitivity to trauma, and its treatment-refractory nature. Clinicians that encounter these pemphigoid disorders may benefit from an overview of their clinical presentation, diagnostic work-up, and therapeutic management, with an emphasis on the most frequently encountered pemphigoid disease, bullous pemphigoid.
Topics: Aged; Humans; Mucous Membrane; Pemphigoid, Bullous; Skin Diseases, Vesiculobullous
PubMed: 34684098
DOI: 10.3390/medicina57101061 -
Autoimmunity Reviews May 2017Bullous pemphigoid (BP) is the most common autoimmune bullous disorder which is characterized by autoantibodies against hemidesmosomal proteins of the skin and mucous... (Review)
Review
Bullous pemphigoid (BP) is the most common autoimmune bullous disorder which is characterized by autoantibodies against hemidesmosomal proteins of the skin and mucous membranes. Collagen XVII and dystonin-e have been identified as target antigens. BP affects mostly the elderly. The incidence of the disease is increasing gradually and is associated with high morbidity and mortality. Clinically, BP is characterized by an intensely pruritic eruption with widespread bullous lesions. The clinical diagnosis can be challenging in the setting of atypical presentations. Diagnosis of BP relies on the integration of clinical, histological, immunopathological, and serological findings. The treatment is mainly based on topical and/or systemic glucocorticoids, but anti-inflammatory antibiotics and steroid sparing adjuvants are useful alternatives. Localised and mild BP can be treated with topical corticosteroids alone.
Topics: Autoantibodies; Autoimmune Diseases; Humans; Pemphigoid, Bullous; Skin
PubMed: 28286109
DOI: 10.1016/j.autrev.2017.03.010 -
Medicina (Kaunas, Lithuania) Apr 2021Dyshidrosiform bullous pemphigoid is a variant of bullous pemphigoid. At least 84 patients with dyshidrosiform bullous pemphigoid have been described. Dyshidrosiform... (Review)
Review
Dyshidrosiform bullous pemphigoid is a variant of bullous pemphigoid. At least 84 patients with dyshidrosiform bullous pemphigoid have been described. Dyshidrosiform bullous pemphigoid usually presents with pruritic blisters in elderly individuals; the hemorrhagic or purpuric lesions on the palms and soles can be the only manifestation of the disease. However, bullae may concurrently or subsequently appear on other areas of the patient's body. Patients typically improve after the diagnosis is established and treatment is initiated. The mainstay of therapy is systemic corticosteroids, with or without topical corticosteroids, and systemic dapsone or immunosuppressants. Drug-related or nickel-induced dyshidrosiform bullous pemphigoid improves after stopping the associated agent; however, systemic therapy has also been required to achieve resolution of the blisters. Similar to classic bullous pemphigoid, neurologic conditions and psychiatric disorders have been observed in dyshidrosiform bullous pemphigoid patients. The new onset of recurrent or persistent blisters on the palms, soles, or both of an elderly individual should prompt the clinician to consider the diagnosis of dyshidrosiform bullous pemphigoid.
Topics: Aged; Humans; Immunosuppressive Agents; Pemphigoid, Bullous
PubMed: 33924249
DOI: 10.3390/medicina57040398 -
Annual Review of Pathology May 2016Pemphigus and bullous pemphigoid are autoantibody-mediated blistering skin diseases. In pemphigus, keratinocytes in epidermis and mucous membranes lose cell-cell... (Review)
Review
Pemphigus and bullous pemphigoid are autoantibody-mediated blistering skin diseases. In pemphigus, keratinocytes in epidermis and mucous membranes lose cell-cell adhesion, and in pemphigoid, the basal keratinocytes lose adhesion to the basement membrane. Pemphigus lesions are mediated directly by the autoantibodies, whereas the autoantibodies in pemphigoid fix complement and mediate inflammation. In both diseases, the autoantigens have been cloned and characterized; pemphigus antigens are desmogleins (cell adhesion molecules in desmosomes), and pemphigoid antigens are found in hemidesmosomes (which mediate adhesion to the basement membrane). This knowledge has enabled diagnostic testing for these diseases by enzyme-linked immunosorbent assays and dissection of various pathophysiological mechanisms, including direct inhibition of cell adhesion, antibody-induced internalization of antigen, and cell signaling. Understanding these mechanisms of disease has led to rational targeted therapeutic strategies.
Topics: Animals; Humans; Pemphigoid, Bullous; Pemphigus
PubMed: 26907530
DOI: 10.1146/annurev-pathol-012615-044313 -
Indian Journal of Dermatology,... 2011Bullous pemphigoid (BP) is a relatively common autoimmune vesicobullous disease encountered in India. It is a subepidermal bullous disorder most commonly seen in the... (Review)
Review
Bullous pemphigoid (BP) is a relatively common autoimmune vesicobullous disease encountered in India. It is a subepidermal bullous disorder most commonly seen in the elderly and manifests as tense blisters on urticarial base, predominantly over flexures, and is associated with pruritus. The diagnosis can be confirmed by histology, direct and indirect immunofluorescence. Several new diagnostic techniques have also been developed. Treatment of BP is based on the extent and rate of progression of the disease. Several topical and systemic anti-inflammatory and immunosuppressive agents have been used with variable results.
Topics: Administration, Topical; Animals; Anti-Inflammatory Agents, Non-Steroidal; Humans; Immunosuppressive Agents; India; Pemphigoid, Bullous
PubMed: 21727692
DOI: 10.4103/0378-6323.82398 -
Seminars in Immunopathology Nov 2019Pemphigoid diseases are a group of autoimmune blistering skin diseases defined by an immune response against certain components of the dermal-epidermal adhesion complex.... (Review)
Review
Pemphigoid diseases are a group of autoimmune blistering skin diseases defined by an immune response against certain components of the dermal-epidermal adhesion complex. They are prototypical, autoantibody-driven, organ-specific diseases with the emergence of inflammatory skin lesions dependent on the recruitment of immune cells, particularly granulocytes, into the skin. During an acute flare of disease, inflammatory skin lesions typically progressing from erythema through urticarial plaques to subepidermal blisters erosions erupt and, finally, completely resolve, thus illustrating that resolution of inflammation is continuously executed in pemphigoid disease patients and can be directly monitored on the skin. Despite these superb conditions for examining resolution in pemphigoid diseases as paradigm diseases for antibody-induced tissue inflammation, the mechanisms of resolution in pemphigoid are underinvestigated and still largely elusive. In the last decade, mouse models for pemphigoid diseases were developed, which have been instrumental to identify several key pathways for the initiation of inflammation in these diseases. More recently, also protective pathways, specifically IL-10 and C5aR2 signalling on the molecular level and T on the cellular level, counteracting skin inflammation have been highlighted and may contribute to the continuous execution of resolution in pemphigoid diseases. The upstream orchestrators of this process are currently under investigation. Pemphigoid disease patients, particularly bullous pemphigoid patients, who are predominantly above 75 years of age, often succumb to the side effects of the immunosuppressive therapeutics nowadays still required to suppress the disease. Pemphigoid disease patients may therefore represent a group of patients benefiting most substantially from the introduction of non-immunosuppressive, proresolving therapeutics into the treatment regimens for their disease.
Topics: Animals; Autoimmunity; Biomarkers; Disease Management; Disease Susceptibility; Epidermis; Humans; Inflammation Mediators; Lipid Metabolism; Pemphigoid, Bullous; Skin
PubMed: 31732776
DOI: 10.1007/s00281-019-00759-y -
Journal of the American Academy of... May 1987Bullous pemphigoid is an autoimmune blistering dermatologic disease characterized clinically by tense bullae that may develop on normal or erythematous skin. The major... (Review)
Review
Bullous pemphigoid is an autoimmune blistering dermatologic disease characterized clinically by tense bullae that may develop on normal or erythematous skin. The major histologic feature is a subepidermal blister with variable degrees of dermal inflammation. Other immunologically mediated blistering skin diseases may mimic bullous pemphigoid, including herpes gestationis, cicatricial pemphigoid, dermatitis herpetiformis, and epidermolysis bullosa acquisita. These diseases will be discussed. Most patients with bullous pemphigoid demonstrate circulating autoantibodies reactive with an antigen located in the lamina lucida region of the basement membrane zone. Complement activation by these autoantibodies initiates influx and activation of mast cells along with other inflammatory cells. Tissue injury with damage and eventual destruction of the basement membrane occurs as a result of the release of inflammatory mediators. The final result is subepidermal blister formation. The course of bullous pemphigoid tends to be self-limited, and successful treatment is usually effected with systemic glucocorticosteroids.
Topics: Animals; Autoimmune Diseases; Dermatitis Herpetiformis; Diagnosis, Differential; Disease Models, Animal; Epidermolysis Bullosa; Female; Humans; Pemphigoid Gestationis; Pemphigoid, Bullous; Pregnancy; Pregnancy Complications; Skin Diseases, Vesiculobullous
PubMed: 3294944
DOI: 10.1016/s0190-9622(87)70115-7 -
Journal of the American Academy of... May 2020
Topics: Humans; Pemphigoid, Bullous; Rituximab
PubMed: 32119892
DOI: 10.1016/j.jaad.2020.02.058 -
Acta Dermato-venereologica Aug 2020Bullous pemphigoid is an autoimmune subepithelial disease characterised by pruritus followed by urticarial plaques and finally bullae on the skin and mucosa....
Bullous pemphigoid is an autoimmune subepithelial disease characterised by pruritus followed by urticarial plaques and finally bullae on the skin and mucosa. Drug-associated bullous pemphigoid (DABP) is a term used to describe instances of bullous pemphigoid demonstrating clinical, histological, or immunopathological features identical or similar to those of the idiopathic form of bullous pemphigoid, associated with the systemic ingestion, or topical application of particular drugs. In this study, we conducted a comprehensive search of the literature according to PRISMA guidelines and a total of 170 publications were included in the final qualitative analysis. In conclusion, 89 drugs were implicated in DABP. The strongest evidence for DABP is seen with gliptins, PD-1/PD-L1 inhibitors, loop diuretics, penicillin and derivatives. An appreciation of the medications associated with bullous pemphigoid enables clinicians to identify potential cases of DABP earlier and cease the offending medication.
Topics: Blister; Humans; Pemphigoid, Bullous; Pharmaceutical Preparations; Pruritus; Skin
PubMed: 32176310
DOI: 10.2340/00015555-3457