British journal of dermatology british association of dermatologists guidelines for the management of bullous pemphigoid 2012 v. Bullous skin disease, skin blistering, vaccination. Files are available under licenses specified on their description page. Bullous pemphigoid is characterized by the presence of immunoglobulin g igg autoantibodies specific for the hemidesmosomal bullous pemphigoid antigens bp230 bpag1 and bp180 bpag2. The blisters may break open and form ulcers or open sores. It was thought that sodium chloride splitskin immunofluorescence could reliably distinguish. Bullous pemphigoid investigations bmj best practice. See epidemiology and pathogenesis of bullous pemphigoid and mucous membrane pemphigoid and clinical features and diagnosis of bullous pemphigoid and mucous membrane pemphigoid. Autoantibodies to type vii collagen were identified by using recessive dystrophic epidermolysis bullosa skin which lacks type vii collagen in an indirect immunofluorescence assay. Pemphigoid diseases including bullous pemphigoid, mucous membrane. Pdf epidermolysis bullosa acquisitaa pemphigoidlike. Bullous pemphigoid is an autoimmune disease characterized by subepidermal blister formation.
Bullous pemphigoid is fairly easy to treat, with most patients responding to prednisone. Bullous pemphigoid, mucous membrane pemphigoid, and. In pemphigoid, autoantibodies target molecules involved in connecting basal epithelial cells to the basement membrane in hemidesmosomes, such as type xvii collagen col17, bp180, dystonine bp230, type vii collagen col7, p200, and laminin 332 2, 3. Bullous pemphigoid is an uncommon blistering disease of the elderly, which often starts with itch and urticated and erythematous lesions. Differentiating between bullous pemphigoid and epidermolysis bullosa acquisita is often difficult because of the similar clinical courses and immunofluorescence. Based on the results of this study we propose a treatment algorithm for infantile bp. The guidelines reflect data available from medline, embase, the cochrane library, literature searches and the experience of the authors of managing patients with bullous pemphigoid in special and general clinics for over 10 years. As with other autoimmune disorders, it is caused when the bodys defenses mistake its own tissues as foreign, and attack the cells. A 42yearold thai man presented to our faculty after developing. Pde4 inhibition as potential treatment of epidermolysis. Home bullous pemphigoid bp pdf bullous pemphigoid bp pdf file.
Occasionally, the inner lining tissue of the mouth, nasal passages, or conjunctivae of the eyes mucous membrane tissue can be involved. Brunstingperry pemphigoid is a rare variant of cicatricial pemphigoid. Recent advances in the understanding and treatment of. There is a danger of serious infection occurring on raw areas of skin. Bullous pemphigoid bulus pemfihgoid is a chronic inflammatory, but rare skin disorder that is characterized by large, fluidfilled blisters these blisters usually develop on the areas of skin that often flex such as the lower abdomen, upper thighs or armpits. There have been reports of various adverse effects associated with the use of atenolol including bullous pemphigoid.
Bullous pemphigoid is the most frequent autoimmune bullous disease and mainly affects elderly individuals. Later, large tense blisters develop on both erythematous and on normal skin and there may be mucosal involvement with blisters and erosions. Bullous pemphigoid primary care dermatology society uk. Can be selflimiting but when necessary treatment includes the use of corticosteroids. Bullous pemphigoid bp is caused by autoantibodies directed against hemidesmosomal proteins. Bullous pemphigoid is the commonest type of autoimmune blistering disease, with an incidence of 12. Pemphigus foliaceus, 3 bullous pemphigoid, 4 mucous membrane pemphigoid, and 5 epidermolysis bullosa acquisita. When there is oral involvement, mmp lesions should be differentiated from pemphigus vulgaris, bullous pemphigoid, erythema multiforme, and erosive lichen planus.
Increase in incidence rates in the past decades has been attributed to population aging. It is classified as a type ii hypersensitivity reaction, with the formation of antihemidesmosome antibodies. Three epidermolysis bullosa acquisita biopsies and seven bullous pemphigoid biopsies examined demonstrated igg staining at sites consistent with their respective diagnoses. They can result in scarring, which may lead to disabling and life threatening complications. A recent publication by zhu and coworkers1 examined antibodypositive serum samples from 100 sequential patients on sodium chloridesplit skin and by western immunoblot to distinguish bullous pemphigoid from epidermolysis bullosa acquisita. Japanese guidelines for the management of pemphigoid. This thesis focuses on the clinical symptoms, diagnosis and treatment in pemphigoid diseases, especially bullous pemphigoid bp, mucous membrane pemphigoid. The vast majority of those affected are elderly, but it has been seen at all ages. Epidermolysis bullosa acquisita is a chronic condition in which blisters and lesions spontaneously appear on otherwise healthylooking skin, either with no apparent cause or caused by minor trauma usually to the elbows, knees, ankles, and buttocks. When there is oral involvement, mmp lesions should be differentiated from pemphigus vulgaris, bullous pemphigoid, erythema multiforme, and. Bjd british journal of dermatology british association of. Epidermolysis bullosa acquisita clinical presentation.
Bullous pemphigoid and pemphigus vulgaris request pdf. Pemphigus support pemphigoid support local support groups. All structured data from the file and property namespaces is available under the creative commons cc0 license. It might present with blisters or welts seen on arms, legs, groin, mouth or abdomen. Pemphigoid is a rare autoimmune disorder that can develop at any age, including in kids, but that most often affects the elderly. The signs and symptoms of bullous pemphigoid may include. Differentiation of bullous pemphigoid from epidermolysis. Itching skin, weeks or months before blisters form. Bullous pemphigoid, mucous membrane pemphigoid, and epidermolysis bullosa acquisita this chapter, in addition to chapters 23 and 25, cover the autoimmune bullous diseases. Consensus on the treatment of autoimmune bullous dermatoses. A case of localized bullous pemphigoid that mimicked an acute contact dermatitis around a stoma site is presented. Dapsone often is used to control the disease, though this therapy for eba is not cur. As a result, we diagnosed our patient as having the brunstingperry pemphigoid variant of epidermolysis bullosa acquisita eba.
Jci the pathophysiology of autoimmune blistering diseases. Bullous pemphigoid bp is an acquired autoimmune subepidermal bullous disease in which autoantibodies are directed against components of the basement. Knowledge of the pathophysiology of immunobullous diseases has been advanced by the demonstration that passive transfer of antibodies against skin autoantigens can induce blisters in experimental animals with clinical, histologic, and immunopathologic features similar to those seen in human patients. Large blisters that dont easily rupture when touched, often along creases or folds in the skin. Bullous pemphigoid and epidermolysis bullosa acquisita.
Bullous pemphigoid an overview sciencedirect topics. Overview of serological diagnostics in autoimmune blisterforming diseases of the skin. Pde4 inhibition as potential treatment of epidermolysis bullosa acquisita hiroshi koga1,7, andreas recke1, gestur vidarsson2, hendri h. The pemphigoid group is a category of autoimmune subepidermal blistering diseases in which autoantibodies deposit linearly at the epidermal basement membrane zone bmz. Bullous pemphigoid symptoms and causes mayo clinic. Bullous pemphigoid occurs more often in people over age 60 but can occur in children. Floorpattern saltsplit skin cannot distinguish bullous. Bullous pemphigoid bp, pemphigoid, levers pemphigoid. Treatments include corticosteroids, mycophenolate mofetil and cyclophosphamide to suppress the. A case of oropharyngeal bullous pemphigoid presenting with.
You may do so in any reasonable manner, but not in. Bullous pemphigoid commonly lasts 15 years and then often eases or goes away. It facilitates differential diagnosis of the acquired subepidermal bullous skin disorders, bullous pemphigoid, and epidermolysis bullosa acquisita, in which ultrastructural identification of the skin site with skinbound igg deposits at the epidermal basement membrane zone may. The comparison and contrast of molecular mechanism of blister formation of these two diseases provide a rational diagnostic and therapeutic approach to affected patients. Bullous pemphigoid is the most commonly seen autoimmune blistering disease in the. It ranges from mildly itchy welts to severe blisters and infection, and may affect a small area of the body or be widespread. Bullous pemphigoid is well known for its cutaneous features. It is a less serious disease than pemphigus vulgaris which also causes blistering, is not usually fatal, and does not result in widespread peeling of skin. Bullous pemphigoid is often associated with multiple comorbidities in elderly individuals, especially neurological disorders and increased thrombotic risk, reaching a 1year mortality rate of 23%. Primary herpes recurrent herpes erosive lichen planus mucous membrane pemphigoid pemphigus vulgaris bullous pemphigoid lupus erythematosus epithelial dysplasia carcinoma in situ squamous cell carcinoma. Iii i i i bullous pemphigoid and rheumatoid arthritis j.
Bullous pemphigoid usually occurs in older persons and is rare in young people. Guidelines for the management of bullous pemphigoid british. In some people, the mouth or genitals are also affected. The main subtypes of pemphigoid mediated by immunoglobulin g autoantibodies are bullous pemphigoid bp, mucous membrane pemphigoid mmp and epidermolysis bullosa acquisita. This revision to the coverage issues manual is a national coverage decision ncd. Diseases with subepidermal blistering pemphigoid group 14.
Kirtschig5 1department of dermatology, churchill hospital, old road, headington, oxford ox3 7lj, u. Bullous pemphigoid is an autoimmune pruritic skin disease preferentially in elderly people, that may involve the formation of blisters in the space between the epidermal and dermal skin layers. Unlike the pemphigus group of diseases, the autoantibodies deposited in the bmz are not suffi. Bullous pemphigoid in infants orphanet journal of rare diseases. As its name indicates, pemphigoid is similar in general appearance to pemphigus, but, unlike pemphigus, pemphigoid does not feature acantholysis, a loss of connections between skin cells. Pemphigoid diseases with the exclusive iga involvement are. Bullous pemphigoid is a skin disorder characterized by large blisters. The diagnosis was confirmed by direct immunofluorescence and by indirect immunofluorescence on 1. Epidermolysis bullosa acquisitaa pemphigoid like disease. We report three cases of bullous pemphigoid in adults younger than 50 years old, discussing the clinical characteristics of the disease in younger patients. Skin around the blisters that is normal, reddish or darker than normal. This disease typically affects the elderly and presents with itch and localized or generalized bullous lesions.
Bullous pemphigoid bullous pemphigoid is the most common ambd, with approximately 2,100 new cases diagnosed in the united states each year. This accounts for more than onehalf of newly diagnosed cases of ambd, says david hamrock, m. Diagnosis is by skin biopsy and immunofluorescence testing of skin and serum. Pemphigus support pemphigoid support local support. The bullae are formed by an immune reaction, initiated by the formation of igg autoantibodies targeting dystonin, also called bullous pemphigoid antigen 1, andor type xvii collagen, also called bullous pemphigoid antigen 2, which is a component of hemidesmosomes. Pemphigus and bullous pemphigoid are distinct autoimmune blistering diseases that are characterised by the presence of autoantibodies directed against specific adhesion molecules of the skin and mucous membranes. Bullous pemphigoid is a chronic autoimmune skin disorder resulting in generalized, pruritic, bullous lesions in elderly patients. The igg appeared in the dermal side of the split biopsies in epidermolysis bullosa acquisita and predominantly, or exclusively, in the epidermal side in bullous pemphigoid. The blisters are usually located on the arms, legs, or middle of the body. If untreated, the blisters and raw areas of skin can cause much discomfort. Epidermolysis bullosa acquisita eba is a rare autoimmune blistering disorder characterized by tense bullae, skin fragility, atrophic scarring, and milia formation. Although these are the most common autoimmune blistering diseases, physicians at penn treat all autoimmune blistering diseases including epidermolysis bullosa acquisita, linear lga bullous dermatosis, p200 pemphigoid, mucous membrane pemphigoid, dermatitis herpetiformis, bullous lupus and others. Bp is an autoimmune subepidermal bullous dermatosis defined immunologically by the existence of autoantibodies directed against 2 structural proteins found in the hemidesmosomes of the dermalepidermal junction.
Repeat direct immunofluorescence to discriminate pemphigoid. Ludwig1,6 pemphigoid diseases such as epidermolysis bullosa acquisita eba may be dif. Bullous pemphigoid bp is the most common autoimmune subepidermal blistering disorder, representing 80% of subepidermal. Epidermolysis bullosa acquista eba is an acquired, subepidermal bullous disease with clinical features similar to the genetic forms of dystrophic epidermolysis bullosa deb. In human beings the autoantibodies are directed toward bullous pemphigoid antigen 1e bpag1e, a 230kd intercellular antigen, and type xvii collagen also called bpag2, a 180kd hemidesmosomal transmembrane molecule. In these diseases, ultrastructural identification of the site of skinbound igg deposits at the epidermal. Mar 05, 2019 epidermolysis bullosa acquisita eba is a chronic autoimmune subepidermal blistering disease of the skin and mucus membranes. Ce 110 a guide to clinical differential diagnosis of. Anatomical level of splitblister separation plane 2. Effectiveness and safety of rituximab in recalcitrant. Bullous pemphigoid may be localised to one area, or widespread on the trunk and proximal limbs.
This file is licensed under the creative commons attributionshare alike 3. In up to 20% of affected patients, bullae may be completely absent, and only excoria. Pemphigus is an autoimmune disorder that causes blistering and raw sores on skin and mucous membranes. They develop on areas of skin that often flex such as the lower abdomen, upper thighs or armpits. Other mucosal sites that might be affected include the nasopharnyx, larynx, genitalia, rectum, and esophagus. It probably represents a heterogeneous disorder with several target antigens. A 42yearold thai man presented to our faculty after. Mucous membrane pemphigoid genetic and rare diseases. Bullous means blistering and pemphigoid comes from the greek word pemphix and means bubbles. Goals of treatment the major goals for the treatment of bullous pemphigoid bp are as follows. Pemphigoid is caused by a malfunction of the immune system and.
Sep 14, 2018 the main differential diagnoses in mmp include bullous pemphigoid, epidermolysis bullosa acquisita, and linear iga dermatosis. Oct 31, 2012 atenolol is commonly used by patients with hypertension, angina pectoris, or myocardial infarction. The disease course is chronic with exacerbations and remissions. Bullous pemphigoid is an uncommon skin disease characterized by tense blisters on the surface of the skin. Epidermolysis bullosa acquisita is characterized clinically by blisters, scars, and milia primarily at the traumaprone areas. Bullous pemphigoid is the most common autoimmune subepidermal blistering disease of the skin and mucous membranes. Pemphigoid diseases including bullous pemphigoid, mucous membrane pemphigoid, pemphigoid gestationis, linear iga dermatosis, lichen planus pemphigoides, and antip200 pemphigoid are a subgroup of autoimmune bullous skin diseases characterized by an autoantibody response toward structural components of the hemidesmosome resulting in subepidermal. University of groningen studies on clinical symptoms.
Aug 10, 2016 bullous pemphigoid is a skin disorder characterized by large blisters. Management of bullous pemphigoid bp consists of general skin care measures as well as pharmacologic therapy and is aimed at attenuating autoantibody inflammation to promote healing, decrease morbidity, and improve quality of life. Bullous pemphigoid merck manuals professional edition. Unilocular, subepidermal, nonacantholytic blisters with festooning suspended in a loop between two points of dermal papillae, infiltrate including eosinophils located in blister cavity and in the dermis.
Pemphigus and pemphigoid are characterized as autoimmune blistering diseases in which immunoglobulin g autoantibodies cause blisters and erosions of the skin or mucosa or both. Bullous pemphigoid is the most common autoimmune dermatosis presenting with crops of tense pruritic blisters, often in older adults. Some patients have a diagnosis of bullous pemphigoid made despite not having any bullae nonbullous pemphigoid. How to apply for disability benefits with a bullous disease. Patients with epidermolysis bullosa acquisita eba and bullous pemphigoid bp can present with similar clinical features. Blistering disease, pemphigus, pemphigoid penn medicine. As its name indicates, pemphigoid is similar in general appearance to pemphigus, but, unlike pemphigus, pemphigoid does not feature acantholysis, a loss of connections between skin cells pemphigoid is more common than pemphigus, and is slightly more common in women than in men. Bullous pemphigoid bulus pemfihgoid is a rare skin condition that causes large, fluidfilled blisters. Bullous pemphigoid bullous pemphigoid bp is a chronic blistering of the skin. The ncds are binding on all medicare carriers, intermediaries. The most common of these diseases is bullous pemphigoid, which mainly affects older people and the reported incidence of which in europe. In deb there is a hereditary defect in the gene that encodes type vii collagen c7, the major component of anchoring fibrils.
Bullous pemphigoid and rheumatoid arthritis sciencedirect. Mucous membrane pemphigoid and epidermolysis bullosa acquisita are rare autoimmune blistering diseases of the skin and mucous membranes eyes and mouth. Aug 10, 2018 bullous pemphigoid is a chronic, autoimmune, subepidermal, blistering skin disease that rarely involves mucous membranes. Recently, understanding of the pathophysiology of pemphigus and pemphigoid has been furthered by genetic analyses, characterization of autoantibodies and autoreactive b cells, and elucidation of cellcell adhesion between keratinocytes. Atenolol is commonly used by patients with hypertension, angina pectoris, or myocardial infarction. Mucosal involvement may occur and a number of clinical subtypes exist. Last, classification may be based on predominant class of autoantibodies igg or iga. Bullous pemphigoid is a subepidermal autoimmune blistering disease. Louisville, ky a case is reported in which a patient with longstanding rheumatoid arthritis r. It can involve a large portion of the skin, however, and can be very uncomfortable. Bullous pemphigoid bp pdf national blood authority. The main subtypes of pemphigoid mediated by immunoglobulin g autoantibodies are bullous pemphigoid bp, mucous membrane pemphigoid mmp and epidermolysis bullosa acquisita eba. Studies on clinical symptoms, diagnosis and treatment in pemphigoid diseases 10 1.
The location of igg deposits after splitting allows differentiation of bp from epidermolysis bullosa acquisita, antilaminin332 mucous membrane pemphigoid and anti. Pemphigoid is a group of rare autoimmune blistering skin diseases. Bullous pemphigoid bp and pemphigoid gestationis pg are acquired autoimmune subepidermal blistering diseases characterized by autoantibodies against the hemidesmosomal proteins bp180type xvii. Blisters inside the mouth and in genital sites are uncommon. Guidelines for the management of bullous pemphigoid. Bullous dermatitis an overview sciencedirect topics. Bullous pemphigoid skin disorders msd manual consumer version. The main differential diagnoses in mmp include bullous pemphigoid, epidermolysis bullosa acquisita, and linear iga dermatosis. A different form of dystonin is associated with neuropathy. Mucous membrane pemphigoid in a patient with hypertension.
Bullous pemphigoid genetic and rare diseases information. We report a case of bullous pemphigoid presenting with haemoptysis, initially presenting to the ear, nose and throat department for investigation. Epidermolysis bullosa herpes simplex herpangina hand, foot and mouth disease herpes zoster infectious mononucleosis varicella chickenpox. Bullous pemphigoid, dermatitis herpetiformis, epidermolysis bullosa, pemphigus vulgaris, and porphyria cutanea tarda are rare, blistering disorders that are important because of their significant mortality or morbidity table 10. It is characterized clinically by blisters, hemorrhagic crusts and atrophic scars con. They should be considered when the more common causes of blistering disease have been ruled out and appropriate. Bullous pemphigoid is a rare autoimmune skin disorder that usually, but not always, affects people who are 60 years old or older. Blister fluid filled cavity within or beneath the epidermis vesicles 0. To the best of our knowledge we present the first case report of atenololinduced mucous membrane pemphigoid. From previous studies, we concluded that the fluorescence overlay antigen mapping foam technique could be of value to the differential diagnosis of the acquired subepidermal bullous skin disorders, bullous pemphigoid bp and epidermolysis bullosa acquisita eba. Jonkman3, takashi hashimoto4, anika kasprick1, saeedeh ghorbanalipoor1, hermann tenor5, detlef zillikens1,6 and ralf j. Topical and systemic corticosteroids are used initially. Definition bp is an autoimmune subepidermal bullous dermatosis defined immunologically by the existence of autoantibodies directed against 2 structural proteins found in the hemidesmosomes of the dermalepidermal junction.