If untreated, it can persist for months or years, with periods of spontaneous remissions and exacerbations. Bullous pemphigoid is an autoimmune blistering disorder that typically presents in elderly patients as pruritic tense subepidermal blisters on the lower trunk, axilla, and groin. Association of scabies with a bullous pemphigoidlike eruption. Bullous pemphigoid bp is an autoimmune subepidermal bullous disease in which autoantibodies are directed against components of the basement membrane. Bullous pemphigoid bp, pemphigoid, levers pemphigoid. 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. Mucous membrane pemphigoid mmp, previously known as cicatricial pemphigoid, is a systemic cicatrizing autoimmune disease characterized by chronic blistering of mucous membranes, including ocular, oral, genital, nasopharyngeal, anogenital, and laryngeal mucosa, with ocular 60. Bullous pemphigoid is a rare skin condition that typically affects people in middle age and beyond. Bullous pemphigoid is an uncommon blistering disease of the elderly, which often starts with itch and urticated and erythematous lesions.
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. Mucous membrane pemphigoid an overview sciencedirect. Genetic and acquired bullous diseases fitzpatricks color. The disease can be fatal, particularly in patients who are debilitated. A few patients have skin involvement which is usually around the head and neck or sites of skin trauma.
Bullous pemphigoid and pemphigus vulgaris request pdf. The immunobullous diseases bullous pemphigoid bp and epidermolysis bullosa acquisita eba are very rare in childhood. Bullous pemphigoid diagnosis and treatment mayo clinic. Minimal diagnostic criteria for pemphigoid are needed but have not yet been established. Although case studies have been detailed, there are no reports of a large series of patients documenting the effectiveness of treatment and long. Goyal, nupur and rao, raghavendra and shenoi, shrutakirthi d and pai, sathish b and kumar, pramod 2017 epidermolysis bullosa acquisita and antip200 pemphigoid as major subepidermal autoimmune bullous diseases diagnosed by floor binding on indirect immunofluorescence microscopy using human saltsplit skin.
Topical and systemic corticosteroids are used initially. Diagnosis is by skin biopsy and immunofluorescence testing of skin and serum. Bullous pemphigoid symptoms and causes mayo clinic. Bullous pemphigoid primary care dermatology society uk. Pemphigoid is a group of rare autoimmune blistering skin diseases. Pdf epidermolysis bullosa acquisitaa pemphigoidlike. Bullous pemphigoid bp affects both mucosal and cutaneous surfaces. Dystrophic epidermolysis bullosa deb is a congenital inherited blistering disorder caused by mutations in col7a1, the gene encoding collagen vii col7, which is a major structural component of anchoring fibrils of the hemidesmosome. Thus the direct immunofluorescent study of previously frozen and subsequently saltsplit skin biopsies may be used for the differential diagnosis of bullous pemphigoid from. Systemic corticosteroids have been the mainstay of therapy for severe or extensive disease but impose an increased risk for postoperative complications including surgical site infections. The gold standard for diagnosis of epidermolysis bullosa acquisita vs bullous pemphigoid was taken to be identification of immune deposits on the dermal side floor for epidermolysis bullosa acquisita or the epidermal side roof for bullous pemphigoid of the saltsplit direct immunofluorescence specimen. Epidermolysis bullosa acquisita eba is an orphan autoimmune disease. Mucous membrane pemphigoid and epidermolysis bullosa acquisita can evolve with cicatricial mucosal involvement, leading to respiratory, ocular andor.
Generalized atrophic benign epidermolysis bullosa gabeb is a form of nonlethal junctional epidermolysis bullosa characterized by universal alopecia and atrophy of the skin. Successful treatment of refractory epidermolysis bullosa. Feb 23, 2015 bullous pemphigoid bp is a chronic, autoimmune, subepidermal, blistering skin disorder unlike pemphigus where the blistering is intraepidermal. Bullous pemphigoid may present with several distinct clinical. 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. Contractors have the discretion to define what constitutes failure of conventional therapy.
In these diseases, ultrastructural identification of the site of skin. Treatment usually helps heal the blisters and ease any itching. The blisters are usually located on the arms, legs, or middle of the body. Junctional epidermolysis bullosa jeb is a heterogeneous autosomal recessively inherited blistering skin disorder associated with fragility at the dermalepidermal junction1. Epidermolysis bullosa acquisita and antip200 pemphigoid as. Later, large tense blisters develop on both erythematous and on normal skin and there may be mucosal involvement with blisters and erosions.
Epidermolysis bullosa acquisita develops in dominant. In children the rare autoimmune skin diseases pemphigus, pemphigoid and epidermolysis bullosa acquisita and their variants may occur. Clear diagnostic criteria can be lacking for definitive diagnosis in less than clearcut cases. Bullous pemphigoid, mucous membrane pemphigoid and epidermolysis bullosa acquisita are subepidermal autoimmune blistering diseases whose antigenic target is located at the basement membrane zone. Vant veen, md a utoimmune diseases probably have a multi tude of causes. Bullous pemphigoid, cicatricial pemphigoid, and chronic bullous disease of childhood. Pemphigoid diseases typically manifest with blistering of the skin and mucous membranes and are associated with autoantibodies that target structural proteins of the hemidesmosome. The most common treatment is prednisone, which comes in pill form. Bullous pemphigoid is an uncommon skin disease characterized by tense blisters on the surface of the skin. Bpautoabs may target regions of collagen xvii other than the nc16a domain.
Specific disorders are described within the pemphigoid spectrum, and it is important to distinguish the variants because medical implications and treatments differ for the various types. Nov 14, 2014 cicatricial pemphigoid is a rare, chronic, blistering and scarring disease that affects the oral and ocular mucosa. This means that in bullous pemphigoid and epidermolysis bullosa acquisita, labeling with sera from patients. Hence, novel treatment options are urgently needed for the care of eba. 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. May, 2019 the main disorders to date include bullous pemphigoid bp, pemphigoid gestationis, mucous membrane pemphigoid mmp, epidermolysis bullosa acquisita eba, linear iga disease labd, dermatitis herpetiformis dh, lichen planus pemphigoides and bullous lupus. Pemphigoid is more common than pemphigus, and is slightly more common in women than in men.
Nov 15, 2019 pemphigoid diseases are a group of autoimmune blistering skin diseases defined by an immune response against certain components of the dermalepidermal adhesion complex. Brunstingperry pemphigoid transitioning from previous. Bullous pemphigoid and epidermolysis bullosa acquisita. Dapsone in the management of the autoimmune bullous diseases. It is classified as a type ii hypersensitivity reaction, with the formation of antihemidesmosome antibodies. Oct 31, 2012 atenolol is commonly used by patients with hypertension, angina pectoris, or myocardial infarction. Current treatment options rely on nonspecific immunosuppression, which in many cases, does not lead to a remission of treatment. Bullous pemphigoid often goes away on its own in a few months, but may take as many as five years to resolve. In contrast to pv, bp may remit spontaneously and can often be treated with lower doses of immunosuppressives. Corticosteroid ointment can be rubbed on your affected skin and causes fewer side effects.
The common subtypes of pemphigoid diseases are bullous pemphigoid bp, mucous membrane pemphigoid mmp, and epidermolysis bullosa acquisita eba. Blistering disease, pemphigus, pemphigoid penn medicine. General anaesthesia of a bullous pemphigoid patient. Vulgaris, 2 pemphigus foliaceus, 3 bullous pemphigoid, 4 mucous membrane pemphigoid a.
Both can result in scarring of mucous membranes which may lead to blindness and life threatening respiratory complications. Seven cases of bullous dermatitis following accidental direct contact with an agricultural formulation of dazomet were described from france in 1993. Differentiation of bullous pemphigoid from epidermolysis. Mucous membrane pemphigoid and epidermolysis bullosa acquisita are rare acquired autoimmune blistering diseases of the skin. Despite growing insights into pemphigoid disease pathogenesis, corticosteroids are still a mainstay of treatment. Most of these antibodies belong to the immunoglobulin g class and bind principally to 2 hemidesmosomal proteins. Aug 10, 2016 bullous pemphigoid is a skin disorder characterized by large blisters. Bullous pemphigoid is the most commonly seen autoimmune blistering disease in the. Differentiation by fluorescence overlay antigen mapping.
Bullous pemphigoid has been reported in the eyes, nose, pharynx, larynx, vulva, urethra, and lung. Gi, is a heterogeneous group of mild to moderate skin blistering phenotypes caused by mutations in laminin. University department of dermatology, royal victoria infirmary, newcastle. We report the clinical presentation, immunopathologic features, disease course, and long. Pemphigus support pemphigoid support local support. They are prototypical, autoantibodydriven, organspecific diseases with the emergence of inflammatory skin lesions dependent on the recruitment of immune cells, particularly granulocytes, into the skin. Fitzpatrick tb, eisen az, wolff k, freedberg im, austen kf eds. 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. It might present with blisters or welts seen on arms, legs, groin, mouth or abdomen. Dyshidrosiform bullous pemphigoid is a rare variant of bullous. In pemphigoid diseases, mucocutaneous blistering is caused by autoantibodies to hemidesmosomal antigens. It is caused by circulating and tissuebound autoantibodies directed against bullous pemphigoid antigen 1 or bullous pemphigoid antigen 2 or both. Autoimmune bullous skin diseases, pemphigus and pemphigoid.
Bullous pemphigoid is a rare autoimmune skin disorder that usually, but not always, affects people who are 60 years old or older. Distinguishing epidermolysis bullosa acquisita from bullous. The condition is caused by antibodies and inflammation abnormally accumulating in a particular layer. Pemphigoid diseases such as epidermolysis bullosa acquisita eba may be difficult to treat. Pemphigoid diseases are characterized by tense blisters and erosions on the skin or mucous membranes due to autoantibodies against structural proteins of the hemidesmosomes 140 fig 1, a and c. Autoantibody profile differentiates between inflammatory and. Highyield biopsy technique for subepidermal blisters.
Epidermolysis bullosa acquisita eba is a rare autoimmune blistering disorder characterized by tense bullae, skin fragility, atrophic scarring, and milia formation. 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. Pemphigoid is a rare autoimmune disorder that results in skin blistering. Dpp4 inactivates incretins by catalyzing the cleavage of those proteins to inactive forms drucker, 2007. The main disorders to date include bullous pemphigoid bp, pemphigoid gestationis, mucous membrane pemphigoid mmp, epidermolysis bullosa acquisita eba, linear iga disease labd, dermatitis. Well go over the three types, what to look for, and the treatment options. Pemphigus, pemphigoid and epidermolysis bullosa acquisita. Patients with eba suffer from chronic inflammation as well as blistering and scarring of the skin and mucous membranes. Clinical features and diagnosis of epidermolysis bullosa. 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.
Distinguishing epidermolysis bullosa acquisita from. Mucous membrane pemphigoid genetic and rare diseases. 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. Dipeptidyl peptidase4 inhibitor dpp4i has been widely used to treat type 2 diabetes. Bullous pemphigoid is a skin disorder characterized by large blisters. Treatment of epidermolysis bullosa acquisita eba is difficult, and most treatment regimens are based on anecdotal reports. Bullous dermatitis an overview sciencedirect topics. There have been reports of various adverse effects associated with the use of atenolol including bullous pemphigoid. Assessment of diagnostic strategy for pemphigoid jama. Unfortunately, delay in diagnosis occurs frequently.
A case of oropharyngeal bullous pemphigoid presenting with. Col7 protein comprises a central triple helical domain flanked by a 145 kda noncollagenous aminoterminal domain nc1 and a 30 kda carboxylterminal. Occasionally, the inner lining tissue of the mouth, nasal passages, or conjunctivae of the eyes mucous membrane tissue can be involved. We report a deficiency of the 180kd bullous pemphigoid antigen in three patients with gabeb from unrelated families. Jci the pathophysiology of autoimmune blistering diseases. Instead, circulating autoantibodies directed to both collagen xvii, also named bp180, and bp230, are hallmarks of the autoimmune blistering disease bullous pemphigoid bp. But longterm use can increase your risk of weak bones, diabetes, high blood pressure, high cholesterol and infection. In these diseases, ultrastructural identification of the site of skinbound igg deposits at the epidermal. Pde4 inhibition as potential treatment of epidermolysis. Pemphigoid nodularis is a rare variant combining features of prurigo nodularis with those of bullous pemphigoid, often in the same lesion. To the best of our knowledge we present the first case report of atenololinduced mucous membrane pemphigoid.
The comparison and contrast of molecular mechanism of blister formation of these two diseases provide a rational diagnostic and therapeutic approach to affected patients. Interventions for mucous membrane pemphigoidcicatricial. Brunstingperry pemphigoid is a variant of cicatricial pemphigoid, bullous pemphigoid, or epidermolysis bullosa acquisita that is commonly characterized by the presence of atrophic scars, with little or no mucosal involvement, and affects the head, face, neck, and upper trunk. Other mucosal sites that might be affected include the nasopharnyx, larynx, genitalia, rectum, and esophagus. Junctional epidermolysis bullosa, generalized intermediate subtype jeb. 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. Druginduced cicatricial pemphigoid and acquired epidermolysis bullosa theodoor van joost, md albert j. Aug 10, 2018 bullous pemphigoid is a chronic, inflammatory, subepidermal, blistering disease. Mutations in the 180kd bullous pemphigoid antigen bpag2, a. Mucous membrane pemphigoid mmp and epidermolysis bullosa acquisita eba are acquired autoimmune bullous disorders of the skin. Cicatricial pemphigoid cp refers to a group of rare chronic autoimmune blistering diseases affecting mainly mucous membranes including the conjunctiva.
Mucous membrane pemphigoid in a patient with hypertension. In some people, the mouth or genitals are also affected. 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 bp is a major autoimmune blistering skin disorder, in which a majority of the autoantibodies autoabs target the juxtamembranous extracellular noncollagenous 16a domain nc16a domain of hemidesmosomal collagen xvii. The traditional approach for confirming the diagnosis of subepidermal blistering diseases such as bullous pemphigoid bp, epidermolysis bullosa acquisita eba, dermatitis herpetiformis dh, and linear iga bullous dermatosis labd requires 2 punch biopsies.
Bullous pemphigoid is well known for its cutaneous features. A 42yearold thai man presented to our faculty after developing. Dpp4i works by inhibiting the action of this enzyme and improves glycemic control aschner and kipnes, 2006. It may include corticosteroid medications, such as prednisone, and other drugs that suppress the immune system.
670 728 1438 12 148 566 783 868 1167 1460 1414 445 573 1010 265 349 913 1159 1074 1460 746 1536 743 1415 182 220 586 1106 1575 242 528 392 1202 1495 567 1241 1278 1405