This article outlines the clinical and pathological features of blistering skin conditions with a particular focus on bullous impetigo, dermatitis herpetiformis, bullous pemphigoid and porphyria cutanea tarda. Bullous pemphigoid antigen 2 180 kd, type xvii collagen and alpha 6 beta 4 integrin are transmembrane components. The objectives of this study are to define clinical and diagnostic characteristics of infantile bp and develop a treatment algorithm. Diagnosis of autoimmune bullous diseases beek 2018. Conditions of the human integumentary system constitute a broad spectrum of. Bullous pemphigoid usually occurs in older persons and is rare in young people. Infections, contact reactions and drug eruptions should always be considered. Andrews diseases of the skin, clinical dermatology, 10th edition. Ppt bullous skin diseases powerpoint presentation free. When the blisters rupture, they leave a sore that typically heals without scarring. Patients typically have skin lesions, some also have mucous membrane. In some of these conditions, eg the immunobullous disorders, the blisters are the primary feature, in others, eg leg oedema, the blisters are secondary to an underlying condition.
They develop on areas of skin that often flex such as the lower abdomen, upper thighs or armpits. Bullous pemphigoid is a chronic autoimmune skin disorder resulting in generalized, pruritic, bullous lesions in elderly patients. Some are of short duration and are quite characteristic, such as those in poison ivy and herpes zoster. Bullous pemphigoid inflammatory diseases online course. Kochs postulates, adapted for autoimmune diseases, were applied on these skin diseases. Early and correct diagnosis and adequate treatment of autoimmune bullous diseases are important as they cause morbidity and mortality in the affected. It is considered the first scientific work dedicated to dermatology. Diaz, in the autoimmune diseases sixth edition, 2020. Winner of the standing ovation award for best powerpoint templates from presentations magazine. Other bullous diseases dermatitis herpetiformis view in chinese epidermolysis bullosa acquisita view in chinese friction blisters linear iga bullous dermatosis view in chinese pemphigoid clinical features and diagnosis of bullous pemphigoid and mucous membrane pemphigoid view in chinese. Autoimmune bullous diseases are rare disorders affecting skin and mucous membranes. Bullous pemphigoid is an autoimmune disorder that occurs when the immune system attacks the skin and causes blistering.
Ppt bullous skin disorders bsd powerpoint presentation. Vesicles and bullae are accumulations of fluid within or under the epidermis. The incidence of bullous sle was estimated to be 0. Plectin in epidermolysis bullosa and autoimmune, bullous diseases. The blisters are usually located on the arms, legs, or middle of the body. Occasionally, the inner lining tissue of the mouth, nasal passages, or conjunctivae of the eyes mucous membrane tissue can be involved.
Bullous pemphigoid bp in infants is a rare but increasingly reported autoimmune blistering skin disease. Diagnosis of autoimmune bullous diseases beek 2018 jddg. May 15, 2017 bullous pemphigoid antigen 1 230 kd and plectin are intracellular components. Chart and diagram slides for powerpoint beautifully designed chart and diagram s for powerpoint with visually stunning graphics and animation effects. Bullous pemphigoid is the most frequent autoimmune bullous disease and mainly affects elderly individuals. The skin can develop vesicles and bullae as a result of diverse causes through. Bullous and vesicular disorders an overview primary.
These signs serve to demonstrate that in some diseases e. Existing clinical and experimental evidence generally supports a pathogenic role of autoantibodies for blister formation. Satellite modules are prepared to strengthen the professional training of each category. Autoimmune bullous skin diseases, pemphigus and pemphigoid. Bullous disease definition of bullous disease by medical. Amongst all autoimmune blistering diseases, bullous pemphigoid is the most common one. This chapter provides an overview of vesicular and bullous disorders, with an aim to help in the diagnosis.
Our new crystalgraphics chart and diagram slides for powerpoint is a collection of over impressively designed datadriven chart and editable diagram s guaranteed to impress any audience. Anatomical level of splitblister separation plane 2. Bullous skin diseases free download as powerpoint presentation. The prototypic bullous skin diseases, pemphigus vulgaris, pemphigus foliaceus, and bullous pemphigoid, are characterized by the blister formation in the skin andor oral mucosa in combination with circulating and deposited autoantibodies reactive with hemidesmosomes. Bullous pemphigoid dermatologic disorders merck manuals. It is the most common type of the pemphigoid group, representing 80% of subepidermal immunobullous cases. People with bullous pemphigoid may develop multiple blisters. Psychiatric and neurological disorders are associated with. In a large cohort of sera taken from patients with immunobullous disorders, 12% were identified as bullous sle 1,2.
There are many dermatological conditions that can lead to the development of blisters. Bullous pemphigoid antigen 1 230 kd and plectin are intracellular components. Autoimmune bullous diseases aibds are a heterogeneous group of disorders defined by autoantibodies against adhesion molecules of the skin. Bullous skin diseases cutaneous conditions dermatology. The bullous pemphigoid disease area index bpdai has separate scores for skin and mucous membrane activity. The polyclonal antibody response against structural skin proteins in autoimmune bullous diseases is heterogeneous, but shows a skewing in subclass distribution of autoantibodies. A few types of bullous disease increase the risk of certain cancers if left untreated. Introduction to bullous diseases dermatologic disorders. Pemphigoid is a group of rare autoimmune blistering skin diseases. Aug 20, 2019 autoimmune bullous dermatoses aibd encompass a variety of organspecific autoimmune diseases that manifest with cutaneous andor mucosal blisters and erosions. Their roofs are relatively thick and so they tend to be tense and intact. The skin shields the interior of a person from the external. Diagnosis is by skin biopsy and immunofluorescence testing of skin and serum.
Other bullous conditions include staphylococcal scalded skin syndrome, toxic epidermal necrolysis, severe. As a matter of fact, various chronic neurological disorders have been reported in association with bp, eg, multiple sclerosis, 111 posttraumatic jeanclaude roujeau, md, personal communication, 1991 or ischemic paresia, 18,19 and shydrager syndrome, 20 suggesting that these diseases share a feature able to induce the bullous disease. A skin condition, also known as cutaneous condition, is any medical condition that affects the integumentary systemthe organ system that encloses the body and includes skin, hair, nails, and related muscle and glands. Pdf the prototypic bullous skin diseases, pemphigus vulgaris, pemphigus foliaceus, and bullous pemphigoid, are characterized by the blister. Light microscopy will provide information on whether or not there is an inflammatory infiltrate and on the nature of the inflammatory infiltrate. Other bullous conditions include staphylococcal scalded skin syndrome, toxic epidermal necrolysis, severe cellulitis, and certain drug eruptions. Intercellular deposition of igg is observed in the epidermis. Autoimmune bullous diseases are a group of rare skin diseases characterized by intraepidermal and subepidermal bullae formations due to autoantibodies directed against the structural proteins of the epidermis or the dermalepidermal junction. In the epidermis, neighboring keratinocytes adhere to each. Bullous pemphigoid genetic and rare diseases information. These diseases are mediated by pathogenic autoantibodies directed against keratinocyte adhesion molecules diaz and giudice, 2000. The mainstays of treatment are dapsone and a life long gluten free diet.
Diagnosis of bullous diseases the diagnosis of bullous disorders is made by thorough clinical evaluation and is confirmed by histopathologic and immunofluorescent examination. Broadly defined, the pemphigus group includes those diseases in. In pemphigoid diseases, autoantibodies reveal linear staining at the dermal. Spectrum of subepidermal immunobullous disorders seen at the national skin centre, singapore. In this article, we will study the epidemiology, pathophysiology, diagnosis, differential diagnosis, therapy and prognosis of bullous pemphigoid. Vesicles and bullae are the primary lesions in many diseases. Early and correct diagnosis and adequate treatment of autoimmune bullous diseases are important as they cause morbidity and. Bullous pemphigoid is an uncommon skin disease characterized by tense blisters on the surface of the skin. 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. They develop on areas of skin that often flex such as the lower abdomen, upper thighs or. Common skin diseases download book free computer books. The bullous skin diseases, including pemphigus and bullous pemphigoid, affect the skin andor oral mucosa.
The strong bias toward production of igg4 autoantibodies in these organspecific autoimmune diseases suggests chronic antigenic stimulation. The ageadjusted incidence of bp has been reported to be on the rise 1,2,3. Pemphigus is a group of autoimmune blistering diseases of the skin and mucous membranes. Evaluation of vesicularbullous rash differential diagnosis. The relevance of the igg subclass of autoantibodies for. Bullous pemphigoid and epidermolysis bullosa acquisita. Pemphigus is a group of autoimmune blistering diseases of the skin. Current guidelines do not address specific aspects of the infantile form of bp.
Immunosuppressive therapy is effective, and anticd20 exerts a dramatic effect, especially on pemphigus disease. They are characterized by autoantibodies targeting structural proteins of the skin, which are responsible for the intercellular contact between epidermal keratinocytes and for adhesion of. Subepidermal blisters occur between the dermis and the epidermis. Although epidermolysis bullosa acquisita is thought to be an autoimmune disease, epidermolysis bullosa is a group of inherited diseases in which epithelial adhesion protein defects lead to epithelial fragility and bullae formation. The blisters may break open and form ulcers or open sores.
Increase in incidence rates in the past decades has been attributed to. Bullous pemphigoid diagnosed according to the following criteria. Feb 03, 2007 autoimmune bullous skin diseases are characterized by autoantibodies and t cells specific to structural proteins maintaining cellcell and cellmatrix adhesion in the skin. Although written primarily for dermatologists, the book can be useful for any primary care provider, medical student, or resident interested in skin disorders. Pdf autoimmune skin blistering diseases aibd are characterized by autoantibodies that are directed against structural. The prototypic bullous skin diseases, pemphigus vulgaris, pemphigus foliaceus, and bullous pemphigoid, are characterized by the blister formation in the skin andor oral mucosa in combination with. The condition is caused by antibodies and inflammation abnormally accumulating in a particular layer. 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. In some people, the mouth or genitals are also affected. Topical and systemic corticosteroids are used initially. The most important of the acquired bullous skin diseases are the pemphigus group, the pemphigoid group, acquired bullous epidermolysis, and dermatitis herpetiformis.
Uptodate, electronic clinical resource tool for physicians and patients that provides information on adult primary care and internal medicine, allergy and immunology, cardiovascular medicine, emergency medicine, endocrinology and diabetes, family medicine, gastroenterology and hepatology, hematology, infectious diseases, nephrology and. Bullous pemphigoid skin disorders msd manual consumer version. Topical steroids alone or associated with methotrexate in. Autoantibody reactivity is usually poorly characterized. Dec 10, 2014 bullous pemphigoid bp in infants is a rare but increasingly reported autoimmune blistering skin disease. Autoimmune bullous dermatoses aibd encompass a variety of organspecific autoimmune diseases that manifest with cutaneous andor mucosal blisters and erosions. Autoimmune bullous diseases approach and management.
Bullous pemphigoid symptoms and causes mayo clinic. Bullius pemphigoid is an autoimmune skin disease, which means that the body, following a. Autoimmune bullous skin diseases are a group of disorders in which an immune response is directed against skin adhesion molecules leading to blister formation. Bullous dermatoses are rare blistering diseases of the outer skin. Incidence and distribution of subepidermal autoimmune bullous skin diseases in three french regions. Diseases of the skin include skin infections and skin neoplasms including skin cancer. Since the skin is a vital organ in the protection of the body against dehydration and infections, these skin diseases may be life threatening. This book consists of a core module and five satellite modules. Diagnosis of bullous diseases dermatology education. The pathological roles of autoantibodies have been investigated in vivo and in vitro.
Circulating antibodies are produced against the proteins present in the dermoepidermal junction dej. The major function of this system is as a barrier against the external environment. They are characterized by autoantibodies targeting structural proteins of the skin, which are responsible for the intercellular contact between epidermal keratinocytes and for adhesion of the basal keratinocytes to the dermis. The core module is prepared for health officers, pubic health nurses, environmental health, medical laboratory technology students and health extension workers. Doctors can diagnose bullous pemphigoid by examining skin samples under a microscope and checking for certain antibody deposits. To further discriminate pemphigoid diseases, 1 m nacl. Ppt bullous skin diseases powerpoint presentation free to. People develop large, itchy blisters with areas of inflamed skin. Aug 10, 2016 bullous pemphigoid is a skin disorder characterized by large blisters.
Theyll give your presentations a professional, memorable appearance the kind of sophisticated look that. In these diseases, ultrastructural identification of the. Bullous pemphigoid is a rare and chronic autoimmune disorder characterised by subepidermal blisters that predominantly involves the skin and less commonly the mucous membrane. Increase in incidence rates in the past decades has been attributed to population aging. Blister fluid filled cavity within or beneath the epidermis vesicles 0. Bullous pemphigoid and amyotrophic lateral sclerosis. Bullous pemphigoid bp is an autoimmune skin disease with incidence varying between 0. Bullous pemphigoid is a skin disorder characterized by large blisters. Pemphigus and pemphigoid diseases are chronic autoantibodymediated autoimmune bullous diseases of the skin. Bullous pemphigoid bulus pemfihgoid is a rare skin condition that causes large, fluidfilled blisters. Worlds best powerpoint templates crystalgraphics offers more powerpoint templates than anyone else in the world, with over 4 million to choose from. Scribd is the worlds largest social reading and publishing site. Bullous pemphigoid skin disorders msd manual consumer.
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