Their roofs are relatively thick and so they tend to be tense and intact. Despite the high prevalence, the symptom is still underrated and there are only a few trials investigating the efficacy of drugs for disease. Bullous pemphigoid is an autoimmune subepidermal blistering disease. Linear iga bullous dermatosis labd, also known as linear iga disease, is a rare, idiopathic or druginduced autoimmune blistering disease characterized by the it seems to us that you have your javascript disabled on your browser. Although most general approaches to the treatment and diagnosis. Mucous membrane pemphigoid mmp, also known as cicatricial pemphigoid, refers to a group of chronic autoimmune diseases that predominantly affect the mucous membranes and, occasionally, skin. Linear iga bullous dermatosis labd is a rare autoimmune blistering disease with an incidence of 0. This is a rare autoimmune subepidermal bullous dermatosis that occurs during pregnancy and postpartum. Evaluation of clinical criteria for diagnosis of bullous pemphigoid. Autoimmune blistering diseases are characterized by the production of pathogenic autoantibodies. The second most common cause of chronic renal failure is glomerulonephritis, which is a collective term used for numerous diseases with the. Conventional beliefs and some publications, had in the past, asserted that systemic auto immune diseases such as inflammatory arthritis, connective tissue. Frontiers serological diagnosis of autoimmune bullous.
Staphylococcal scalded skin syndrome bullous impetigo bullous tinea, eczema herpeticum blistering distal dactylitis bullous scabies varicella virus, herpes simplex virus. As in dogs, pemphigus foliaceus is the most common feline autoimmune dermatosis. Vesicles and bullae are accumulations of fluid within or under the epidermis. Linear iga dermatosis introduction linear iga dermatosis or chronic bullous disease of childhood cbdc is generally a rare, nonhereditary, autoimmune disease. The three most significant autoimmune blistering diseases are bullous pemphigoid, pemphigus vulgaris, and dermatitis herpetiformis. Bullous systemic lupus erythematosus bsle is a rare subset of systemic lupus erythematosus sle associated with autoimmunity to type vii collagen. Dermatitis herpetiformis dh, bullous pemphigoid bp, and pemphigus vulgaris pv are autoimmune bullous skin conditions with eosinophilic and neutrophilic infiltrations. Autoimmune bullous diseases are rare disorders affecting skin and mucous membranes. See the separate pemphigoid gestationis article diagnosis is made on the basis of the presence of a subepidermal vesicle on routine histological examination and of linear deposition of complement along the basement membrane zone of perilesional skin. In most patients, the condition goes away after several years. 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. Epidermolysis bullosa acquisita eba is an autoimmune subepidermal bullous dermatosis that affects the skin and mu cous membranes, characterized by autoantibodies that are directed against type vii collagen colvii. Early and correct diagnosis and adequate treatment of autoimmune bullous diseases are important as they cause morbidity and mortality in the affected. 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.
Pruritus in autoimmune and inflammatory dermatoses frontiers. It can occur in younger adults, but bullous pemphigoid in infants and children is rare. Autoimmune blistering diseases are skin conditions characterized by the formation of blisters, which are the result of the destruction of cellular or extracellular adhesion molecules by antibodies. In the epidermis, neighboring keratinocytes adhere to each other through organelles known as desmosomes, whereas dermalepidermal junction adhesion is mediated by. The full text of this article is available in pdf format.
Pdf autoimmune bullous diseases in childhood freda. High doses of corticosteroids and immunosuppressive drugs are used as firstline therapy in autoimmune bullous dermatoses. Evaluation of clinical criteria for diagnosis of bullous. Evidence of molecular mimicry in chronic periodontitis. Depending on the respective target proteins of the autoimmune response and their location in the skin. Autoimmune bullous dermatoses are defined by autoantibodies directed against adhesion proteins in the epidermis or basement membrane zone, resulting in blister formation on the skin and mucosa. We report an exceptional association of vitiligo, autoimmune thrombocytopenia and autoimmune dermoepidermal bullous dermatosis. Autoimmune diseases j genet syndr gene ther keywords.
The diagnosis of autoimmune bullous diseases is based on clinical observation and on the presence of autoantibodies directed to molecules involved in the adhesion systems of the skin. Autoimmune blistering diseases knowledge for medical. Correct diagnosis depends on correlation of clinical, histologic, and immunofluorescence findings, with if studies remaining an essential, and routinely available, part of this diagnostic triad. Autoimmune bullous dermatoses aibd encompass a variety of organspecific autoimmune diseases that manifest with cutaneous andor mucosal blisters and erosions. It is classified as a type ii hypersensitivity reaction, with the formation of antihemidesmosome antibodies. The major function of this system is as a barrier against the external environment. Autoimmune bullous dermatoses bullous dermatoses are rare blistering diseases of the outer skin and nei ghbouring mucous membranes.
Item 116 dermatoses bulleuses autoimmunes emconsulte. Clinical manifestations of lad vary, from patients with vesicular lesions. S y n drom e n e t ic journal of genetic syndromes g t. The etiology remains unclear, and while there is an association with other autoimmune disorders in females, this is not the case in males. Erythema ab igne eai is a reticular erythematous hyperpigmentation of skin repeatedly exposed to moderate heat. Space heaterinduced bullous erythema ab igne mdedge. Moreover, a brief overview on the treatment of different forms of aiha is given. Autoimmune vesicular, bullous, and pustular dermatoses. Pemphigus encompasses a group of autoimmune blistering diseases of the skin and bullous dermatoses can be debilitating and possibly fatal. Bullous pemphigoid is an autoimmune disorder which occurs when the bodys immune system attacks and destroys healthy body tissue by mistake. Autoimmune hemolytic anemia, hemolysis, coldautoantibodies, warm. 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. Universite medicale virtuelle francophone dadhesion secondaire a.
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. Depending on the respective target proteins of the autoimmune response and their location in the skin, a distincti. Subepidermal blisters occur between the dermis and the epidermis. Bullous skin disease an overview sciencedirect topics. Le pemphigus est une maladie bulleuse autoimmune intra. Multiple autoimmune syndrome with vitiligo, autoimmune. Immunofluorescence assays are the currently accepted method for detection of autoantibodies. It is more prevalent in europe than in north america and is rarely seen in african or asian patients. It is characterized by tense blisters, usually on an erythematous base, usually in the perineum and perioral regions. Serological diagnosis of bullous autoimmune dermatoses. This companion article to canine pemphigus foliaceus, which appeared in our june 2012 issue, presents an overview of this disease in cats. Most of these diseases are associated with substantial morbidity, and a few may result in death.
Additional diagnostic methods include indirect immunofluorescence, enzymelinked. The primary lesions of pemphigus foliaceus are superficial pustules, which as a result of being thinroofed frequently rupture to form crusts with underlying erosions. Lichen sclerosus ls is a dermatosis that has a predilection for the anogenital skin and is significantly more common in females than in males. Consensus on the treatment of autoimmune bullous dermatoses. These diseases are mediated by pathogenic autoantibodies against keratinocyte adhesion molecules diaz and giudice, 2000. Bullous pemphigoid often presents in people over 80 years of age, and mostly affects people over 50. The accurate diagnosis of autoimmune bullous dermatoses is the cornerstone of good therapy for patients with this group of potentially serious diseases. Multiple autoimmune syndrome is defined by the coexistence of at least three different autoimmune diseases which can occur simultaneously or over several years of evolution. In 23% of patients with sle, cutaneous involvement is the initial manifestation. It is, though, the most common chronic bullous disease during the first decade of life. Bullous pemphigoid occurs equally in males and females. Diagnosis depends on lesional biopsy for histopathology and perilesional biopsy for direct immunofluorescence. While cytokines are crucial for the affinity and activation of different leukocyte cells in the inflammation and blister formation, there are no studies concerning a role of il36.
Bullous dermatoses are a variety of autoimmune skin diseases that are characterized by the presence of bullae or blisters. Autoimmune hepatitis aih, primaire biliaire cir rhose pbc, primaire scleroserende cholangitis. Mhc variants involved in autoimmune and infectious diseases. Bullous pemphigoid bp is the most common autoimmune bullous disorder which is characterized by autoantibodies against hemidesmosomal proteins of the skin and mucous membranes. Pruritus in autoimmune and inflammatory dermatoses is a common symptom that can be severe and affect the quality of life of patients. If you continue browsing the site, you agree to the use of cookies on this website. In some diseases, pruritus is related to disorders activity and severity or may occur independent of the disease. Autoimmune hemolytic anemia aiha is a relatively uncommon disorder caused by autoantibodies directed against self red blood cells, with an estimated. Advances in the diagnosis of autoimmune bullous dermatoses.
Pathophysiologically, bullous autoimmune dermatoses are caused by autoantibodies directed against adhesion molecules or structural proteins of the skin and mucous membranes, clinically resulting in blister formation. Vancomycin mediates iga autoreactivity in druginduced. Serological diagnosis of bullous autoimmune dermatoses envoplakin bp230 bp180 basal keratinocytes desmoglein 1 desmoglein 3 lamina lucida lamina densa sublamina densa antidesmosomes e. These are auto immune diseases in w hich the immune system produces an tibodies against structural components of the desmosomes or hemidesmosomes.
Maladies bulleuses autoimmunes dermatose a iga lineaire. Autoimmune bullous skin diseasespemphigus and pemphigoid. Its onset is usually in the 4th to 6th decade of life. Bullous pemphigoid is an uncommon skin disease characterized by tense blisters on the surface of the skin. Linear iga bullous dermatosis treated with rituximab. Bullous pemphigoid genetic and rare diseases information. Autoimmune bullous diseases in childhood 119 dermatitis herpetiformis dh is a chronic autoimmune blistering disorder associ ated with celiac disease cd. Introduction chronic bullous dermatosis of childhood, or linear immunoglobulin a bullous dermatosis lad of childhood, is a rare, autoimmune subepidermal bullous disease. The most commonly reported chronic skin disorders exacerbated by menses were psoriasis, behcets disease, and eczematous dermatoses. Bullous autoimmune dermatoses wiley online library. The mhc locus and genetic susceptibility to autoimmune and. While mucosal lesions occur in less than 30 % of bullous pemphigoid patients, the second most common bullous autoimmune dermatosis, pemphigus vulgaris, typically presents with oral erosions as the predominant and frequently initial symptom. Occasionally, the inner lining tissue of the mouth, nasal passages, or conjunctivae of the eyes mucous membrane tissue can be involved. Linear iga bullous dermatosis lad is an autoimmune blistering disease characterized by subepidermal blisters with linear deposits of iga along the basement membrane zone bmz on direct immuno.
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