Linear iga bullous dermatosis labd is a rare autoimmune subepithelial vesiculobullous disease due to iga autoantibodies directed against different antigens of the basement membrane zone bmz of the skin andor mucosae. Linear iga disease is a very rare blistering condition of the skin in adults. Linear iga bullous dermatosis labd is classically described as an acquired autoimmune blistering eruption that may be idiopathic, related to systemic disease, or associated with exposure to various medications. S y n drom e n e t ic journal of genetic syndromes g t. Institute of dental sciences, mangalore 575004, india. Pubmed is a searchable database of medical literature and lists journal articles that discuss linear iga disease. This leaflet has been written to help you understand more about linear iga disease. Mapping of epitopes on the bp180 ectodomain targeted by iga and igg autoantibodies in patients with the lamina lucidatype of linear iga disease. The disease onset in childhood has been termed chronic bullous dermatosis of childhood, which is now considered. Certain drugs, autoimmune diseases, infection, chronic renal insufficiency, and malignancies have been implicated as the etiologic factors of this.
Linear iga bullous dermatosis labd, also known as linear iga disease, is a rare, idiopathic or druginduced autoimmune blistering disease characterized by the linear deposition of iga at the dermoepidermal junction. The following list of medications are in some way related to, or used in the treatment of this condition. Bowen described in 1901 the first six cases of linear iga bullous disease of childhood, by that time considered as dermatitis. Linear iga disease lad is an autoimmune subepidermal lying beneath or constituting the innermost part of the epidermis disease that may be idiopathic or druginduced.
Children and adults are affected, with disease of the former historically referred to as chronic bullous dermatosis of childhood. Nair2, rajesh shanker kashyap3 1department of oral medicine and radiology, a. Lad is an autoimmune disease histopathologically characterised by the linear deposition of iga at the bmz. Two main clinical syndromes are distinguished, chronic bullous disease of childhood, beginning in childhood, and adult. A similar condition affecting children is known as chronic bullous disease of childhood cbdc. Linear iga bullous dermatosis is a rare immunemediated blistering skin disease frequently associated with medication exposure, especially vancomycin, with men and women being equally affected. A 43yearold man with linear immunoglobulin a iga dermatosis associated with gluten intolerance presented with progressive vision loss, pain and photosensitivity in both eyes. Linear immunoglobulin a iga disease, also known as linear iga bullous dermatosis lad, is a rare acquired subepidermal blistering disease characterized by continuous linear iga deposits in the basement membrane zone bmz when visualized on. Two main clinical syndromes are distinguished, chronic bullous disease of childhood, beginning in. Lad can be subdivided into adultonset, childhoodonset, and druginduced lad zone et al. Clinically, labd has a variable presentation characterized by tense vesiculobullous lesions on the trunk and extremities that often appear in a herpetiform arrangement or at the center of annular erythematous plaques. The autoimmune registry supports research for linear iga disease by collecting information about patients with this and other autoimmune diseases. One function of the bmz is to maintain the contiguity of the dermalepidermal junction.
Linear iga bullous dermatosis of childhood, also known as chronic bullous dermatosis of childhood, is a rare, acquired, selflimited autoimmune subepidermal bullous disease. Linear iga dermatosis or chronic bullous disease of childhood cbdc is generally a rare, nonhereditary, autoimmune disease. Linear iga disease genetic and rare diseases information. Linear immunoglobulin a iga bullous dermatosis labd, also known in the literature as linear iga dermatosis, linear iga disease, iga pemphigoid, and linear.
Two main clinical syndromes are distinguished, chronic bullous disease of childhood, beginning in childhood, and adult linear iga disease. Pdf patients with bullous pemphigoid and linear iga disease. Linear immunoglobulin a iga bullous dermatosis labd presents as a rare autoimmune disease that can either occur spontaneously or induced by certain drugs, the most common of which is vancomycin. Ocular involvement and blindness secondary to linear iga. Accordingly, many cases are indistinguishable on light microscopy from dermatitis herpetiformis, with the presence of dermal papillary microabscesses. What is linear iga bullous disease linear iga bullous disease is an autoimmune blistering disease in which blisters form in the skin and mucous membranes. Lad is one of the rarer blistering diseases, with an incidence of only 0. Bouldin mb, clowerswebb he, davis jl, mcevoy mt, davis md. Linear iga disease primary care dermatology society uk. Linear iga disease is a rare blistering disease affecting the skin and less often the oral mucosa. It is rare with an incidence in western europe of 0.
Background linear iga bullous dermatosis is an autoimmune blistering disease characterized clinically by the presence of small tense blisters and immunologically by the presence of iga at the dermalepidermal junction. Linear iga disease lad is a rare chronic autoimmune subepithelial mucocutaneous bullous disorder characterized by linear deposition of iga immunoreactants at the cutaneous or mucosal basement membrane zone bmz. Linear iga bullous dermatosis labd, also known as linear iga dermatosis, is a rare immunemediated disease that results in blistering. Linear iga bullous dermatosis treated with rituximab jaad. You can join the registry to share your information with researchers and receive updates about participating in new research studies.
Georgi m, scheckenbach c, kromminga a, partscht k, messer g, brocker eb, et al. Linear iga disease lad is a subepidermal blistering disorder characterized by pruritic lesions and linear deposition of iga autoantibodies at the bmz nemzer et al. The disease has bimodal age predilection and occurs in children up to the age of 10 years and in adults usually after the age. The aetiology is unknown but there is a recognized association with lymphoproliferative malignancies. A morbilliform variant of vancomycininduced linear iga. Feb 26, 2019 linear iga bullous dermatosis labd, also known as linear iga dermatosis, is a rare immunemediated disease that results in blistering of the skin, in the subepidermal layers. Summary linear iga disease lad is a well recognized subepidermal blistering disorder characterized by linear deposits of iga at the basement membrane zone. Labd is a subepidermal blistering disease that is diagnosed by detecting linear iga bands along the basement membrane. The bodys immune system produces antibodies known as immunoglobulin a iga which are incorrectly deposited in the top layer of the skin in a line hence the term linear. Linear iga bullous dermatosis labd is a rare, blistering autoimmune disease characterized by linear deposits of iga at the basement membrane zone bmz, with the possible presence of circulating. The condition is called linear iga disease because a type of protein called. It is, though, the most common chronic bullous disease during the first decade of life. Linear iga disease is a chronic, acquired, immunobullous condition of children and adults, with cutaneous and mucosal involvement, characterised by iga basementmembrane antibodies. It is a rare immunobullous disorder with an incidence in western europe of 0.
We present a case of a 59yearold man with worsening blistering skin rash who. Bullous pemphigoid bp and linear iga disease lad are autoimmune subepidermal blistering skin diseases associated with autoantibodies against the transmembrane hemidesmosomal protein bp180type xvii collagen. Linear iga bullous dermatosis successfully treated with. Mar 06, 2020 linear immunoglobulin a iga dermatosis lad is an autoimmune subepidermal vesiculobullous disease that may be idiopathic or druginduced. Linear immunoglobulin a iga bullous dermatosis, also known as linear iga disease, is a rare disorder with an incidence of about 0. Linear iga disease is a very rare blistering condition of the skin in.
Adult linear iga disease is an acquired, autoimmune blistering. Described for the first time in the 1970s as a different entity from dermatitis herpetiformis, linear iga dermatosis is a rare autoimmune bullous disease characterized. The condition is called linear iga disease because a type of protein called immunoglobulin a iga can be seen deposited under the outer layer of the skin the epidermis in a line linear when a small sample of skin biopsy is prepared specially and examined under the microscope. Skin biopsy is usually essential to differentiate it from other blistering disorders, and the diagnosis of linear iga disease is usually established by immunofluorescence. Linear iga bullous dermatosis clinics in dermatology. Pdf patients with bullous pemphigoid and linear iga. Click on the british association of dermatologists link to view this leaflet. The age of peak is a childhood variant of labd termed chronic. The following information is not intended to endorse any particular medication. Adult linear iga disease and chronic bullous disease of childhood. Linear iga bullous dermatosis in adults and children. The british association of dermatologists provides an informational leaflet on linear iga disease. Cureus a case of druginduced linear iga bullous dermatosis.
It is also called linear iga disease, and in children, chronic bullous. Clinically, labd has a variable presentation characterized by tense vesiculobullous lesions on the trunk and extremities that often appear in a herpetiform arrangement or at the center of annular. Chronic bullous dermatosis of childhood, linear immunoglobulin a dermatosis. Its name comes from the characteristic findings on direct immunofluorescence of a skin biopsy, in which a line of iga antibodies can be found just below the epidermis. Linear iga bullous dermatosis successfully treated with omalizumab. Pemphigus vulgaris usually starts in the oral mucosa followed by blistering of the skin, which is often painful. It affects mainly preschoolaged children and adults, with only few studies on large series. Linear iga disease an overview sciencedirect topics. Linear iga disease has been reported in association with inflammatory bowel disease, in particular ulcerative colitis. Linear iga bullous dermatosis an overview sciencedirect. Initially, broad serum testing, including pemphigoid and pemphigus panels and endomysial antibodies, should be performed unless a specific immunobullous skin disease type is suspected. We experienced a case of linear iga disease that occurred simultaneously with colonic crohns disease in a 55yearold woman and rather unusually both skin and bowel disease improved in tandem.
The disease mainly involves the skin and mucosa, leading to blister formation. This disease has a varied clinical presentation especially when the disease affects the oral mucosa. Idiopathic, systemic diseaserelated, and drugrelated versions of this disorder have been described, with the latter most commonly. Linear iga bullous disease is an autoimmune blistering disease in which blisters form in the skin and mucous membranes. The exact cause of linear iga disease is unknown however it is considered to be an autoimmune disease. The usual clinical presentation is tense vesicles and bullae, erythematous patches, and occasional erosions. Linear iga disease and ulcerative colitis paige 1997. The condition has a bimodal age predilection and occurs in children between 6 months and 10 years of age, rarely persisting after puberty.
Collier pm, wojnarowska f, welsh k, mcguire w, black mm. Treatment of linear iga bullous dermatosis of childhood with. Linear iga bullous dermatosis treated with rituximab. Linear iga bullous dermatosis labd is a rare autoimmune bullous disease characterized by linear deposition of iga along the cutaneous basement membrane. Linear iga dermatosis and chronic bullous dermatosis of. Linear iga disease in a patient with castlemans disease. It affects young children usually before 5 years of age or adults usually after 40 years of age. Iga linear bullous dermatosis, also known as chronic bullous dermatosis of childhood, is an autoimmune disease which may be idiopathic or.
It was first described by tadeusz chorzelski in 1979 and may be divided into two types. His visual acuity was light perception lp in both eyes. Wed like to understand how you use our websites in order to improve them. Linear iga bullous dermatosis labd is a mucocutaneous autoimmune disease that may be druginduced or idiopathic. The doctors provides information on linear iga disease. Linear iga disease what are the aims of this leaflet. A physical examination revealed bullous, papular lesions with erythematous borders in periocular tissues, limbs, and thorax. Linear iga bullous dermatosis labd is a rare, blistering autoimmune disease characterized by linear deposits of iga at the basement membrane zone.
Immunemediated subepidermal blistering disease in both adults and children idiopathic, autoimmune disorders, malignancy medications vancomycin, amoxicillinclavulanate, tmpsmx linear iga deposits in two distinct patterns. Pdf linear iga disease presenting as prurigo nodularis. Linear iga disease is a blistering disorder of the skin and mucous membranes that is also known as linear iga bullous dermatosis in adults and chronic bullous disease of childhood in children. The aim of this study was to assess possible differences between adults and.
Click on the link to view a sample search on this topic. Pdf on aug 1, 1989, s e kelly and others published linear iga disease in association with hydatidiform mole find, read and cite all the research you need on researchgate. Linear iga bullous dermatosis labd is a rare autoimmune blistering disease with an incidence of 0. What you should be alert for in the history patients with pruritic itchy blisters should alert the physician. Linear iga bullous dermatosis is a subepidermal blistering disease in which neutrophils are usually the predominant cell in the infiltrate fig. It is characterized by linear deposition of iga and disruption of the dermoepidermal junction, resulting in blisters with a tense clinical appearance. We report a case of lad occurring in a patient with multicentric castlemans disease angiofollicular lymph. Linear iga disease lad is a group of heterogeneous autoimmune subepidermal bullous diseases characterized by exclusively iga autoantibodies targeting a component of the epidermal basement membrane zone. Classic iga antibodies to 97kda andor 120 kd fragment of bp180 split in the lamina lucida. The disease onset can be in adulthood or in childhood, even in the neonate. Linear immunoglobulin a iga dermatosis lad is an autoimmune subepidermal vesiculobullous disease that may be idiopathic or druginduced.
Seventy adult patients with linear iga disease were studied. Pdf linear iga disease in association with hydatidiform mole. Pdf chronic bullous disease or linear iga dermatosis of. Linear iga disease is a chronic, acquired, subepidermal condition of children and adults, with cutaneous and mucosal involvement, characterised by iga basementmembrane antibodies. Pdf linear iga dermatosis or chronic bullous disease of childhood cbdc is a nonhereditary, autoimmune subepidermal bullous disease, characterized by. Linear iga disease is a rare skin blistering condition. Linear iga disease in association with hydatidiform mole article pdf available in journal of the royal society of medicine 827. Linear iga disease is a rare, idiopathic or druginduced autoimmune blistering disease characterized by the linear deposition of iga at the dermoepidermal junction. The disease has bimodal age predilection and occurs in children up to the age of 10 years and in adults usually. Linear iga disease is a rare but very severe side effect of vancomycin. It tells you what it is, what causes it, what can be done about it, and where you can find out more about it. Skin manifestations include large tense bullae as seen in bullous pemphigoid andor vesiclular lesions characteristic of dermatitis herpetiformis. Treatment of linear iga bullous dermatosis of childhood. Jul 02, 20 linear iga bullous dermatosis labd is a mucocutaneous autoimmune disease that may be druginduced or idiopathic.
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