Pdf linear iga disease lad is an acquired subepidermal bullous disorder. Linear iga bullous dermatosis in a child successfully responding to oral antibiotics published. The autoimmune registry supports research for linear iga disease by collecting information about patients with this and other autoimmune diseases. 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, subepithelial blistering condition that predominantly affects the skin and mucosal surfaces. Linear iga disease is a rare disease characterized by linear deposition of solely iga along the ebmz. Linear iga disease is a subepidermal blistering disorder of unknown cause. Linear iga bullous dermatosis protracted by vancomycinloaded. The disease mainly involves the skin and mucosa, leading to blister formation.
We report a similar case to further highlight this entity, as we suspect it is underreported and likely to be an increasing issue given our aging population. Successful treatment of oral linear iga disease using mycophenolate. Two main clinical syndromes are distinguished, chronic bullous disease of childhood, beginning in childhood, and adult linear iga disease. Vancomycin mediates iga autoreactivity in druginduced. A case of linear iga disease limited to the oral mucosa. Their beneical effects in labd may be due to their antiinlammatory. It is, though, the most common chronic bullous disease during the first decade of life. Oral mucosal involvement as the sole or main manifestation of linear. The usual clinical presentation is tense vesicles and bullae, erythematous patches, and occasional erosions. Linear iga disease is a rare, idiopathic or druginduced autoimmune blistering disease characterized by the linear deposition of iga at the dermoepidermal junction.
A rare case of vancomycininduced linear immunoglobulin a. It commonly is drug induced, often in association with systemic vancomycin. Here we discuss a case of a vesiculobullous lesion with severe oral and ocular mucosal involvement mimicking pemphigoid with histopathological evidence of subepithelial blisters. Unusual clinicopathological and immunological presentation. Desquamative gingivitis, linear iga disease, oral cavity. Jul 16, 2019 the oral mucosa may show erosions, bullae, gingivitis, loss of teeth, and reduced ability to open the mouth. This disease has a varied clinical presentation especially when. Linear immunoglobulin a iga dermatosis lad is a rare autoimmune disorder that presents as a vesiculobullous lesion with cutaneous manifestations, but rare oral mucosal involvement. Along with dermatologic blistering, other manifestations include oral ulceration and desquamative gingivitis. A case of linear iga disease in which oral mucosal involvement remained the sole manifestation of the disease for twentythree years is presented. Interestingly, head, notably perioral, involvement and string of pearls arrangement occurred more frequently in the paediatric than adult group. Jul 01, 2011 linear deposition of iga along the basement membrane zone of epidermis iga band is linear, at basement membrane, compared to dermatitis herpetiformis, which has granular band at dermal papillae igg in 25% of cases br j dermatol 1997.
Cicatricial pemphigoid is a rare, blistering disease of the skin, characterized by severe, erosive lesions of the skin and mucous mem branes. Linear iga disease presenting as desquamative gingivitis. The typical manifestation is a continuous linear iga deposition along the basement membrane. Children and adults are affected, with disease of the former historically referred to as chronic bullous dermatosis of childhood. The disease has bimodal age predilection and occurs in children up to the age of 10 years and in adults usually after the age. Blistering mucocutaneous diseases of the oral mucosa a. A provisional diagnosis of oral lichen planus olp was made. Antibody deposition leads to complement activation and neutrophil chemotaxis, which eventuates in loss of adhesion at the dermalepidermal junction and in blister formation. A morbilliform variant of vancomycininduced linear iga. Mapping of epitopes on the bp180 ectodomain targeted by iga and igg autoantibodies in patients with the lamina lucidatype of linear iga disease.
Topical corticosteroids are the first line of treatment although the choice remains challenging as none of the available treatments is supported by strong clinical evidence. In this case, we describe an unusual presentation of. We present a case of linear iga disease that manifested simply as oropharyngeal ulceration and desquamative gingivitis, resulting in considerable delay in. Linear iga bullous dermatosis labd is an autoimmune blistering rash caused by iga autoantibodies against the epidermal basement membrane zone. It is also called linear iga disease, and in children, chronic bullous. 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. Certain drugs, autoimmune diseases, infection, chronic renal insufficiency, and malignancies.
Vancomycininduced linear immunoglobulin a bullous dermatosis. Linear iga disease is a rare but very severe side effect of vancomycin. Pemphigus vulgaris usually starts in the oral mucosa followed by blistering of the skin, which is often painful. Linear immunoglobulin a iga dermatosis lad is a rare chronic autoimmune disorder, which presents with vesicles and bullae formation affecting both the skin and the mucous membrane, with a characteristic immunofluorescence finding of homogeneous linear deposits of iga in the cutaneous basement membrane zone. One function of the bmz is to maintain the contiguity of the dermalepidermal junction. Linear iga disease limited to the oral mucosa journal of.
Oral findings, for both patients, were of widespread painful ulceration present for two to three months and. Linear iga disease lad is a rare acquired autoimmune bullous disorder, characterized by linear deposition of iga along the dermoepidermal basement membrane zone. Mar 06, 2020 linear iga dermatosis is an autoimmune disease histopathologically characterized by the linear deposition of iga at the basement membrane zone bmz. Pdf linear iga dermatosis adult variant with oral manifestation. Oral lesions heal with scar formation, and are often therapy resistant 29, 39. Linear iga disease is a rare blistering disease affecting the skin and less often the oral mucosa. Chronic bullous dermatosis of childhood is equivalent to linear iga disease of adulthood and is characterized by an abrupt onset of large, widespread and tense bullae on a normal or erythematous base. Linear iga bullous dermatosis in adults and children. Druginduced linear iga bullous dermatosis in a patient. Topical steroids alone or associated with methotrexate in. Linear iga disease limited to the oral mucosa request pdf. Although a number of case reports have been published describing instances in which oral mucosal involvement was the only or main manifestation of the disease, the fact that linear iga. The exact cause of linear iga disease is unknown however it is considered to be an autoimmune disease.
Two main clinical syndromes are distinguished, chronic bullous disease of childhood, beginning in childhood, and adult. Farthing, oral mucosal involvement as the sole or main manifestation of linear iga disease. Idiopathic, systemic diseaserelated, and drugrelated versions of this disorder have been described, with the latter most commonly associated with vancomycin. Mucous membrane involvement, notably oral cavity and conjunctiva, is relatively frequent, potentially leading to scarring sequelae 3,14,15,16. It is rare with an incidence in western europe of 0. Most cases are idiopathic but some drugs, infection, trauma, autoimmune disorders, and malignancies have been documented as potential inducers. 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 disease is an autoimmune blistering disease in which blisters form in the skin and mucous membranes. Linear iga disease is a rare blistering disease affecting the skin and less. A 73yearold male with advanced nonsmall cell lung cancer achieved a durable response to nivolumab monotherapy. The disease is thought to be related to the immune system iga protein attacking structures in the skin layers i.
Bowen described in 1901 the first six cases of linear iga bullous disease of childhood, by that time considered as dermatitis. Georgi m, scheckenbach c, kromminga a, partscht k, messer g, brocker eb, et al. Response to linear iga bullous dermatosis protracted by. Lesions typically appear 24 hours to 15 days after the first dose of vancomycin. Successful treatment of oral linear iga disease using. This disease has a varied clinical presentation especially when the disease affects the oral mucosa. Linear immunoglobulin a iga bullous dermatosis, also known as linear iga disease, is a rare disorder with an incidence of about 0.
Lad can be subdivided into adultonset, childhoodonset, and druginduced lad zone et al. Linear iga bullous dermatosis labd is an autoimmune vesiculobullous disease, which is typically idiopathic but can also rarely be caused by medications or infections. After 1 year of treatment, he developed gingival swelling and pain. Linear iga disease of adults lad is defined as a dapsoneresponsive subepidermal bullous disease commencing after puberty in which linear iga deposits are found at. Iga band is linear, at basement membrane, compared to dermatitis herpetiformis, which has granular band at dermal papillae igg in 25% of cases br j dermatol 1997. Linear iga disease is a blistering disease of skin characterized by a linear deposit of iga at the bmz by direct immunofluorescence of skin lesions. 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. Linear iga disease limited to the oral mucosa journal of the.
Pdf oral manifestations caused by the linear iga disease. Oral findings, for both patients, were of widespread painful ulceration present for two to three months and diagnosis was confirmed by biopsy and direct immunofluorescence. 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. We report a case of a previously healthy 77yearold man who developed a diffuse macular rash and hemorrhagic bullae on the left leg 10 days after placement of a vancomycin. 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 disease lad is a subepidermal blistering disorder characterized by pruritic lesions and linear deposition of iga autoantibodies at the bmz nemzer et al. 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 5 it was first described by tadeusz chorzelski in 1979 and may be divided into two types 587 adult linear iga disease is an acquired, autoimmune blistering disease that may present with a clinical.
Mar 06, 2020 linear immunoglobulin a iga dermatosis lad is an autoimmune subepidermal vesiculobullous disease that may be idiopathic or druginduced. You can join the registry to share your information with researchers and receive updates about participating in new research studies. The clinical presentation of lad consists of vesiculobullous lesions affecting the skin and mucosal surfaces. We read with great interest the case report by nartker et al1 on linear iga bullous disease labd associated with vancomycinloaded bone cement. It is best characterized pathologically by subepidermal bulla blister formation with. Vancomycin is the most common drug associated with labd. In our patient the oral manifestations predominated and were the only clinical manifestations for 5 years before the skin lesions appeared. It is a rare immunobullous disorder with an incidence in western europe of 0. Two cases of linear iga disease with clinical manifestations limited to the gingiva. 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. Linear iga bullous dermatosis protracted by vancomycin. Linear iga bullous dermatosis labd is a subepidermal, vesiculobullous eruption that can be drug induced, often by vancomycin. Pdf linear immunoglobulin a iga dermatosis lad is a rare autoimmune disorder that. Mmp lesions may also show iga immunofluorescence but are distinguished from linear iga disease by clinical presentation.
Certain drugs, autoimmune diseases, infection, chronic renal insufficiency, and malignancies have been implicated as the etiologic factors of this. Two cases of adult linear iga disease lad with oral and colonic involvement are presented. Linear iga disease is characterized by blisters occurring on the skin and mucous membranes. Linear iga disease of the gingiva following nivolumab. Request pdf successful treatment of oral linear iga disease using mycophenolate linear iga disease lad is a rare acquired autoimmune bullous disorder, characterized by linear deposition of. 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 of adults lad is defined as a dapsoneresponsive subepidermal bullous disease commencing after puberty in which linear iga deposits are found at the basement membrane zone bmz. Linear iga disease is a chronic, acquired, immunobullous condition of children and adults, with cutaneous and mucosal involvement, characterised by iga basementmembrane antibodies. Sometimes, infections and occasionally medicines may trigger linear iga disease. Linear iga disease is a chronic, acquired, subepidermal condition of children and adults, with cutaneous and mucosal involvement, characterised by iga basementmembrane antibodies. A case of linear iga disease limited to the oral mucosa ncbi. Linear iga dermatosis adult variant with oral manifestation.
Adult linear iga disease is an acquired, autoimmune blistering. It was first described by tadeusz chorzelski in 1979 and may be divided into two types. Linear iga dermatosis or chronic bullous disease of childhood cbdc is generally a rare, nonhereditary, autoimmune disease. Occasionally, other immunoreactants such as igg, igm, and. Linear iga disease an overview sciencedirect topics. 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. Linear iga dermatosis is an autoimmune disease histopathologically characterized by the linear deposition of iga at the basement membrane zone bmz. Glutensensitive enteropathy has been reported in some patients with adult lad. I have a very rare autoimmune disease oral linear iga it is attacking my mucus membranes in my mouth. Reader in the department of oral medicine and radiology at a. Oral manifestations of linear iga disease sciencedirect. The aim of this study was to assess possible differences between adults and. Vancomycininduced linear iga bullous dermatosis demonstrating the isomorphic phenomenon. Linear iga bullous dermatosis labd is an autoimmune vesiculobullous disease and can be caused by medications, autoimmune disorder, infections or malignancy.
Lad is an autoimmune disease histopathologically characterised by the linear deposition of iga at the bmz. While the majority of disorders of the mouth are centred upon the direct action of plaque, the oral tissues can be subject to change or damage as a. Linear iga disease genetic and rare diseases information. Skin biopsy is usually essential to differentiate it from other blistering disorders, and the diagnosis of linear iga disease is usually established by immunofluorescence. Vancomycin mediates iga autoreactivity in druginduced linear. S y n drom e n e t ic journal of genetic syndromes g t.
Biopsy revealed linear immunoglobulin a disease of the gingiva which was effectively treated with systemic steroids. The oral mucosa may show erosions, bullae, gingivitis, loss of teeth, and reduced ability to open the mouth. Linear iga bullous dermatosis in a child successfully. Early diagnosis is important for its management, because discontinuing the causative drug is most crucial. The following list of medications are in some way related to, or used in the treatment of this condition.
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