Linear IgA Research Articles

Chronic bullous disease of childhood

Chronic bullous disease of childhood

Bullöse Autoimmunkrankheiten bei Kindern

We review the pathogenesis, clinical features, diagnosis, differential diagnosis, and therapy of autoimmune bullous skin diseases of childhood, especially of the most common linear IgA dermatosis. In autoimmune bullous diseases, autoantibodies are formed against different adhesion molecules of the skin. These are not only pathophysiologically relevant, but also serve as basis for diagnosis and follow-up of these diseases. In case an autoimmune bullous disease is suspected, histopathology and immunohistopathology (direct immunofluorescence microscopy) as well as serological tests (indirect immunofluorescence microscopy, ELISA, immunoblot) should be performed. Therapy depends on the diagnosis. In IgA-mediated pathogenesis, dapsone can be successfully used. In IgG-mediated diseases, immunosuppression with corticosteroids and steroid-sparing agents should be initiated, although only local therapy is sufficient to control a self-limiting pemphigus neonatorum. In dermatitis herpetiformis, a life-long gluten-free diet is recommended.

Sulfasalazine-Induced Linear Immunoglobulin A Bullous Dermatosis with DRESS

Linear immunoglobulin (Ig) A dermatosis is an immune-mediated bullous disease characterized by linear deposits of IgA along the basal membrane. While usually idiopathic, it can occasionally be induced by drug exposure. We report the case of a 60-year-old woman with rheumatoid arthritis being treated with sulfasalazine who developed linear IgA dermatosis and drug rash with eosinophilia and systemic symptoms (DRESS). The dermatosis and associated symptoms resolved following withdrawal of the drug and treatment with systemic corticosteroids for 2 months. This is the first report of sulfasalazine-induced linear IgA dermatosis in association with DRESS and we believe that sulfasalazine should be added to the list of drugs that can cause linear IgA dermatosis.

Fluorescence overlay antigen mapping using laser scanning confocal microscopy differentiates linear IgA bullous dermatosis from epidermolysis bullosa acquisita mediated by IgA

Linear IgA bullous dermatosis (LABD) and epidermolysis bullosa acquisita (EBA) mediated by IgA antibodies belong to the group of autoimmune subepidermal bullous diseases mediated by IgA autoantibodies. Early and correct diagnosis is crucial because the management and prognosis of the diseases are different. To determine whether fluorescence overlay antigen mapping using laser scanning confocal microscopy (FOAM-LSCM) is helpful in the differentiation between these diseases. FOAM-LSCM and immunoblot studies were performed in 19 patients with disseminated tense blisters who presented with in vivo bound and circulating IgA antibasement membrane zone (BMZ) antibodies on immunofluorescence. Using FOAM-LSCM, in vivo bound IgA above type IV collagen, which is characteristic for LABD, was seen in 14 of the 19 cases, whereas five of the 19 cases had IgA deposits below type IV collagen, typical for EBA. Immunoblot studies showed that IgA antibodies in 11 of the 14 patients with deposits above type IV collagen reacted with different epitopes on BP180, mainly with LAD-1, which is a target antigen in LABD. Among the five patients with deposits below type IV collagen, one showed IgA antibodies to the 200-kDa laminin γ-1 and one had antibodies to the 290-kDa type VII collagen, EBA antigen. Additionally, enzyme-linked immunosorbent assay with recombinant type VII collagen was positive in three of the five cases who presented with IgA deposits below type IV collagen on FOAM-LSCM. The results using FOAM-LSCM were consistent with those obtained on immunoblotting. FOAM-LSCM is useful in routine diagnostics in cases with undetectable circulating anti-BMZ antibodies, and can differentiate LABD from IgA-EBA, the former with in vivo bound IgA above type IV collagen and the latter with IgA deposits below type IV collagen.

Linear IgA bullous dermatosis herpeticum: Disseminated herpes simplex virus infection within the lesions of vancomycin-induced linear IgA bullous dermatosis

Linear IgA bullous dermatosis herpeticum: Disseminated herpes simplex virus infection within the lesions of vancomycin-induced linear IgA bullous dermatosis

Ofloxacin-induced linear IgA dermatosis mimicking toxic epidermal necrolysis

Ofloxacin-induced linear IgA dermatosis mimicking toxic epidermal necrolysis

Chronic Bullous Disease or Linear IgA Dermatosis of Childhood -Revisited

Linear IgA dermatosis or chronic bullous disease of childhood (CBDC) is a nonhereditary, autoimmune subepidermal bullous disease, characterized by the presence of continuous linear deposits of IgA autoantibodies along the basement membrane zone. Antigens mainly involved in the pathogenesis of CBDC are a 97-kDa and a 120 –kDa protein, which represent fragments of the extracellular domain of collagen XVII (BP180), a transmembrane protein playing a critical role in maintaining the linkage between the intracellular and the extracellular structural elements involved in epidermal adhesion. Humoral and cellular response contributes to the pathogenetic mechanism leading to blister formation. CBDC is characterized by a clinical polymorphism in each patient, with the “cluster of jewels” pattern being the most typical clinical lesion. For the establishment of CBDC diagnosis, combination of histology and immunofluorescence studies are of utmost importance. The majority of patients respond to dapsone, sulfapiridine or systemic steroids in cases of widespread disease. CBDC tends to resolve spontaneously within several months to 5 years after its onset. In this review article, the key features of this rare autoimmune bullous disease are revisited.

Linear IgA disease in an adult with unusual clinical features

A 19-year-old male presented with complaints of fluid-filled lesions on the body of 2 weeks duration. On examination, he was found to have multiple tense bullae distributed on the flexures, face, and genitalia with associated oral ulcers and “cluster of jewels” sign. The diagnosis was confirmed by histopathology and direct immunofluorescence. There are very few reports of linear IgA earlier from India according to the literature available so far, that too from the Southern part of the country. The patient had bullous pemphigoid-like lesions typically seen in adults, but the distribution of lesions was akin to that of the “chronic bullous disease of childhood variant” found in children. This case has been reported for rarity in this region and also because the patient had atypical morphology and distribution of lesions.

Linear IgA bullous dermatosis

Linear IgA disease is a rare chronic autoimmune subepidermal blistering disorder characterized by the linear deposition of IgA along the dermoepidermal junction. A 40-year-old housewife presented with symmetrically distributed generalized intensely pruritic vesiculobullous lesions of 1 year duration. Previous treatment with oral and topical steroids had induced only a temporary remission of the skin lesions. There was no history of drug intake in the recent past and her medical history was insignificant and there was no similar illness in her family members. Examination findings included vesicles arranged at the periphery of healed lesions along with post-inflammatory hyperpigmented macules on the trunk and extremities. There was no genital, mucosal or ocular involvement. Skin biopsy from the leg showed dense neutrophilic infiltration along with eosinophils at the tip of the dermal papillae and focal areas showed dermoepidermal clefting with bulla formation. Direct immunofluorescence of perilesional skin revealed linear deposits of IgA at the basement membrane zone. Therapeutically the patient responded well to dapsone. Though the presentation was classical, the case is reported for its rarity.

Application of dapsone 5% gel in a patient with dermatitis herpetiformis

Application of dapsone 5% gel in a patient with dermatitis herpetiformis

A further case of subacute prurigo‐like linear IgA bullous dermatosis: growing evidence of a new subset

A further case of subacute prurigo‐like linear IgA bullous dermatosis: growing evidence of a new subset

Toxic Epidermal Necrolysis

Toxic epidermal necrolysis (TEN) is a severe mucocutaneous drug-induced syndrome that causes massive keratinocyte apoptosis and therefore hydro-electrolytic disorders and systemic infection. TEN approximately affects one to two cases per million per year. Mortality rate may reach thirty percent of cases. Thus, TEN constitutes a therapeutic emergency at diagnosis. Typically, clinical examination shows a mucocutaneous detachment involving more than thirty percent of body area. Definitive diagnosis is made on cutaneous biopsy with histological exam that shows the blister of necrotic keratinocytes. Main differential diagnosis are acute staphylococcus epidermis, acute generalized exanthematous pustulosis, linear IgA bullous dermatosis, paraneoplastic pemphigus, bullous fixed pigmented erythema, acute lupus erythematosus. In the early days, SCORTEN gives a good estimation and is now widely used as prognostic score. Drugs are generally considered as the main etiology of TEN but in some cases bacterial or viral infections could be involved. Physiopathology remains unclear even if recent advances have reported the possible implication of immune pathways based on activation of T and NK cells. Treatment of TEN requires to be instituted as soon as the diagnosis is made and the patient is preferentially referred to a specialized unit. Supportive care consist of covering areas of cutaneous detachment. No other therapy has demonstrated its efficiency, but high-dose intravenous immunoglobulin might improve the prognosis.

Linear IgA bullous dermatosis following human papillomavirus vaccination

ejd.2012.1851 Auteur(s) : Shigeki Ikeya1 shigeki450@yahoo.co.jp, Shoko Urano1, Yoshiki Tokura2 1 Section of Dermatology, JA Shizuoka Koseiren Enshu Hospital, Hamamatsu 430-0929, Japan 2 Department of Dermatology, Hamamatsu University School of Medicine, Hamamatsu, Japan Linear IgA bullous dermatosis (LABD) is characterized by linear deposits of IgA at the basement membrane zone (BMZ) with IgA autoantibodies directed against several different antigens in the BMZ. Most cases are idiopathic, but medications, [...]

Linear IgA bullous dermatosis: report of five cases in Chile

Linear IgA bullous dermatosis (LABD) is an acquired autoimmune sub-epidermal vesiculobullous disease characterized by continuous linear IgA deposit on the basement membrane zone, as visualized on direct immunofluorescence microscopy. LABD can affect both adults and children. The disease is very uncommon, with a still unknown incidence in the South American population. All confirmed cases of LABD by histological and immunofluorescence in our hospital were studied. The confirmed cases were three females and two males, aged from 8 to 87 years. Precipitant events associated with LABD were drug consumption (non-steroid inflammatory agents in two cases) and ulcerative colitis (one case). Most of our patients were treated with dapsone, resulting in remission. Our series confirms the heterogeneous clinical features of this uncommon disease in concordance with a larger series of patients reported in the literature.

Development of linear IgA bullous dermatosis in a patient with psoriasis taking ustekinumab

Development of linear IgA bullous dermatosis in a patient with psoriasis taking ustekinumab

Prurigo even as a symptom of a linear IgA bullous disease

Prurigo even as a symptom of a linear IgA bullous disease

Enfermedad cutánea ampollosa en el lupus eritematoso sistémico

El lupus eritematoso sistémico ampolloso (LESA) es una manifestación infrecuente del lupus eritematoso sistémico (LES) causada por autoanticuerpos contra el colágeno tipo VII y otros componentes esenciales de la unión dermoepidérmica. Se presenta en pacientes que cumplen los criterios revisados de clasificación para LES del Colegio Americano de Reumatología y en presencia de alta actividad sistémica. El diagnóstico diferencial incluye otras enfermedades ampollosas autoinmunes como el penfigoide ampolloso, la epidermólisis ampollosa adquirida, la dermatosis ampollosa IgA lineal y la dermatitis herpetiforme las cuales también han sido informadas en LES.

Dermatosis ampollosa inducida por inmunoglobulina A lineal con clínica de síndrome DRESS por sulfasalazina

Linear immunoglobulin (Ig) A dermatosis is an immune-mediated bullous disease characterized by linear deposits of IgA along the basal membrane. While usually idiopathic, it can occasionally be induced by drug exposure. We report the case of a 60-year-old woman with rheumatoid arthritis being treated with sulfasalazine who developed linear IgA dermatosis and drug rash with eosinophilia and systemic symptoms (DRESS). The dermatosis and associated symptoms resolved following withdrawal of the drug and treatment with systemic corticosteroids for 2 months. This is the first report of sulfasalazine-induced linear IgA dermatosis in association with DRESS and we believe that sulfasalazine should be added to the list of drugs that can cause linear IgA dermatosis.

Linear IgA dermatosis in a patient with primary Sjogren's syndrome

Linear IgA dermatosis in a patient with primary Sjogren's syndrome

Diagnosis of linear IgA disease: The results of immunopathologic examinations can be ambiguous

Diagnosis of linear IgA disease: The results of immunopathologic examinations can be ambiguous