A clinical and serological study of linear IgA bullous dermatosis without linear immunoglobulin deposition other than IgA at the basement membrane zone using direct immunofluorescence

A 68-year-old woman was referred to the unit of dermatology for a large basal cell carcinoma on the chin. She was treated with imiquimod cream 5%, and 4 weeks after she developed isolated and grouped tense serum-filled vesicles and bullae on lips, nose, scalp, ankles and lumbar area, and then expanded to the whole body. Histological examination was consistent with a subepidermal bullous dermatosis. Moreover, direct immunofluorescence showed linear deposition of IgA at the basement membrane zone supporting the diagnosis of linear IgA bullous dermatosis (LABD). Dapsone 50 mg/day was administered, and the lesions gradually improved within some weeks, and no new lesions appeared. The temporal relationship between the application of the drug and the development of the disease indicates a role of this topical agent in triggering LABD.

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A clinical and serological study of linear IgA bullous dermatosis

Journal of the American Academy of Dermatology ◽

10.1016/j.jaad.2016.02.554 ◽

2016 ◽

Vol 74 (5) ◽

pp. AB141

Keyword(s):

Serological Study ◽

Bullous Dermatosis ◽

Linear Iga Bullous Dermatosis

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Autoimmune bullous disorders

Clinical Chemistry and Laboratory Medicine (CCLM) ◽

10.1515/cclm.2006.027 ◽

2006 ◽

Vol 44 (2) ◽

Cited By ~ 12

Author(s):

Rüdiger Eming ◽

Michael Hertl

Keyword(s):

Basement Membrane ◽

Skin Diseases ◽

Immunoblot Analysis ◽

Primary Diagnosis ◽

Basement Membrane Zone ◽

Epidermal Keratinocytes ◽

Direct Immunofluorescence ◽

Organ Specific ◽

Circulating Autoantibodies ◽

Bullous Skin Diseases

AbstractBullous skin diseases represent a group of organ-specific autoimmune disorders characterised by binding of circulating autoantibodies to adhesion molecules of the epidermis and the dermo-epidermal basement membrane zone. Binding of these autoantibodies to their antigenic targets results in loss of adhesion between epidermal keratinocytes and at the level of the basement membrane zone. Chronic blisters and secondary painful erosions are the clinical hallmark of autoimmune bullous disorders. Histopathology reveals the location of blister formation and helps to classify the subtype of the bullous skin disorder. Immunofluorescence is crucial for diagnosing autoimmune bullous skin disorders. Tissue-bound autoantibodies are detected by direct immunofluorescence of perilesional skin. Circulating autoantibodies can be visualised by indirect immunofluorescence using tissue substrates such as monkey oesophagus and sodium chloride-split human skin. Most of the autoantigens are available as recombinant proteins, which allows for autoantibody screening by ELISA or immunoblot analysis to confirm the primary diagnosis and, importantly, for immunoserological follow-up of patients.

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Linear IGA bullous dermatosis potentially triggered by vaccination

International Journal of Immunopathology and Pharmacology ◽

10.1177/20587384211021218 ◽

2022 ◽

Vol 36 ◽

pp. 205873842110212

Author(s):

Alberto Corrà ◽

Veronica Bonciolini ◽

Lavinia Quintarelli ◽

Alice Verdelli ◽

Marzia Caproni

Keyword(s):

Basement Membrane Zone ◽

Dermoepidermal Junction ◽

Pathogenetic Role ◽

Autoimmune Blistering Disease ◽

Bullous Dermatosis ◽

Iga Antibodies ◽

Blistering Disease ◽

Adults And Children ◽

Linear Iga Bullous Dermatosis

Linear IgA bullous dermatosis (LABD) is a mucocutaneous autoimmune blistering disease affecting both adults and children. It is caused by IgA antibodies targeting multiple antigens along the basement membrane zone, leading to disruption of dermoepidermal junction and development of bullous lesions which often presents in characteristic arrangement. Although most LABD cases have been reported to be idiopathic, different triggers have been described, including several drugs and infection. However, the occurrence of vaccine-induced cases of LABD is not widely known and accepted due to the few reports available. We present two cases of LABD occurred following different triggers, rising the suspicion for a possible pathogenetic role of vaccines.

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Other Vesiculobullous Diseases

10.2310/im.1105 ◽

2020 ◽

Author(s):

Aakaash Varma ◽

Annette Czernik ◽

Jacob Levitt

Keyword(s):

Bullous Pemphigoid ◽

Epidermolysis Bullosa ◽

Tissue Transglutaminase ◽

Direct Immunofluorescence ◽

Cicatricial Pemphigoid ◽

Positive Serology ◽

Fixed Drug Eruption ◽

Varicella Zoster ◽

Bullous Dermatosis ◽

Linear Iga Bullous Dermatosis

Less common immunobullous diseases include cicatricial pemphigoid, epidermolysis bullosa acquisita, and linear IgA bullous dermatosis. Diagnosis of these entities are made through direct immunofluorescence, sometimes requires salt-split skin, as well as, in the case of cicatricial pemphigoid, mucosal scarring. As in pemphigus vulgaris and bullous pemphigoid, common therapies include rituximab, prednisone, and IVIg. Dapsone can be particularly effective in linear IgA bullous dermatosis and bullous lupus. Dermatitis herpetiformis is a rare cutaneous manifestation of gluten sensitivity, characterized by pruritic vesicles on extensor surfaces, that responds to dapsone and gluten avoidance. This diagnosis is confirmed with biopsy and positive serology for anti-tissue transglutaminase IgA. Blistering hypersensitivity reactions include TEN, SJS, erythema multiforme, and fixed drug eruption. All are characterized by varying degrees of keratinocyte necrosis. Common to the management of all include cessation of the offending agent. TEN can be managed by cyclosporine, TNF-inhibition, or—more controversially—IVIg. SJS can be effectively managed with systemic steroids. EM responds variably to a number of agents, including antiviral nucleoside analogues, prednisone, thalidomide, apremilast, and tofacitinib. Infectious causes of blisters include Staphylococcus aureus, HSV, and varicella zoster virus. Epidermolysis bullosa comprises a variety of genetically defective structural proteins of the skin. Recessive variants and those affecting deeper proteins carry more severe phenotypes. Management is best achieved at specialty centers and involves careful wound care as well as prevention of friction. Gene therapy is on the horizon for these disorders. Other blistering entities, mechanical or inflammatory in nature, are also discussed at the end of this chapter. This review contains 13 figures, 1 table, and 86 references. Keywords: Blisters, bullae, bullous, pemphigoid, necrolysis

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Chronic bullous disease of childhood – A case report / Hronična bulozna bolest Kod Dece – Prikaz Slučaja

Serbian Journal of Dermatology and Venerology ◽

10.2478/v10249-011-0030-z ◽

2010 ◽

Vol 2 (4) ◽

pp. 137-142

Author(s):

Đorđi Gocev ◽

Katerina Damevska ◽

Suzana Nikolovska ◽

Ljubica Pavlova ◽

Nada Petrova

Keyword(s):

First Episode ◽

Direct Immunofluorescence ◽

Rapid Progression ◽

Upper Respiratory Infection ◽

Bullous Disease ◽

Systemic Corticosteroids ◽

Bullous Dermatosis ◽

The Face ◽

Linear Iga Bullous Dermatosis

Abstract Linear IgA bullous dermatosis is a chronic, acquired, autoimmune subepidermal vesiculobullous disease. Both children and adults are affected. It is characterized by direct immunofluorescence findings of linear immunoglobulins class A (IgA) deposits along the dermal-epidermal junction (basement membrane zone). In children, the disease is commonly referred to as chronic bullous disease of childhood and it mostly affects children between 2 and 5 years. The onset of the disease is acute; the first episode is the most severe, while recurrences tend to wax and wane in severity and last till puberty or even longer. Diaminodiphenylsulfone is the treatment of choice, although systemic corticosteroids are reported to be very effective as well. We report a 3-year-old boy with a vesiculobullous eruption which developed one week following administration of cephalexin for upper respiratory infection. He was referred to our Clinic from other health institutions as treatment failure for suspected strophulus or impetigo bullosus. On admission, the patient had fever and numerous vesiculobullous and erosive lesions distributed on the face and trunk. After immunohistological verification, the treatment with prednisone 25 mg/d was introduced, due to rapid progression of the disease and the fact that diaminodiphenylsulfone was not available. Improvement occurred after 2 weeks, so the dose was carefully tapered, taking into account the possibility of adrenal suppression. The medication was completely excluded within the next three months. No serious side effects were observed, except transitory hirsutism. The patient has had no relapses over the last 20 months of clinical follow-up.

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