Linear IgA bullous dermatosis in one of two piroxicam-induced eruptions: A distinct direct immunofluorescence trend revealed by the literature

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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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

British Journal of Dermatology ◽

10.1111/bjd.15232 ◽

2017 ◽

Vol 177 (1) ◽

pp. 152-157 ◽

Cited By ~ 10

Author(s):

C. Ohata ◽

N. Ishii ◽

H. Koga ◽

T. Nakama

Keyword(s):

Basement Membrane ◽

Basement Membrane Zone ◽

Direct Immunofluorescence ◽

Serological Study ◽

Bullous Dermatosis ◽

Linear Iga Bullous Dermatosis

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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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Other Vesiculobullous Diseases

10.2310/fm.1105 ◽

2020 ◽

Author(s):

Annette Czernik ◽

Aakaash Varma ◽

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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A Case of Linear IgA Bullous Dermatosis Induced by Aspirin Therapy

Iranian Journal of Allergy Asthma and Immunology ◽

10.18502/ijaai.v19i5.4473 ◽

2020 ◽

Author(s):

Mohammad Nabavi ◽

Afshin Rezaeifar ◽

Ali Sadeghinia ◽

Saba Arshi ◽

Sima Bahrami ◽

...

Keyword(s):

Direct Immunofluorescence ◽

Causative Role ◽

Drug Induced ◽

Autoimmune Blistering Disease ◽

Bullous Dermatosis ◽

Causative Agents ◽

History Of ◽

Blistering Disease ◽

Linear Iga Bullous Dermatosis ◽

String Of Pearls

Linear IgA bullous dermatosis (LABD) is a rare autoimmune blistering disease that may be triggered by some diseases and medications. For the latter one, non-steroidal anti-inflammatory drugs (NSAIDs) have been identified as one of the potential causative agents to develop LABD. Here, a rare case of drug-induced LABD is introduced. A 13-month-old Iranian boy presented with a history of generalized blisters, displaying the classic “string of pearls” sign who was eventually diagnosed as a case of LABD. In his admission, he was diagnosed whit Mucocutaneous lymph node syndrome and treated with aspirin. Some features like appearing the characteristic lesions one week following the administration of aspirin, rapid clearance of lesions after the withdrawal of the drug, and reappearance of new lesions after readministration of aspirin were highly suggestive of aspirin-induced LABD. To establish the diagnosis, we used the “Naranjo probability score” which determined the probable causative role of aspirin. The diagnosis was confirmed by showing the positive IgA deposition in the basement membrane zone in a direct immunofluorescence study of the skin biopsy. The child was treated with dapsone with dramatical response to the drug.

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A Case of Bullous Rash Apparently Triggered by Meningococcal and Rotavirus Vaccines in an Infant: Focus on Infantile Bullous Pemphigoid

Dermatopathology ◽

10.3390/dermatopathology8010006 ◽

2021 ◽

Vol 8 (1) ◽

pp. 33-36

Author(s):

Iria Neri ◽

Valeria Evangelista ◽

Alba Guglielmo ◽

Andrea Sechi ◽

Annalucia Virdi

Keyword(s):

Bullous Pemphigoid ◽

Rare Condition ◽

Direct Immunofluorescence ◽

Rotavirus Vaccines ◽

Systemic Corticosteroids ◽

Bullous Dermatosis ◽

Linear Iga Bullous Dermatosis ◽

Bullous Diseases ◽

Annular Lesions

Bullous pemphigoid (BP) is an autoimmune bullous disease and is a rare condition in childhood. Acquired tense acral bullae and fixed urticarial annular lesions on the trunk are diagnostic clues of infantile BP. Diagnosis is supported by immunosorbent assay (IgG anti-BP180 and BP230) and direct immunofluorescence (linear deposition of IgG at the dermo-epidermal junction). Topical and/or systemic corticosteroids are the first-line treatment. The prognosis is good with a self-limited clinical course. Differential diagnoses include impetigo and other bullous diseases in children, such as dermatitis herpetiformis, linear IgA bullous dermatosis and erythema multiforme. The etiopathogenesis is still unknown, and the role of antigen stimuli such as infections, drugs and vaccination is still debated.

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Dermatitis herpetiformis: Potential for confusion with linear IgA bullous dermatosis on direct immunofluorescence

Dermatology Online Journal ◽

10.5070/d32kw0r6bh ◽

2008 ◽

Vol 14 (1) ◽

Author(s):

Livia Van ◽

John C Browning ◽

Ravi S Krishnan ◽

Brandi M Kenner-Bell ◽

Sylvia Hsu

Keyword(s):

Dermatitis Herpetiformis ◽

Direct Immunofluorescence ◽

Bullous Dermatosis ◽

Linear Iga Bullous Dermatosis

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Topical imiquimod-induced linear IgA bullous dermatosis

BMJ Case Reports ◽

10.1136/bcr-2019-230037 ◽

2019 ◽

Vol 12 (7) ◽

pp. e230037 ◽

Cited By ~ 1

Author(s):

Giulia Tadiotto Cicogna ◽

Martina Ferranti ◽

Daniele Vaccari ◽

Mauro Alaibac

Keyword(s):

Temporal Relationship ◽

Basement Membrane Zone ◽

Whole Body ◽

Direct Immunofluorescence ◽

Topical Agent ◽

Imiquimod Cream ◽

Bullous Dermatosis ◽

Lumbar Area ◽

Linear Iga Bullous Dermatosis

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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Idiopathic linear IgA bullous dermatosis treated with prednisone

BMJ Case Reports ◽

10.1136/bcr-2021-242237 ◽

2021 ◽

Vol 14 (5) ◽

pp. e242237

Author(s):

Christopher Cantoria Garces ◽

M Fahad Salam ◽

Brian Nohomovich ◽

Merryl Treasa Varghese

Keyword(s):

Case Reports ◽

Acute Onset ◽

Direct Immunofluorescence ◽

Dermoepidermal Junction ◽

Mucosal Surfaces ◽

Bullous Dermatosis ◽

History Of ◽

Linear Iga Bullous Dermatosis ◽

Linear Band ◽

Subepidermal Blister

We present a case of a 43-year-old man with a medical history of paroxysmal atrial fibrillation that presented with acute onset generalised vesiculobullous rash of 1-week duration. The rash was initially noticed on his groin and then spread to his hands, feet and mucosal surfaces. Laboratory tests were unremarkable, including an extensive infection aetiology work-up. Punch biopsies were obtained of a fresh lesion and were stained with H&E and sent for direct immunofluorescence. Light microscopy and immunofluorescence study demonstrated a subepidermal blister with predominant neutrophilic infiltrates and a linear band of IgA at the dermoepidermal junction, respectively. The patient was diagnosed with linear IgA bullous dermatosis and was subsequently treated with 0.5 mg/kg of prednisone daily following previous case reports. At 1-week follow-up as an outpatient, the bullae became crusted, and the rash was nearly completely regressed.

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