049 97-kD linear IgA bullous dermatosis antigen localizes at lamina lucida between NC16A domain and C-terminal domain of 180-kD bullous pemphigoid antigen

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 Child With Antibodies Targeting Both Linear IgA Bullous Dermatosis and Bullous Pemphigoid Antigens

Archives of Dermatology ◽

10.1001/archderm.1995.01690240102017 ◽

1995 ◽

Vol 131 (12) ◽

pp. 1438 ◽

Cited By ~ 19

Author(s):

Thomas N. Darling

Keyword(s):

Bullous Pemphigoid ◽

Bullous Dermatosis ◽

Linear Iga Bullous Dermatosis

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A child with antibodies targeting both linear IgA bullous dermatosis and bullous pemphigoid antigens

Archives of Dermatology ◽

10.1001/archderm.131.12.1438 ◽

1995 ◽

Vol 131 (12) ◽

pp. 1438-1442 ◽

Cited By ~ 7

Author(s):

T. N. Darling

Keyword(s):

Bullous Pemphigoid ◽

Bullous Dermatosis ◽

Linear Iga Bullous Dermatosis

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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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Immunoelectronmicroscopic differentiation of linear IgA bullous dermatosis of adults with coexistence of IgA and IgG deposition from bullous pemphigoid

Journal of the American Academy of Dermatology ◽

10.1016/0190-9622(92)70205-t ◽

1992 ◽

Vol 27 (3) ◽

pp. 394-399 ◽

Cited By ~ 13

Author(s):

Atsuko Adachi ◽

Masahiro Tani ◽

Shuho Matsubayashi ◽

Koichi Shibata ◽

Tatsuya Horikawa ◽

...

Keyword(s):

Bullous Pemphigoid ◽

Bullous Dermatosis ◽

Linear Iga Bullous Dermatosis

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Clinical Relevance of Autoantibodies in Patients with Autoimmune Bullous Dermatosis

Clinical and Developmental Immunology ◽

10.1155/2012/369546 ◽

2012 ◽

Vol 2012 ◽

pp. 1-9 ◽

Cited By ~ 12

Author(s):

Lilla Mihályi ◽

Mária Kiss ◽

Attila Dobozy ◽

Lajos Kemény ◽

Sándor Husz

Keyword(s):

Bullous Pemphigoid ◽

Clinical Relevance ◽

Distinct Entity ◽

Diphenyl Sulfone ◽

Bullous Dermatosis ◽

Iga Pemphigus ◽

Iga Antibodies ◽

Linear Iga Bullous Dermatosis ◽

Perilesional Skin ◽

Diamino Diphenyl Sulfone

The authors present their experience related to the diagnosis, treatment, and followup of 431 patients with bullous pemphigoid, 14 patients with juvenile bullous pemphigoid, and 273 patients with pemphigus. The detection of autoantibodies plays an outstanding role in the diagnosis and differential diagnosis. Paraneoplastic pemphigoid is suggested to be a distinct entity from the group of bullous pemphigoid in view of the linear C3 deposits along the basement membrane of the perilesional skin and the “ladder” configuration of autoantibodies demonstrated by western blot analysis. It is proposed that IgA pemphigoid should be differentiated from the linear IgA dermatoses. Immunosuppressive therapy is recommended in which the maintenance dose of corticosteroid is administered every second day, thereby reducing the side effects of the corticosteroids. Following the detection of IgA antibodies (IgA pemphigoid, linear IgA bullous dermatosis, and IgA pemphigus), diamino diphenyl sulfone (dapsone) therapy is preferred alone or in combination. The clinical relevance of autoantibodies in patients with autoimmune bullous dermatosis is stressed.

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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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