2017
DOI: 10.1007/s12026-017-8975-2
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Abstract: Subepidermal autoimmune bullous diseases of the skin and mucosae comprise a large group of chronic diseases, including bullous pemphigoid, pemphigoid gestationis, mucous membrane pemphigoid, linear IgA bullous dermatosis, epidermolysis bullosa acquisita, and anti-p200 pemphigoid. These diseases are characterized by an antibody response toward structural components of the basement membrane zone, resulting in subepidermal blistering. The epidemiological features of these diseases vary substantially in different … Show more

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Cited by 70 publications
(71 citation statements)
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“…It is characterized by a linear deposition of IgA along the blister base, with a predominantly neutrophilic dermal infiltrate . LABD usually presents with tense blisters in a pearl necklace‐like arrangement and polycyclic urticarial plaques on the trunk and extremities . The immunofluorescence of perilesional skin is the gold standard to confirm the diagnosis …”
Section: Discussionmentioning
confidence: 99%
“…It is characterized by a linear deposition of IgA along the blister base, with a predominantly neutrophilic dermal infiltrate . LABD usually presents with tense blisters in a pearl necklace‐like arrangement and polycyclic urticarial plaques on the trunk and extremities . The immunofluorescence of perilesional skin is the gold standard to confirm the diagnosis …”
Section: Discussionmentioning
confidence: 99%
“…58 The medication has been used in one patient with PV following approval for compassionate use. 62 The patient was unable to be weaned off prednisone and had failed to go into complete remission following three doses of Table 1 Listing of various pemphigus and pemphigoid disorders with their major autoantigens 12,13,221,222 rituximab. Veltuzumab was administered as two subcutaneous 320 mg doses 2 weeks apart.…”
Section: B-cell-directed Therapiesmentioning
confidence: 99%
“…18 The annual incidence ranges between 0.2 and 1.0 cases per million, estimated in different regions. 20 Childhood LAD has a distinct clinical presentation, with blisters on urticarial Figure 4 (a, b) Linear IgA disease in a 2-year-old girl, presenting with bullae and vesicles in a circinate configuration, known as 'crown of jewels' or 'string of pearls'. plaques in a typical circinate or serpiginous configuration, forming a 'crown of jewels' or a 'string of pearls' (Fig.…”
Section: Epidermolysis Bullosa Acquisitamentioning
confidence: 99%