Annular bullous diseases

Sussman, Morgan; Zhai, Lisa; Morquette, Alexandra J; Huang, Simo; Hsu, Sylvia

doi:10.1016/j.clindermatol.2021.12.012

Cited by 3 publications

(1 citation statement)

References 53 publications

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“…In idiopathic LABD, several dermal antigenic targets of IgA autoantibodies have been identified, including the 97-kDa (LABD97) and 120-kDa (LAD-1) fragments of bullous pemphigoid (BP) antigen 180, BP230, LAD285, laminin 332, and type VII collagen (COL7) [6,8,9]. In contrast, the pathomechanisms underlying DILABD are still being investigated [10]. Yamagami et al recently demonstrated that IgA reactivity to the basement membrane zone was enhanced by the addition of vancomycin to patient serum in a dosedependent manner on IIF and identified COL7 as a target antigen of IgA autoantibodies [8] Similarly, no unifying pathomechanism exists for SJS/TEN.…”

Section: Discussionmentioning

confidence: 99%

Stevens-Johnson Syndrome or Linear IgA Bullous Dermatosis? Sheet-Like Desquamation in a Hospitalized Patient

Park¹,

Hamilton²,

Patel³

et al. 2022

Cureus

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Linear IgA bullous dermatosis (LABD) is a rare autoimmune blistering disease characterized by linear IgA deposition along the dermo-epidermal junction on direct immunofluorescence (DIF). LABD appears clinically as erythematous polycyclic lesions in younger patients but can show considerable phenotypic heterogeneity in older patients, often leading to misdiagnoses such as bullous pemphigoid, pemphigus vulgaris, Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN), or other bullous conditions. Cases of LABD mimicking SJS/TEN require prompt skin biopsies for histopathology and DIF for disease differentiation and medical decision-making. In cases of suspected drug-induced LABD or SJS/TEN, identification and removal of the offending agent are paramount. The preferred treatment for LABD is oral dapsone, while SJS/TEN may respond better to cyclosporine or a combination of intravenous immunoglobulin and systemic corticosteroids. This case highlights the rare occurrence of LABD mimicking SJS/TEN and emphasizes the details that clinicians must know to guide patient management.

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