Idiopathic linear IgA bullous dermatosis with mucosal involvement
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Austin B. Ambur
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Timothy A. Nyckowski
A 62-year-old woman presented to the hospital in July 2021 with a diffuse blistering rash. She had been suffering with limited cutaneous involvement for several years, which significantly worsened over a 2-month period which prompted her hospital admission. Cutaneous examination revealed diffuse tense vesicles and bullae in an annular arrangement on the arms, legs, trunk, ocular mucosa, and oral mucosa (Figures 1 and 2). Direct immunofluorescence (DIF) revealed linear IgA deposited along the basement membrane zone (BMZ). Medication reconciliation was unremarkable for any causative agents. The patient was diagnosed with idiopathic linear IgA bullous dermatosis (LABD) and initiated on oral dapsone with full resolution within the following week.
Tense bullae and erosions on the dorsal tongue.
Generalized erosions and tense vesicles in a polycyclic arrangement on the back and dorsal arms.
LABD is a rare autoimmune blistering disease which is characterized by subepidermal blister formation with a linear deposition of IgA along the BMZ [1]. It typically affects adults older than 60 years of age and may be idiopathic or drug-induced [2]. While vancomycin is the most common culprit medication, penicillin, captopril, and non-steroidal anti-inflammatory drugs (NSAIDs) may also be involved [2]. IgA autoantibodies are typically formed against antigens related to BPAG2, namely LAD-1 and LABD97. Clinically it typically presents as tense vesicles or bullae and urticarial plaques in an annular, polycyclic, or herpetiform arrangement which have been described as a “crown of jewels.” Mucosal involvement presents in up to 80% of patients as bullae or erosions and most commonly affects the oral and ocular mucosa [1]. Histologically neutrophils will diffusely line up along the BMZ with basal vacuolar change. DIF demonstrates linear IgA along the BMZ. This is an important diagnostic finding, as this condition may clinically resemble other blistering conditions such as bullous pemphigoid, dermatitis herpetiformis, and epidermolysis bullosa aquisita, which will all be distinguished by the DIF [2]. Dapsone is the treatment of choice and typically demonstrates rapid response within 72 h.
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Research funding: None reported.
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Author contributions: Both authors provided substantial contributions to conception and design, acquisition of data, or analysis and interpretation of data; both authors drafted the article or revised it critically for important intellectual content; both authors gave final approval of the version of the article to be published; and both authors agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
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Competing interests: None reported.
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Informed consent: The patient described in this report provided written informed consent.
References
1. Wojnarowska, F, Marsden, RA, Bhogal, B, Black, MM. Chronic bullous disease of childhood, childhood cicatricial pemphigoid, and linear IgA disease of adults. A comparative study demonstrating clinical and immunopathologic overlap. J Am Acad Dermatol 1988;19:792–805. https://doi.org/10.1016/s0190-9622(88)70236-4.Search in Google Scholar
2. Bolognia, JL, Schaffer, JV, Cerroni, L. Chapter 2: drug reactions. In: Dermatology, 4th ed. Philadelphia: Elsevier; 2018:360–1 pp.Search in Google Scholar
© 2022 Austin B. Ambur and Timothy A. Nyckowski, published by De Gruyter, Berlin/Boston
This work is licensed under the Creative Commons Attribution 4.0 International License.
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- Frontmatter
- Behavioral Health
- Commentary
- Overcoming reward deficiency syndrome by the induction of “dopamine homeostasis” instead of opioids for addiction: illusion or reality?
- General
- Original Article
- The association between operating margin and surgical diversity at Critical Access Hospitals
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