Linear IgA Bullous Dermatosis: A Retrospective Study of 23 Patients in Denmark

Abstract

Linear IgA bullous dermatosis (LAD) is an autoimmune, chronic bullous disease affecting primarily young children and adults. Studies on LAD are relatively sparse and from Scandinavia we could only find a few case reports. Therefore we decided to conduct a retrospective investigation of patients seen at our department since 1972. A total of 23 patients were identified; 7 children (F:M ratio 0.75) and 16 adults (F:M ratio 0.78). Mean age at disease onset in the two age groups were 2.7 and 56.8 years. Estimated incidence rate in our region: 0.67 per million per year. The most commonly used treatment modalities were corticosteroids, dapsone and sulphapyridine.

Piperacillin-tazobactam-induced linear IgA bullous dermatosis presenting clinically as Stevens-Johnson syndrome/toxic epidermal necrolysis overlap

“…The reported incidence ranges between 0.23 and 2.3 cases per million person-years. 1 Our patient with drug-induced LABD had no target lesions or oral/ocular involvement, which are distinguishing features in classic SJS/TEN. However, he had other clinical features that were in keeping with drug-induced SJS/TEN overlap.…”

“…LABD is a rare autoimmune mucocutaneous disorder. The reported incidence ranges between 0.23 and 2.3 cases per million person‐years . Our patient with drug‐induced LABD had no target lesions or oral/ocular involvement, which are distinguishing features in classic SJS/TEN.…”

“…Linear IgA bullous dermatosis (LABD) is a subepidermal autoimmune bullous disease characterized by linear IgA deposition at the basement membrane zone (BMZ), which can be seen with direct immunofluorescence. [1][2][3] LABD classically presents with a widespread annular eruption of vesicles and tense bullae. 4 However, the clinical presentation of LABD is heterogeneous.…”

Non‐infectious diffuse vesiculobullous rash in a pancreas and renal transplant recipient

“…In comparison, LABD is a rare dermatologic condition, with an incidence on the order of 1 case per million individuals per year . It is subclassified into idiopathic and drug‐induced forms, with the latter representing up to 37.5% of adult cases . Multiple antibiotics such as vancomycin, piperacillin‐tazobactam, aminopenicillins have been implicated in LABD .…”

Successful treatment of a case of idiopathic linear IgA bullous dermatosis with oral sulfasalazine

“…Linear IgA bullous dermatosis (LABD) is an acquired autoimmune chronic disorder. It primarily affects young children (4–5 years) and older adults (60–65 years) and often characterized by erythematous papules, diffuse bullae, vesicles, and erosions (Lings & Bygum, ). LABD is also associated with the expression of continuous linear IgA deposits in the basement membrane zone (BMZ).…”