Linear IgA bullous dermatosis (LABD) is a rare blistering autoimmune disease. Although most cases are idiopathic, numerous drugs have been identified as capable of eliciting LABD. This paper provides an overview of the pathogenesis, diagnosis and treatment of drug-induced LABD, together with a report of a representative case of vancomycin-induced LABD. In addition, the results of a comprehensive literature review are reported to provide an overview of putative culprit drugs and their frequency, in order to enable clinicians to diagnose patients with putative adverse drug reactions presenting with blistering. Linear IgA bullous dermatosis (LABD) is a rare subepidermal autoimmune blistering disease characterized by linear deposition of IgA along the basement membrane zone. Although most reported cases are idiopathic, there is a subset of patients with drug-induced LABD. Various drugs have been associated with the drug-induced form of the disease. This paper reviews the literature on drugs reported to elicit linear IgA dermatosis and its specific clinical presentation. In addition, a case report of a 77-year-old male patient with linear IgA dermatosis induced by vancomycin is described. The aim of this paper is to emphasize the need to include this differential diagnosis in cases of suspected adverse drug reactions, as well as to highlight the role of drugs in LABD.
Summary The autosomal‐dominant genodermatoses Darier disease and Hailey‐Hailey disease present special challenges to dermatologists. Despite their similar pathogenesis featuring impaired adhesion of suprabasal keratinocytes as a result of defective ATPases in epidermal calcium channels, the two diseases differ considerably in clinical presentation and therapeutic options. Darier disease is characterized by reddish brown, keratotic papules in seborrheic and intertriginous areas, which may coalesce into extensive lesions. Individuals affected with Hailey‐Hailey disease primarily develop intertriginous papulovesicles and small blisters, which often evolve into erythematous plaques with erosions and painful fissures. Quality of life is significantly reduced because of complaints (itch, burning sensation, pain), body malodor and chronicity. Therapeutic options remain limited. Antiseptics and intermittent topical corticosteroids are a cornerstone of therapy, and systemic anti‐infective treatment is often required in cases of superinfection. Ablative surgical interventions such as dermabrasion and CO2 laser surgery can lead to long‐term remissions in intertriginous Hailey‐Hailey disease, while temporary relief may also be achieved by intralesional injections of botulinum toxin. Of the systemic medications available for Darier disease, acitretin, which is approved for this purpose, has the best supporting evidence. The efficacy of immunosuppressants and immune modulators is inconsistent. Low‐dose naltrexone produces more satisfactory results in Hailey‐Hailey than Darier disease. The present CME article summarizes current knowledge of the two dermatoses, taking recent developments into account.
Abbildung 1 Klinisches Bild. Ödematöse Schwellung und eine gelblich-krustige fokale Erosion des rechten Ohres der Patientin zum Zeitpunkt der Vorstellung an unserer Klinik.
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