Linear IgA bullous dermatosis
Section snippets
Clinical features
Cutaneous manifestations in LAD are heterogeneous and may mimic other bullous diseases. Descriptions range from target-like to erythematous papules, urticarial plaques, or vesicobullous eruptions. Lesions may appear as tense arciform bullae in a “cluster of jewels” configuration (Fig 1) as seen in bullous pemphigoid (BP) or less commonly as grouped papulovesicles as seen in dermatitis herpetiformis (DH). Clinical presentations with similarities to erythema multiforme,2 Stevens Johnsons, and
Laboratory diagnosis
The histology of perilesional or lesional skin in LAD typically reveals a subepidermal bulla with a predominantly neutrophilic inflammatory infiltrate in the superficial dermis (papillary microabscesses), although mononuclear cells and eosinophils may also be present. The subepidermal process distinguishes LAD from such intraepithelial bullous diseases as pemphigus vulgaris and pemphigus filiaceus. LAD, however, may be difficult to distinguish from other subepidermal diseases based on histology
Therapy
Dapsone is considered the standard drug of choice and is begun at low doses (<0.5 mg/kg) and slowly increased until control of blistering and pruritis is achieved,65 a dose of 2.5–3.0 mg/kg is usually considered maximal. Common early side effects that should be routinely screened for include hemolytic anemia, which can be severe in patients with glucose-6-phosphate dehydrogenase (G6PD) deficiency, and methemoglobinemia, which can cause symptoms from benign cyanosis to weakness, dypsnea,
Molecular pathogenesis
Epithelial tissue integrity is maintained through cell attachment to the extracellular matrix (ECM) via specialized junctional complexes called hemidesmosomes (HD). Hemidesmosomes promote adhesion of epithelial cells to the basement membrane and may also serve as signaling devices through transmembrane receptor molecules such as α6β4 integrin.68 The HD plaque, intermediate filaments (IF), anchoring filaments, and anchoring fibrils comprise a functional unit termed the hemidesmosomal adhesion
Conclusions
Linear IgA disease is a rare subepidermal bullous disease that is usually diagnosed based on linear IgA deposition on DIF. Linear IgA disease shares many characteristics with other subepidermal bullous diseases, and it has been postulated that LAD represents an IgA response to BP immunoantigens; however, there are clinical and immunopathologic distinctions that have led to the recognition of LAD as a separate entity. For the most part, LAD needs only be differentiated from DH and BP. Features
Acknowledgements
The authors would like to acknowledge the funding of the Office of Research and Development, Palo Alto VA Health Care System, and NIH grant P01-AR44012.
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