Medication Summary
Large, randomized, placebo-controlled, double-blind studies have not been performed for the treatment of linear IgA dermatosis in children or adults. Most cases have been reported to respond to dapsone or sulfapyridine. [27, 28] Some clinicians favor the use of sulfapyridine because of the lower incidence of adverse effects [29] ; however, sulfapyridine is not available in the United States. The closely related sulfasalazine has been reported as a therapeutic option. [30] Some patients' conditions may not respond to sulfapyridine but do respond to treatment with dapsone. A response may be seen in 48-72 hours. A patient with concomitant linear IgA dermatosis and chronic idiopathic urticaria exhibited resolution of both diseases with omalizumab; a trial off the drug led to reactivation of disease. [31] Other reportedly useful medications include prednisone, sulfamethoxypyridazine, colchicine, dicloxacillin, mycophenolate mofetil, and intravenous immunoglobulin.
Tetracycline and niacinamide combination therapy has been reported as a possible therapeutic option. [32] This regimen, or niacinamide with any tetracycline-class antibiotic, has been well documented in the treatment of bullous pemphigoid.
Rituximab has shown efficacy in the treatment of bullous dermatoses and has been reported in the treatment of linear IgA dermatosis. [33, 34, 35, 36]
Drug-induced disease may be treated merely by withdrawal of the offending agent. In cases of linear IgA dermatosis induced by vancomycin, new lesions stop forming within approximately 2 weeks of withdrawal. [37] Particularly severe cases of drug-induced linear IgA dermatosis respond to a short course of oral corticosteroids.
Leprostatic agents
Class Summary
These agents have been shown to be beneficial in the treatment of linear IgA dermatosis.
Dapsone (Avlosulfon)
Dapsone is bactericidal and bacteriostatic against mycobacteria; the mechanism of action is similar to that of sulfonamides where competitive antagonists of PABA prevent formation of folic acid, inhibiting bacterial growth.
Corticosteroids
Class Summary
These agents have anti-inflammatory properties and cause profound and varied metabolic effects. In addition, these agents modify the body's immune response to diverse stimuli.
Prednisone (Deltasone)
Prednisone is an immunosuppressant used for the treatment of autoimmune disorders; it may decrease inflammation by reversing increased capillary permeability and suppressing PMN activity. Prednisone stabilizes lysosomal membranes and suppresses lymphocyte and antibody production.
Anti-inflammatory agents
Class Summary
These agents modulate events leading to inflammatory reactions.
Colchicine
Colchicine decreases leukocyte motility and phagocytosis in inflammatory responses.
Sulfasalazine (Azulfidine, Azulfidine EN)
Sulfasalazine elicits anti-inflammatory effects. It has a high affinity for distribution to connective tissues. Sulfasalazine is metabolized to 5-aminosalicylic acid and sulfapyridine.
Antibiotics
Class Summary
Empiric antimicrobial therapy must be comprehensive and should cover all likely pathogens in the context of the clinical setting.
Dicloxacillin (Dycill, Dynapen)
Dicloxacillin is used in the treatment of infections caused by penicillinase-producing staphylococci. It may be used to initiate therapy when staphylococcal infection is suspected.
Tetracycline (Achromycin V, Actisite, Sumycin)
Although an antibiotic, tetracycline has proven effective in linear IgA dermatosis, either alone or in conjunction with niacinamide (2 g/d). Efficacy may be due to anti-inflammatory properties.
Immunoglobulins
Class Summary
These agents are used to improve the clinical and immunologic aspects of the disease. They may decrease autoantibody production and increase solubilization and removal of immune complexes.
Immune globulins intravenous (Carimune NF, Flebogamma, Gammagard Liquid, Gammagard S/D, Privigen)
Immune globulins neutralize circulating antibodies and down-regulate proinflammatory cytokines, including INF-gamma; They block Fc receptors on macrophages, suppress inducer T and B cells, augment suppressor T cells, and block the complement cascade.
Antineoplastics, Anti-CD20 Monoclonal Antibodies
Rituximab (Rituxan)
Rituximab is a genetically engineered chimeric murine/human monoclonal antibody against human CD20, a molecule present in normal and malignant B lymphocytes. Rituximab is described in case reports as an effective treatment for linear IgA dermatosis.
Monoclonal Antibodies, Anti-asthmatics
Class Summary
Monoclonal antibody effects vary depending on their receptor target. Omalizumab binds to IgE on the surface of mast cells and basophils. It reduces the release of these mediators that promote an allergic response.
Omalizumab (Xolair)
Omalizumab is a recombinant, DNA-derived, humanized IgG monoclonal antibody that binds selectively to human IgE on the surface of mast cells and basophils. It reduces mediator release, which promotes an allergic response.
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Annular lesions demonstrating the string of beads sign.
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Bullous lesions on the genital area in a child with linear IgA dermatosis.
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Persons with linear immunoglobulin A (IgA) dermatosis may present with prominent ocular signs and symptoms.
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Neutrophilic microabscesses in linear immunoglobulin A (IgA) dermatosis.
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Bullous lesions of the palmar surface in an elderly man with vancomycin-induced linear immunoglobulin A (IgA) dermatosis.
