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.[13] Some clinicians favor the use of sulfapyridine because of the lower incidence of adverse effects.[14] However, some patients' conditions may not respond to sulfapyridine but do respond to treatment with dapsone. A response may be seen in 48-72 hours. Other reportedly useful medications include prednisone, sulfamethoxypyridazine, colchicine, dicloxacillin, mycophenolate mofetil, and intravenous immunoglobulin.
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.[15] 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)
Bactericidal and bacteriostatic against mycobacteria; mechanism of action is similar to that of sulfonamides where competitive antagonists of PABA prevent formation of folic acid, inhibiting bacterial growth.
Sulfonamides
Class Summary
These agents exert bacteriostatic action by competitive antagonism of para-aminobenzoic acid (PABA). Microorganisms that require exogenous folic acid and do not synthesize folic acid are not susceptible to the action of sulfonamides.
Sulfapyridine
Competitive antagonist of PABA. Mechanism of action in linear IgA dermatosis is unknown. Currently not available in the United States.
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)
Immunosuppressant for treatment of autoimmune disorders; may decrease inflammation by reversing increased capillary permeability and suppressing PMN activity. Stabilizes lysosomal membranes and also suppresses lymphocyte and antibody production.
Anti-inflammatory agents
Class Summary
These agents modulate events leading to inflammatory reactions.
Colchicine
Decreases leukocyte motility and phagocytosis in inflammatory responses.
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)
Treatment of infections caused by penicillinase-producing staphylococci. May use to initiate therapy when staphylococcal infection is suspected.
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)
Neutralizes circulating antibodies and down-regulates proinflammatory cytokines, including INF-gamma; blocks Fc receptors on macrophages; suppresses inducer T and B cells; augments suppressor T cells; blocks complement cascade.
Zone JJ, Taylor TB, Meyer LJ, Petersen MJ. The 97 kDa linear IgA bullous disease antigen is identical to a portion of the extracellular domain of the 180 kDa bullous pemphigoid antigen, BPAg2. J Invest Dermatol. Mar 1998;110(3):207-10. [Medline].
Bernard P, Vaillant L, Labeille B, et al. Incidence and distribution of subepidermal autoimmune bullous skin diseases in three French regions. Bullous Diseases French Study Group. Arch Dermatol. Jan 1995;131(1):48-52. [Medline].
Kharfi M, Khaled A, Karaa A, Zaraa I, Fazaa B, Kamoun MR. Linear IgA bullous dermatosis: the more frequent bullous dermatosis of children. Dermatol Online J. Jan 15 2010;16(1):2. [Medline].
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. Nov 1988;19(5 Pt 1):792-805. [Medline].
Collier PM, Kelly SE, Wojnarowska F. Linear IgA disease and pregnancy. J Am Acad Dermatol. Mar 1994;30(3):407-11. [Medline].
Billet SE, Kortuem KR, Gibson LE, El-Azhary R. A morbilliform variant of vancomycin-induced linear IgA bullous dermatosis. Arch Dermatol. Jun 2008;144(6):774-8. [Medline].
Chan LS, Regezi JA, Cooper KD. Oral manifestations of linear IgA disease. J Am Acad Dermatol. Feb 1990;22(2 Pt 2):362-5. [Medline].
Aultbrinker EA, Starr MB, Donnenfeld ED. Linear IgA disease. The ocular manifestations. Ophthalmology. Mar 1988;95(3):340-3. [Medline].
Nousari HC, Kimyai-Asadi A, Caeiro JP, Anhalt GJ. Clinical, demographic, and immunohistologic features of vancomycin-induced linear IgA bullous disease of the skin. Report of 2 cases and review of the literature. Medicine (Baltimore). Jan 1999;78(1):1-8. [Medline].
Alberta-Wszolek L, Mousette AM, Mahalingam M, Levin NA. Linear IgA bullous dermatosis following influenza vaccination. Dermatol Online J. Nov 15 2009;15(11):3. [Medline].
Yhim HY, Kwon DH, Lee NR, Song EK, Yim CY, Kwak JY. Linear IgA bullous dermatosis following autologous PBSC transplantation in a patient with non-Hodgkin's lymphoma. Bone Marrow Transplant. Apr 12 2010;[Medline].
Godfrey K, Wojnarowska F, Leonard J. Linear IgA disease of adults: association with lymphoproliferative malignancy and possible role of other triggering factors. Br J Dermatol. Oct 1990;123(4):447-52. [Medline].
Krejci-Manwaring J, West DA, Tonkovic-Capin V. If at first you don't succeed: a difficult case of Linear IgA. Dermatol Online J. Sep 15 2009;15(9):16. [Medline].
Jablonska S, Chorzelski TP, Rosinska D, Maciejowska E. Linear IgA bullous dermatosis of childhood (chronic bullous dermatosis of childhood). Clin Dermatol. Jul-Sep 1991;9(3):393-401. [Medline].
Klein PA, Callen JP. Drug-induced linear IgA bullous dermatosis after vancomycin discontinuance in a patient with renal insufficiency. J Am Acad Dermatol. Feb 2000;42(2 Pt 2):316-23. [Medline].
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