Graham-Little-Piccardi-Lasseur Syndrome Medication
- Author: Patricia T Ting, MD, MSc, FRCPC, LMCC(Canada); Chief Editor: Dirk M Elston, MD more...
Topical, intralesional, and systemic corticosteroids; retinoids; and PUVA have been used with limited success. In recent years, various case reports have documented successful treatment of Graham-Little-Piccardi-Lasseur syndrome (GLPLS) with cyclosporine A, thalidomide, and metronidazole (authors' observation). No definite treatments have been developed for GLPLS. Patients tend to be treated empirically for this condition.
Immunosuppressant agents interfere with the immune processes that promote inflammation.
Cyclosporine A is an 11-amino acid cyclic peptide and natural product of fungi. It acts on T-cell replication and activity. It is a specific modulator of T-cell function and an agent that depresses cell-mediated immune responses by inhibiting helper T-cell function. Preferential and reversible inhibition of T lymphocytes in the G0 or G1 phase of the cell cycle is suggested.
Cyclosporine A binds to cyclophilin, an intracellular protein, which, in turn, prevents the formation of IL-2 and subsequent recruitment of activated T cells.
Cyclosporine A has approximately 30% bioavailability, but marked interindividual variability is reported. It specifically inhibits T-lymphocyte function with minimal activity against B cells. Maximum suppression of T-lymphocyte proliferation requires that the drug be present during the first 24 hours of antigenic exposure.
Cyclosporine A suppresses some humoral immunity and, to a greater extent, cell-mediated immune reactions (eg, delayed hypersensitivity, allograft rejection, experimental allergic encephalomyelitis, graft vs host disease) for a variety of organs.
Thalidomide is an immunomodulatory agent that may suppress excessive production of TNF-alpha and may down-regulate selected cell-surface adhesion molecules involved in leukocyte migration.
Tacrolimus topical reduces itching and inflammation by suppressing the release of cytokines from T cells. It also inhibits the transcription of genes that encode IL-3, IL-4, IL-5, GM-CSF, and TNF-alpha, all of which are involved in the early stages of T-cell activation. Additionally, it may inhibit the release of preformed mediators from skin mast cells and basophils and may down-regulate the expression of FcERI on Langerhans cells. Tacrolimus topical can be used in patients as young as 2 years. Drugs of this class are more expensive than topical corticosteroids.
Empiric antimicrobial therapy must be comprehensive and should cover all likely pathogens in the context of the clinical setting.
Metronidazole is an imidazole ring-based antibiotic active against various anaerobic bacteria and protozoa. It is used in combination with other antimicrobial agents (except to treat Clostridium difficile enterocolitis).
Corticosteroids have anti-inflammatory properties and cause profound and varied metabolic effects. Corticosteroids modify the body's immune response to diverse stimuli.
Betamethasone dipropionate is a class I superpotent topical steroid; it suppresses mitosis and increases the synthesis of proteins that decrease inflammation and cause vasoconstriction.
Clobetasol is a class I superpotent topical steroid; it suppresses mitosis and increases the synthesis of proteins that decrease inflammation and cause vasoconstriction.
Prednisone may decrease inflammation by reversing increased capillary permeability and suppressing PMN activity.
Hydroxychloroquine inhibits chemotaxis of eosinophils and locomotion of neutrophils and impairs complement-dependent antigen-antibody reactions. Hydroxychloroquine sulfate 200 mg is equivalent to 155 mg hydroxychloroquine base and 250 mg chloroquine phosphate. Weight-based dose adjustment and monitoring help to mitigate the risk of retinal toxicity.
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