Graham-Little-Piccardi-Lasseur Syndrome Medication

  • Author: Scott Richard Albert Walsh, MD, PhD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Jan 17, 2012
 

Medication Summary

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.

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Immunosuppressant agents

Class Summary

Interfere with immune processes that promote inflammation.

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Cyclosporine A (Sandimmune, Neoral)

 

An 11-amino acid cyclic peptide and natural product of fungi.

Acts on T-cell replication and activity. 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 G0 or G1 phase of cell cycle suggested.

Binds to cyclophilin, an intracellular protein, which, in turn, prevents formation of IL-2 and subsequent recruitment of activated T cells.

Has approximately 30% bioavailability, but marked interindividual variability is reported. Specifically inhibits T-lymphocyte function with minimal activity against B cells. Maximum suppression of T-lymphocyte proliferation requires that drug be present during first 24 h of antigenic exposure.

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.

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Thalidomide (Thalomid)

 

Immunomodulatory agent that may suppress excessive production of TNF-alpha and may down-regulate selected cell-surface adhesion molecules involved in leukocyte migration.

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Tacrolimus topical (Protopic)

 

Reduces itching and inflammation by suppressing release of cytokines from T cells. Also inhibits 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, may inhibit release of preformed mediators from skin mast cells and basophils and may down-regulate expression of FcERI on Langerhans cells. Can be used in patients as young as 2 y. Drugs of this class are more expensive than topical corticosteroids.

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Antibiotics

Class Summary

Empiric antimicrobial therapy must be comprehensive and should cover all likely pathogens in the context of the clinical setting.

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Metronidazole (Protostat, Flagyl)

 

Imidazole ring-based antibiotic active against various anaerobic bacteria and protozoa. Used in combination with other antimicrobial agents (except to treat Clostridium difficile enterocolitis).

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Corticosteroids

Class Summary

Have anti-inflammatory properties and cause profound and varied metabolic effects. Corticosteroids modify the body's immune response to diverse stimuli.

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Betamethasone dipropionate (Diprolene)

 

Class I superpotent topical steroid; suppresses mitosis and increases synthesis of proteins that decrease inflammation and cause vasoconstriction.

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Clobetasol (foam, spray, cream, ointment)

 

Class I superpotent topical steroid; suppresses mitosis and increases synthesis of proteins that decrease inflammation and cause vasoconstriction.

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Prednisone (Orasone, Meticorten, Sterapred, Deltasone)

 

May decrease inflammation by reversing increased capillary permeability and suppressing PMN activity.

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Contributor Information and Disclosures
Author

Scott Richard Albert Walsh, MD, PhD  Assistant Professor, Program Director, Department of Dermatology, University of Toronto, Sunnybrook Health Sciences Centre

Scott Richard Albert Walsh, MD, PhD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, Canadian Dermatology Association, International Society of Dermatology, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Coauthor(s)

Patricia T Ting, MD, MSc  Resident Physician, Division of Dermatology and Cutaneous Sciences, Department of Medicine, University of Alberta Faculty of Medicine and Dentistry, Canada

Patricia T Ting, MD, MSc is a member of the following medical societies: Alberta Medical Association, Canadian Dermatology Association, and Canadian Medical Association

Disclosure: Nothing to disclose.

Neil Shear, MD  Professor and Chief of Dermatology, Professor of Medicine, Pediatrics and Pharmacology, University of Toronto Faculty of Medicine; Head of Dermatology, Sunnybrook Women's College Health Sciences Center and Women's College Hospital, Canada

Neil Shear, MD is a member of the following medical societies: American Academy of Dermatology, American Society for Clinical Pharmacology and Therapeutics, Canadian Dermatology Association, Canadian Medical Association, Ontario Medical Association, and Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

Specialty Editor Board

Franklin Flowers, MD  Chief, Division of Dermatology, Professor, Department of Medicine and Otolaryngology, Affiliate Associate Professor of Pediatrics and Pathology, University of Florida College of Medicine

Franklin Flowers, MD, is a member of the following medical societies: American College of Mohs Micrographic Surgery and Cutaneous Oncology

Disclosure: Nothing to disclose.

David F Butler, MD  Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Director, Dermatology Residency Training Program, Scott and White Clinic, Northside Clinic

David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Association of Military Dermatologists, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Jeffrey J Miller, MD  Associate Professor of Dermatology, Pennsylvania State University College of Medicine; Staff Dermatologist, Pennsylvania State Milton S Hershey Medical Center

Jeffrey J Miller, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, Association of Professors of Dermatology, North American Hair Research Society, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Catherine M Quirk, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania

Catherine M Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD  Director, Ackerman Academy of Dermatopathology, New York

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

References

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Follicular lichen planus eruption.
Lichen planopilaris of the scalp resulting in cicatricial alopecia.
 
 
 
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