Systemic Approaches to Granulomatous Disorders S001...Systemic Approaches to Granulomatous Disorders . Conflicts of Interest • Celgene ... Necrobiotic Xanthogranuloma
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Misha Rosenbach, MD Assistant Professor, Dermatology & Internal Medicine
Director, Inpatient Consult Service Director, Cutaneous Sarcoidosis Clinic
Systemic Approaches to Granulomatous Disorders
Conflicts of Interest • Celgene - Advisory Board
• Off label uses of medications will be discussed (everything
is off label)
Antimalarials
Methotrexate
TNF inhibitors
Topicals, Intralesional Phototherapy
Dapsone Retinoids
Case reported random
assortment of treatments
Tetracycline antibiotics
GIVE EVERY
THERAPY AT LEAST
THREE MONTHS TO WORK
Sarcoidosis
Wanat KA, Rosenbach M. A practical approach to cutaneous sarcoidosis. Am J Clin Dermatol 2014 Wanat KA, Rosenbach M. Cutaneous Sarciodosis. Clin Chest Med (In Press)
Cutaneous Sarcoidosis Treatment Algorithm
Topicals
Intralesional
SEVERITY
Antimalarials
Minocycline
Methotrexate
Thalidomide
Adalimumab
Infliximab
Wanat KA, Rosenbach M. Am J Clin Dermatol 2014
Granuloma Annulare
Piette E, Rosenbach M. Granuloma Annulare: CME. JAAD (In Press) Keimig EL. Granuloma Annulare. Dermatol Clin 2015 Thornsberry LA, English JC 3rd. Am J Clin Dermatol 2013
Antimalarials • Hydroxychloroquine 400mg • Chloroquine 250mg
First Line:
If persistent lesions after 3 months, switch
Phototherapy • PUVA 3x/week • NBUVB 3x/wk
Consider alternative systemic agents
Piette E, Rosenbach M. Granuloma Annulare: CME. JAAD (In Press)
Second Line:
GA: evidence-based treatment algorithm
• 1980-2013 review of GA treatment • 100 studies • Average # of patients: 3, median is 1 • Only studies with double digits:
Phototherapy
• Largest studies in history: • Phototherapy • Antimalarials
Necrobiosis Lipoidica
Sibbald C, Reid S, Alavi A. Necrobiosis Lipoidica. Dermatol Clin 2015 Wanat KA, Rosenbach M. Necrobiosis Lipoidica. UpToDate
Inject, excise
Colchicine Antimalarials
Dapsone Allopurinol
Consider accelerated nodulosis –
stop offending
agent (MTX, other)
Necrobiotic Xanthogranuloma
Kerstetter J, Wang J. Adult Orbital Xanthogranulomatous Disease. Dermatol Clin 2015
Necrobiotic Xanthogranuloma • Rare non-Langerhans cell histiocytosis • Generally around the eyes • Characteristic violaceous and xanthomatous
plaques, may ulcerate • Extracutaneous disease: heart, eyes, various organ
systems • Strongly associated with paraproteinemia,
especially IgGk
Treatment options • Limited data • NXG with myeloma: treat myeloma • NXG without myeloma:
• Chemo (chlroambucil, melphalan, +/- steroids) • IFN-alpha • AZA, MTX, Thalidomide, Dapsone, Clofazamine,
TCNs – Local: XRT, excision, intralesional steroids – Newer:
• IVIg (6 cases since 2010)1
• Topical nitrogen mustard2 1. Hallermann C, Arch Dermatol 2010 2. Rodriguez O, JAMA Dermatol 2016
Antimalarials Hydroxychloroquine safer Chloroquine works better
Methotrexate 15-20mg weekly
TNF inhibitors Higher dose/Frequency than
psoriasis
Phototherapy: PUVA is Best
Dapsone Retinoids
TCN Abx: Minocycline is
Best
THREE MONTHS TO
ASSESS EFFICACY
Key References All • Clinical Atlas of Granulomatous Disorders. English JC 3rd. Dermatol Clin 2015 GA • Piette E, Rosenbach M. Granuloma Annulare: CME. JAAD (In Press) • Keimig EL. Granuloma Annulare. Dermatol Clin 2015 NL • Sibbald C, Reid S, Alavi A. Necrobiosis Lipoidica. Dermatol Clin 2015 • Wanat KA, Rosenbach M. Necrobiosis Lipoidica. UpToDate NXG • Kerstetter J, Wang J. Adult Orbital Xanthogranulomatous Disease. Dermatol Clin 2015 Sarcoidosis • Wanat KA, Rosenbach M. A practical approach to cutaneous sarcoidosis. Am J Clin
Dermatol 2014 • Wanat KA, Rosenbach M. Cutaneous Sarciodosis. Clin Chest Med (In Press)
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