Systemic Approaches to Granulomatous Disorders S001...Systemic Approaches to Granulomatous Disorders . Conflicts of Interest • Celgene ... Necrobiotic Xanthogranuloma

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

Keimig EL. Dermatol Clin 2015

• 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

Min MS, Lebwohl M. JAAD 2016

Necrobiosis Lipoidica

Sibbald C, Reid S, Alavi A. Necrobiosis Lipoidica. Dermatol Clin 2015 Wanat KA, Rosenbach M. Necrobiosis Lipoidica. UpToDate

Steroids under occlusion

PUVA, Immunosuppressives / TNFs, Immunomodulatory drugs

Rheumatoid Nodules

Tilstra JS, Lienesch DW. Rheumatoid Nodules. Dermatol Clin 2015.

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

The Dermatology Foundation

has supported & advanced my career.

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)