Medical Dermatology Society

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1 A Mashup of Instructive Cases Presenting With Subacute Cutaneous LE (SCLE) Skin Lesions Medical Dermatology Society Denver, Colorado March 20, 2014 Rick Sontheimer, M.D. Professor of Dermatology University of Utah School of Medicine

2 Potential Conflicts of Interest March, 2014 Consultant Paid speaker Centocor (Remicadeinfliximab) Plaquenil Winthrop (Sanofi) Genentech (Raptivaefalizumab) Amgen (etanercept-enbrel) (hydroxychloroquine) Alexion (eculizumab) Connetics/Stiefel MediQuest Therapeutics Royalties P&G (ChelaDerm) Lippincott, Celgene Williams Sanofi & Wilkins Research collaboration 3Gen, LLT

3 Gilliam JN:: The cutaneous signs of lupus erythematosus. Continuing Education for the Family Physician 1977; 6:34 Subacute cutaneous lupus erythematosus

4 38 yrs of SCLE

5 The First Two Decades: Dallas Prognostic significance of SCLE lesional morphology and anatomic distribution SCLE and pseudolupus SjS complicating SCLE

6 SCLE Papulosquamous (Psoriasiform Array) Annular (Polycyclic array)

7 Risk of Systemically- Significant SLE in SCLE <

8 Relative Sparing of Central Face

9 Risk Factors SCLE Clinically-Significant SLE (?) Papulosquamous/ psoriasiform morphology Central facial involvement (ACLE) Resistance to antimalarials Leukopenia High titer ANA Circulating dsdna antibodies

10 SCLE and Pseudolupus Dallas 1990s

11 Greenwald s Law of Lupus Greenwald RA. Greenwald's law of lupus. J Rheumatol Sep;19(9):1490. anything happening to a patient with SLE which is not immediately otherwise explicable will automatically be blamed on the lupus, regardless of pathophysiologic validity."

12 Greenwald s Law of Lupus

13 LE DM

14 Greenwald s Law of Lupus

15 Norwegian Scabies

16 Greenwald s Law of Lupus

17 Pediculosis Capitis

18 Lesson Learned Individuals with cutaneous lupus lesions are at risk for also contracting one or more of the other 1,999 skin disorders that are listed in our textbooks

19 Sjögren s Syndrome complicating patients presenting with SCLE skin lesions

20 HLA-B8,DR3 Anti-Ro/SS-A SCLE SjS SCLE/SjS Overlap (10-40%)

21 SCLE Patient 10 Years Later Cutaneous small vessel LCC vasculitis ~30% of primary SjS

22 SCLE/SjS Overlap Patient 3 Years Later Brought to clinic by pt s family on a stretcher. Too weak to standup.

23 SCLE/SjS Overlap Patient 3 Years Later Admission W/U Serum potassium 2.6 mmol/l Muscle weakness resolved quickly upon IV potassium repletion

24 SjS Interstitial Nephropathy

25 The 3 rd Decade: Iowa City & Oklahoma City Drug-induced SCLE

26 Drug-induced SCLE Reed et al at the Univ. of Colorado (Ann Intern Med 103:49 51) 5 patients with typical SCLE skin lesions developed while on hydrochlorothiazide (HCTZ) All 5 patients, the SCLE lesions resolved following HCTZ discontinuation

27

28 Systematic Review of Druginduced SCLE 71 reported cases (as of 2007) 47% - Cardiovascular disease (esp. anti-hbp) Epidemiology Demographics - Caucasian females, mean age yrs Incubation period - 2 weeks years Mean duration to clearance of lesions when drug was stopped weeks (1-24 weeks)

29 Immune modulators Antiepileptics Betablockers Chemotherapeutics Antihistamines Diuretics Antifungals Calcium channel blockers Percentage Fig. 1. Rates of DI-SCLE by drug class

30 Pantoprazole (Protonix)

31 24 patientslansoprazole (12), omeprazole (six), esomeprazole (four) and pantoprazole

32 The 4th Decade: Salt Lake City Pseudo SCLE still lives! Most SCLE patients that are referred to me now appear to be drug induced Recurrent tinea corporis in a man with terbinafine-induced SCLE was confused with reactivation of SCLE

33

34 Lesson Learned Antimalarial-resistant SCLE? Recurring SCLE Think drug-induced SCLE

35 Need of a Biomarker for Drug-Induced LE? Drug-induced T cell stimulation assay Experimental model Chronic eczematous drug eruption of the aged

36 C E L The Next 35 Years?

37 The End

38

39 For Questions & Comments

40 Thank You

41 For questions

42 The End The End

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44

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46 Skin Disease in LE Patients LE-Specific Skin Disease Acute Cutaneous LE Subacute cutaneous LE Chronic Cutaneous LE LE-Nonspecific Skin Disease LCV Urticarial vasculitis Livedo reticularis Secondary to Treatment of LE Steroid acne Drug eruptions Opportunistic Infections True-True and Unrelated Psoriasis Eczema BCC

47 The 2 nd Decade: Dallas & Iowa City Risk for clinically-significant SLE in patients presenting with papulosquamous SCLE skin lesions with central facial involvement Greenwald s Law of Lupus Norwegian scabies; head lice Beware SjS complications in SCLE patients

48 Skin Findings in SjS

49 Learning Objective Present a series of SCLE cases that illustrate salient clinical points of understanding that I have personally gained over the past 4 decades of caring for patients with this form of cutaneous LE

50 LE-Specific Skin Disease Acute Cutaneous LE Subacute cutaneous LE Chronic Cutaneous LE LE-Nonspecific Skin Disease LCV Urticarial vasculitis Livedo reticularis Secondary to Treatment of LE Steroid acne Drug eruptions Opportunistic Infections True-True and Unrelated Psoriasis Eczema BCC Greenwald s Law of Lupus