1. Grand Rounds Conference Lara Rosenwasser Newman, MD University of Louisville Department of Ophthalmology and Visual Sciences December 5, 2014

2. Subjective CC: Red eyes and eyelids, eyes burning x 2 days HPI: 51 yo WM, inpt for dyspnea, dysphagia, who began to have burning in his eyes, red eyes, and redness and burning of the skin around the eyes, progressively worsening over 2 days. Had upper endoscopy 2 days prior, awaiting path. Felt that placing cold washcloth over eyes helped. Primary team had placed on cipro gtts x1 day.

3. History PMHx: Barrett’s esophagus, esophageal cancer, COPD Barrett’s esophagus, esophageal cancer, COPD per pt Hep C treated w/IFN, in remission since 2004 or 2005 per pt Hep C treated w/IFN, in remission since 2004 or 2005PSHx: Multiple upper endoscopies, Nissen fundoplication Multiple upper endoscopies, Nissen fundoplicationPOHx: Presbyopia PresbyopiaMedications: At home: Paxil, Prilosec, Singulair, albuterol inhaler At home: Paxil, Prilosec, Singulair, albuterol inhaler

4. History Continued Medications as inpatient: Medications as inpatient: Dilaudid, morphine, Zofran Dilaudid, morphine, Zofran 1x doses atropine/hyoscyamine/PB/scopolamine, GI cocktail, hydroxide/Mg hydroxide/simethicone 1x doses atropine/hyoscyamine/PB/scopolamine, GI cocktail, hydroxide/Mg hydroxide/simethicone Lovenox, hydromorphone, lidocaine morphine Lovenox, hydromorphone, lidocaine morphine Pantoprazole, Paxil Pantoprazole, Paxil Cipro 2 gtts OU q4h while awake Cipro 2 gtts OU q4h while awake Rocephin 1g daily Rocephin 1g daily Flu shot Flu shot

5. IOP (tonopen):18mmHg18mmHg EOM: Clinical Exam OD OS OD OS VA (near, +2.00s): 20/2520/20 Pupils: 2.5  1.5 2.5  1.5 000 0 (-) rAPD 000 0

6. Clinical Exam PLE: External/LidsErythema lower>upper lids, red-racoon- eyes appearance, skin rough/sandpapery, mild edema, not indurated Conjunctiva/ScleraSevere injected OU, did not blanche w/ phenylephrine, mucoid discharge Corneadiffuse fine SPEs on fluorescein Anterior ChamberFormed OU IrisNormal OU LensClear OU VitreousNormal OU DFE: ON pink & sharp OU, M/V/P WNL

7. External Appearance

8. Approx 1 cm excoriated plaque on posterior left neck, no other skin lesions Approx 1 cm excoriated plaque on posterior left neck, no other skin lesions Afebrile, stable vitals Afebrile, stable vitals Mucoid to purulent appearing discharge, sent for aerobic and anaerobic cultures Mucoid to purulent appearing discharge, sent for aerobic and anaerobic cultures Final results: negative Final results: negative Exam continued

9. Assessment 51 yo WM w/dysphagia, chest pain, dyspnea, presenting with acute dermatoblepharoconjunctivitis. 51 yo WM w/dysphagia, chest pain, dyspnea, presenting with acute dermatoblepharoconjunctivitis.

10. Differential Considerations Contact dermatoconjunctivitis Contact dermatoconjunctivitis Detergent hospital uses for washcloths? Detergent hospital uses for washcloths? Dermatomyositis Dermatomyositis Preseptal cellulitis Preseptal cellulitis

11. Plan Discontinue Cipro gtts Discontinue Cipro gtts Aggressive lubrication w/preservative-free artificial tears q2-4 hrs Aggressive lubrication w/preservative-free artificial tears q2-4 hrs Follow-up cultures (negative) Follow-up cultures (negative)

12. Follow-up Resolved over next several days Resolved over next several days Pt was discharged days later Pt was discharged days later Surg path from upper endoscopy: Barrett’s, otherwise benign Surg path from upper endoscopy: Barrett’s, otherwise benign Symptoms attributed to gastroparesis, gastritis/esophagitis Symptoms attributed to gastroparesis, gastritis/esophagitis Now in hospital again for dyspnea, dysphagia, and chest pain Now in hospital again for dyspnea, dysphagia, and chest pain

13. Review of 215 pts who presented for eyelid dermatitis in a 42 month period Review of 215 pts who presented for eyelid dermatitis in a 42 month period 165 allergic contact dermatitis (personal care products, metals) 165 allergic contact dermatitis (personal care products, metals) 9 protein contact dermatitis (no positive patch test) 9 protein contact dermatitis (no positive patch test) 35 atopic eczema (33 of these also had contact allergies) 35 atopic eczema (33 of these also had contact allergies) 35 psoriasis or seborrheic dermatitis or both 35 psoriasis or seborrheic dermatitis or both 5 rosacea or periorbital dermatitis 5 rosacea or periorbital dermatitis 2 dermatomyositis 2 dermatomyositis Guin JD. Eyelid dermatitis: a report of 215 patients. Contact Dermatitis. 2004 Feb;50(2):87-90. PubMed PMID: 15128319.

14. Contact dermatitis Most common eruption of eyelid Most common eruption of eyelid Can involve lids & eyes Can involve lids & eyes Unilateral or symmetrical Unilateral or symmetrical Pruritic, scaling erythematous eruption of lid(s) Pruritic, scaling erythematous eruption of lid(s) May see periorbital edema, blepharitis, conjunctivitis May see periorbital edema, blepharitis, conjunctivitis Watery discharge, papillary or follicular conj rxn Watery discharge, papillary or follicular conj rxn Allergic (pruritus) or irritant (burning/stinging) Allergic (pruritus) or irritant (burning/stinging) Can be very difficult to distinguish between Can be very difficult to distinguish between 3 top causes: cosmetics, topical ophthalmic meds, CL solutions 3 top causes: cosmetics, topical ophthalmic meds, CL solutions

15. Provocative substances Drugs Drugs Cosmetics/personal care products Cosmetics/personal care products including nail polish, hand soap including nail polish, hand soap Preservatives Preservatives Dyes Dyes Plant resins Plant resins Heavy metals Heavy metals Plastic or nickel in glasses Plastic or nickel in glasses

16. Contact dermatitis Dx: patch testing, clinical picture Dx: patch testing, clinical picture Tx: identify/eliminate offending allergen/irritant Tx: identify/eliminate offending allergen/irritant Cool compresses Cool compresses Topical corticosteroids Topical corticosteroids Oral antihistamines Oral antihistamines

17. Contact Dermatitis

18. Dermatomyositis Systemic vascular disorder Systemic vascular disorder Skin & muscle inflammation, acute or insidious Skin & muscle inflammation, acute or insidious Atonic, weak, achy proximal muscle groups Atonic, weak, achy proximal muscle groups Gottron’s papules = diagnostic Gottron’s papules = diagnostic Flat-topped erythematous papules over knuckles Flat-topped erythematous papules over knuckles Scaly areas on backs of hands, knuckles, elbows, knees, and nail changes (shininess, erythema) Scaly areas on backs of hands, knuckles, elbows, knees, and nail changes (shininess, erythema) Telangiectasia, skin rash in malar region, neck, shoulders, upper chest, and back Telangiectasia, skin rash in malar region, neck, shoulders, upper chest, and back Assoc w/breast, ovary, lung, pancreas, stomach, colon, rectum CA & NHL (18-32% of DM pts) Assoc w/breast, ovary, lung, pancreas, stomach, colon, rectum CA & NHL (18-32% of DM pts) Can have GI & respiratory involvement Can have GI & respiratory involvement

19. Dermatomyositis Etiology: unknown Etiology: unknown Genetic susceptibility + exposure to environmental agents or cancers  immune activation/inflam Genetic susceptibility + exposure to environmental agents or cancers  immune activation/inflam Injury to capillaries & myofibers Injury to capillaries & myofibers 2 theories: 2 theories: Induction of type 1 IFN-inducible gene products Induction of type 1 IFN-inducible gene products Antibody & complement-mediated microangiopathy Antibody & complement-mediated microangiopathy AutoAbs incl myositis-specific Abs (MSAs) AutoAbs incl myositis-specific Abs (MSAs) Can be precipitated/caused by penicillins, sulfonamides, and D-penicillamine Can be precipitated/caused by penicillins, sulfonamides, and D-penicillamine

20. Dermatomyositis Dx: muscle biopsy Dx: muscle biopsy Labs: Labs: High transaminases, CK, aldolase, LDH High transaminases, CK, aldolase, LDH sometimes (+) ANA, anti-Jo-1, anti-Mi-2, RF sometimes (+) ANA, anti-Jo-1, anti-Mi-2, RF Tx: systemic corticosteroids, usually w/satisfactory response in classic DMS Tx: systemic corticosteroids, usually w/satisfactory response in classic DMS Less so in pts w/anti-Jo Abs Less so in pts w/anti-Jo Abs If steroids fail, cytotoxic agents (MTX, azathioprine) and/or IVIG If steroids fail, cytotoxic agents (MTX, azathioprine) and/or IVIG

21. Dermatomyositis Features Credit: Ostler, HB, Maibach, HI, Hoke, AW, and Schwab, IR. Diseases of the Eye & Skin: A Color Atlas. Philadelphia, PA; Lippincott Williams & Wilkins, 2004: 14-19 and 112-122.

22. DM – Ocular Findings Heliotrope telangiectasias of eyelids = characteristic Heliotrope telangiectasias of eyelids = characteristic CONJ CHEMOSIS = COMMON CONJ CHEMOSIS = COMMON Can cause nonspecific conjunctivitis, rarely pseudomembranous conjunctivitis Can cause nonspecific conjunctivitis, rarely pseudomembranous conjunctivitis

24. Dermatomyositis Features Credit: Ostler, HB, Maibach, HI, Hoke, AW, and Schwab, IR. Diseases of the Eye & Skin: A Color Atlas. Philadelphia, PA; Lippincott Williams & Wilkins, 2004: 14-19 and 112-122.

25. Dermatomyositis Features Credit: Mannis, MJ, Macsai, MS, Huntley, AC. Eye and Skin Disease. Philadelphia, PA; Lippincott-Raven Publishers, 1996: 233-238.

26. DM – Ocular Findings Nonspecific episcleritis or scleritis Nonspecific episcleritis or scleritis Exophthalmos Exophthalmos Anterior uveitis Anterior uveitis Retinopathy w/cotton wool spots Retinopathy w/cotton wool spots Late sequelae of pigmentary maculopathy & optic atrophy Late sequelae of pigmentary maculopathy & optic atrophy EOM paralysis and nystagmus EOM paralysis and nystagmus Rare but important: orbital polymyositis or ocular myositis assoc w/giant-cell myocarditis Rare but important: orbital polymyositis or ocular myositis assoc w/giant-cell myocarditis

27. References 1. Park IK, Chun YS, Kim KG, Yang HK, Hwang JM. New clinical grading scales and objective measurement for conjunctival injection. Invest Ophthalmol Vis Sci. 2013 Aug 5;54(8):5249- 57. doi: 10.1167/iovs.12-10678. PubMed PMID: 23833063. 2. 2. Ostler, HB, Maibach, HI, Hoke, AW, and Schwab, IR. Diseases of the Eye & Skin: A Color Atlas. Philadelphia, PA; Lippincott Williams & Wilkins, 2004: 14-19 and 112-122. 3. 3. Mannis, MJ, Macsai, MS, Huntley, AC. Eye and Skin Disease. Philadelphia, PA; Lippincott- Raven Publishers, 1996: 233-238. 4. Guin JD. Eyelid dermatitis: a report of 215 patients. Contact Dermatitis. 2004 Feb;50(2):87- 90. PubMed PMID: 15128319. 5. Ebert EC. Review article: the gastrointestinal complications of myositis. Aliment Pharmacol Ther. 2010 Feb 1;31(3):359-65. doi: 10.1111/j.1365-2036.2009.04190.x. Epub 2009 Nov 3. Review. PubMed PMID: 19886949. 6. Iaccarino L, Ghirardello A, Bettio S, Zen M, Gatto M, Punzi L, Doria A. The clinical features, diagnosis and classification of dermatomyositis. J Autoimmun. 2014 Feb-Mar;48-49:122-7. doi: 10.1016/j.jaut.2013.11.005. Epub 2014 Jan 24. Review. PubMed PMID: 24467910.

28. Dermatomyositis: Resp & GI Dysphagia from involvement of muscles of tongue, pharynx, & upper 1/3 of esophagus Dysphagia from involvement of muscles of tongue, pharynx, & upper 1/3 of esophagus Can get dysphagia for liquids and solids Can get dysphagia for liquids and solids Pharyngeal and upper esophageal involvement can cause asphyxiation and/or aspiration Pharyngeal and upper esophageal involvement can cause asphyxiation and/or aspiration Nasal regurgitation  characteristic nasal voice Nasal regurgitation  characteristic nasal voice Muscles of respiration and myocardium may also be affected Muscles of respiration and myocardium may also be affected

29. Dermatomyositis can be associated with: Dermatomyositis can be associated with: Crohn’s/UC (IBD) Crohn’s/UC (IBD) Celiac (may respond to gluten-free diet) Celiac (may respond to gluten-free diet) Hep C virus Hep C virus Primary biliary cirrhosis Primary biliary cirrhosis Can develop during IFN tx of HCV Can develop during IFN tx of HCV Usually resolves w/discontinuation of IFN Usually resolves w/discontinuation of IFN Myopathies can occur during tx w/PPIs Myopathies can occur during tx w/PPIs Mentions polymyositis & rhabdo Mentions polymyositis & rhabdo