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Skin Signs of Rheumatic Disease Gideon P Skin Signs of Rheumatic Disease Gideon P. Smith MD PhD MPH Vice Chair for Clinical Affairs Director of Rheumatology-Dermatology Program Director of Connective Tissue Diseases Fellowship Associate Director of Clinical Trials Department of Dermatology Massachusetts General Hospital Harvard University www.mghcme.org Disclosures “Neither I nor my spouse/partner has a relevant financial relationship with a commercial interest to disclose.” www.mghcme.org CONNECTIVE TISSUE DISEASES CLINIC •Schnitzlers •Interstitial •Chondrosarcoma •Eosinophilic Fasciitis Granulomatous induced •Silicone granulomas Dermatitis with Dermatomyositis Arthritis •AML arthritis with •Scleroderma granulomatous papules •Cutaneous Crohn’s •Lyme arthritis with with arthritis •Follicular mucinosis in papular mucinosis JRA post-infliximab •Acral Anetoderma •Celiac Lupus •Calcinosis, small and •Granulomatous exophytic •TNF-alpha induced Mastitis sarcoid •NSF, Morphea •IgG4 Disease •Multicentric Reticul •EED, PAN, DLE ohistiocytosis www.mghcme.org • Primary skin disease recalcitrant to therapy Common consults • Hair loss • Nail dystrophy • Photosensitivity • Cosmetic concerns – post- inflammatory pigmentation, scarring, volume loss, premature photo-aging • Erythromelalgia • Dry Eyes • Dry Mouth • Oral Ulcerations • Burning Mouth Syndrome • Urticaria • Itch • Raynaud’s • Digital Ulceration • Calcinosis cutis www.mghcme.org Todays Agenda Clinical Presentations Rashes (Cutaneous Lupus vs Dermatomyositis vs ?) Hard Skin (Scleroderma vs Other sclerosing disorders) www.mghcme.org Case 1: Is this Lupus? www.mghcme.org Common Mimickers www.mghcme.org Common Mimickers www.mghcme.org Contact Dermatitis www.mghcme.org Contact Dermatitis exudative geometric ID reaction www.mghcme.org Common Mimickers www.mghcme.org Seborrheic Dermatitis • Greasy scale, often nasolabial fold prominence • Sternum, under arms, inguinal folds www.mghcme.org Common Mimickers www.mghcme.org Rosacea www.mghcme.org Common Mimickers www.mghcme.org Dermatomyositis www.mghcme.org Dermatomyositis vs Lupus • A lot of similarities – Photosensitive – Often Facial involvement – +/- ANA – +/- systemic symptoms www.mghcme.org Dermatomyositis: A Clinicopathological Study of 40 Patients Smith, Edward S MD*; Hallman, James R MD†; DeLuca, Amena M BS‡; Goldenberg, Gary MD§; Jorizzo, Joseph L MD*; Sangueza, Omar P MD†American Journal of Dermatopathology: February 2009 - Volume 31 - Issue 1 - pp 61-67 • Ten biopsy specimens each of DM and SLE (matched for anatomical site and lesion morphology) were randomized. • Blinded histopathologic diagnosis (DM versus SLE) by expert academic dermatopathologists • The correct histopathologic diagnosis of DM or SLE was made in 11 of the 20 skin biopsies without clinical information. www.mghcme.org So what are the Differences? Lupus Dermatomyositis www.mghcme.org Acute Cutaneous Lupus www.mghcme.org Malar Rash www.mghcme.org Malar Rash www.mghcme.org www.mghcme.org Malar Rash www.mghcme.org www.mghcme.org www.mghcme.org Any other cutaneous clues? www.mghcme.org www.mghcme.org www.mghcme.org www.mghcme.org www.mghcme.org www.mghcme.org www.mghcme.org Nailfold Capillaroscopy www.mghcme.org Nailfold Capillaroscopy www.mghcme.org Nailfold Capillaroscopy www.mghcme.org Nailfold Capillaroscopy www.mghcme.org Lupus Dermatomyositis Malar rash spares nasolabial folds Facial Rash enters nasolabial folds Rare calcinosis Microcalcifications actually common Nailfolds largely normal Capillaries often chaotic re-angiogenesis www.mghcme.org CASE 2 www.mghcme.org SCLE • Annular • Psoriasiform www.mghcme.org SCLE www.mghcme.org Dermatomyositis www.mghcme.org Not much scale; Ruddy; Telangiectasias www.mghcme.org Dermatomyositis www.mghcme.org Dermatomyositis www.mghcme.org Lupus Dermatomyositis Malar rash spares nasolabial folds Facial Rash enters nasolabial folds Rare calcinosis Microcalcifications actually common Nailfolds largely normal Capillaries often chaotic re-angiogenesis Hand rash between joints Hand rash joints Rash light pink Rash darker due to capillary component Malar and photodistributed Scalp, eyelids, hips, back www.mghcme.org Photosensitivity SLE vs DM Goreshi R, Chock M, Foering K, Feng R, Okawa J, Rose M, et al. Quality of life in dermatomyositis. J Am Acad Dermatol. 2011;65(6):1107-16. www.mghcme.org Pruritus SLE vs DM Goreshi R, Chock M, Foering K, Feng R, Okawa J, Rose M, et al. Quality of life in dermatomyositis. J Am Acad Dermatol. 2011;65(6):1107-16. www.mghcme.org Pruritus SLE vs DM Goreshi R, Chock M, Foering K, Feng R, Okawa J, Rose M, et al. Quality of life in dermatomyositis. J Am Acad Dermatol. 2011;65(6):1107-16. www.mghcme.org Pruritus SLE vs DM Goreshi R, Chock M, Foering K, Feng R, Okawa J, Rose M, et al. Quality of life in dermatomyositis. J Am Acad Dermatol. 2011;65(6):1107-16. www.mghcme.org Lupus Dermatomyositis Malar rash spares nasolabial folds Facial Rash enters nasolabial folds Rare calcinosis Microcalcifications actually common Nailfolds largely normal Capillaries often chaotic re-angiogenesis Hand rash between joints Hand rash joints Rash light pink Rash darker due to capillary component Malar and photodistributed Scalp, eyelids, hips, back Pain/burning > itch Itch > pain www.mghcme.org Hair www.mghcme.org Lupus Hairs www.mghcme.org www.mghcme.org PASTE • P – plugging • A – Atrophy • S – Scale • T – Telangiectasias • E – Erythema www.mghcme.org www.mghcme.org Dermoscopy DLE www.mghcme.org Dermoscopy DLE www.mghcme.org Lupus Dermatomyositis Malar rash spares nasolabial folds Facial Rash enters nasolabial folds Rare calcinosis Microcalcifications actually common Nailfolds largely normal Capillaries often chaotic re-angiogenesis Hand rash between joints Hand rash joints Rash light pink Rash darker due to capillary component Malar and photodistributed Scalp, eyelids, hips, back Pain/burning > itch Itch > pain Hairloss patchy Hairloss diffuse www.mghcme.org Summary Differences Lupus Dermatomyositis Malar rash spares nasolabial folds Facial Rash enters nasolabial folds Rare calcinosis Microcalcifications actually common Nailfolds largely normal Capillaries often chaotic re-angiogenesis Hand rash between joints Hand rash joints Rash light pink Rash darker due to capillary component Malar and photodistributed Scalp, eyelids, hips, back Pain/burning > itch Itch > pain Hairloss patchy Hairloss diffuse www.mghcme.org Is it scleroderma? www.mghcme.org RAYNAUD’S ‘WHITE’ www.mghcme.org RAYNAUD’S ‘BLUE’ www.mghcme.org NOTE DISTRIBUTION lSSc dSSc www.mghcme.org Puffy Fingers of dSSc www.mghcme.org Puffy Fingers of dSSc www.mghcme.org Nailfold Capillaroscopy www.mghcme.org Nailfold Capillaroscopy www.mghcme.org www.mghcme.org Matted Telangiectasias • More often in patients with limited disease/CREST • Common on cheek, lips www.mghcme.org Matted Telangiectasias • Common on palms www.mghcme.org Pterygium Inversum www.mghcme.org Note Distribution Morphea LS and A www.mghcme.org NOTE DISTRIBUTION Morphea LS and A www.mghcme.org Clinical Features: Plaque Type • Most common variant • peripheral violaceous ‘lilac’ ring www.mghcme.org Clinical Features: Plaque Type • Central area transforms into sclerotic, shiny white tissue www.mghcme.org Clinical Features: Plaque Type • Central area transforms into sclerotic, shiny white tissue www.mghcme.org Clinical Features: Plaque Type • Once burnt out post- inflammatory hyperpigmentation common over sclerosis • Hair and sweat glands frequently lost • Pruritus from xeroderma www.mghcme.org Clinical Features: Plaque Type • Once burnt out post-inflammatory hyperpigmentation common over sclerosis www.mghcme.org Can Koebnerize Can koebnerize into areas of friction or other inflammator y disorders eg eczema as here www.mghcme.org Can Koebnerize Can koebnerize into areas of friction or other inflammator y disorders eg eczema as here www.mghcme.org Variation in Appearance • Can look very different in different skin types • Still with loss of adnexal structures (no hair, dryer, more PIH) www.mghcme.org Variation in Appearance • Can look very different in different skin types • Still with loss of adnexal structures (no hair, dryer, more PIH) www.mghcme.org NOTE DISTRIBUTION Scleredema Early Scleromyxedema www.mghcme.org NOTE DISTRIBUTION Scleredema Scleromyxedema www.mghcme.org NOTE DISTRIBUTION Eosinophilic Nephrogenic Systemic Fasciitis Fibrosis www.mghcme.org Clinical Appearance www.mghcme.org Clinical Appearance www.mghcme.org Clinical Appearance Arm www.mghcme.org “Groove sign”: linear depression overlying vein www.mghcme.org “Groove sign”: linear depression overlying vein www.mghcme.org Symmetric and spares hands, feet, face www.mghcme.org When Thinking Sclerosis Disorders • LOOK AT THE HANDS – Sclerosis or Puffiness? – True Raynaud’s? – Capillary Changes? – Telangiectasias? – Calcinosis – Pterygium Inversa? • LOOK AT THE DISTRIBUTION www.mghcme.org Thank you! www.mghcme.org.
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