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