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Skin Signs of Rheumatic DiseaseGideon P. Smith MD PhD MPH
Vice Chair for Clinical Affairs Director of Rheumatology-Dermatology ProgramDirector of Connective Tissue Diseases Fellowship
Associate Director of Clinical Trials Department of Dermatology
Massachusetts General HospitalHarvard University
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Disclosures
“Neither I nor my spouse/partner has a relevant financial relationship with a
commercial interest to disclose.”
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CONNECTIVE TISSUE DISEASES CLINIC
•Schnitzlers
•Eosinophilic Fasciitis
•Silicone granulomas
•AML arthritis with
granulomatous papules
•Follicular mucinosis in
JRA post-infliximab
•Calcinosis, small and
exophytic
•NSF, Morphea
•EED, PAN, DLE
•Interstitial
Granulomatous
Dermatitis with
Arthritis
•Cutaneous Crohn’s
with arthritis
•Acral Anetoderma
Lupus
•TNF-alpha induced
sarcoid
•Multicentric Reticul
ohistiocytosis
•Chondrosarcoma
induced
Dermatomyositis
•Scleroderma
•Lyme arthritis with
papular mucinosis
•Celiac
•Granulomatous
Mastitis
•IgG4 Disease
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Common consults• Primary skin disease recalcitrant to therapy
• 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
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Todays Agenda
Clinical Presentations
Rashes(Cutaneous Lupus vs Dermatomyositis vs ?)
Hard Skin(Scleroderma vs Other sclerosing disorders)
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Case 1: Is this Lupus?
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Common Mimickers
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Common Mimickers
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ContactDermatitis
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ContactDermatitis
exudativegeometricID reaction
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Common Mimickers
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Seborrheic Dermatitis
• Greasy scale, often nasolabial fold prominence
• Sternum, under arms, inguinal folds
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Common Mimickers
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Rosacea
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Common Mimickers
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Dermatomyositis
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Dermatomyositis vs Lupus
• A lot of similarities
– Photosensitive
– Often Facial involvement
– +/- ANA
– +/- systemic symptoms
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Dermatomyositis: A Clinicopathological Study of 40 PatientsSmith, 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.
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So what are the Differences?
Lupus Dermatomyositis
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Acute Cutaneous Lupus
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Malar Rash
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Malar Rash
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Malar Rash
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Any other cutaneous clues?
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Nailfold Capillaroscopy
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Nailfold Capillaroscopy
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Nailfold Capillaroscopy
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Nailfold Capillaroscopy
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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
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CASE 2
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SCLE
• Annular
• Psoriasiform
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SCLE
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Dermatomyositis
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Not much scale; Ruddy; Telangiectasias
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Dermatomyositis
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Dermatomyositis
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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
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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.
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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.
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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.
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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.
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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
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Hair
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Lupus Hairs
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PASTE
• P – plugging
• A – Atrophy
• S – Scale
• T – Telangiectasias
• E – Erythema
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Dermoscopy DLE
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Dermoscopy DLE
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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
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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
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Is it scleroderma?
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RAYNAUD’S ‘WHITE’
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RAYNAUD’S ‘BLUE’
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lSSc dSSc
NOTE DISTRIBUTION
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Puffy Fingers of dSSc
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Puffy Fingers of dSSc
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Nailfold Capillaroscopy
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Nailfold Capillaroscopy
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Matted Telangiectasias
• More often in patients with limited disease/CREST
• Common on cheek, lips
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Matted Telangiectasias
• Common on palms
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Pterygium Inversum
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Note Distribution
Morphea LS and A
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Morphea LS and A
NOTE DISTRIBUTION
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Clinical Features: Plaque Type
• Most common variant
• peripheral violaceous ‘lilac’ ring
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Clinical Features: Plaque Type
• Central area transforms into sclerotic, shiny white tissue
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Clinical Features: Plaque Type
• Central area transforms into sclerotic, shiny white tissue
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Clinical Features: Plaque Type
• Once burnt out post-inflammatory hyperpigmentation common over sclerosis
• Hair and sweat glands frequently lost
• Pruritus from xeroderma
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Clinical Features: Plaque Type
• Once burnt out post-inflammatory hyperpigmentation common over sclerosis
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Can Koebnerize
Can koebnerize into areas of friction or other inflammatory disorders eg eczema as here
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Can koebnerize into areas of friction or other inflammatory disorders eg eczema as here
Can Koebnerize
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Variation in Appearance
• Can look very different in different skin types
• Still with loss of adnexal structures (no hair, dryer, more PIH)
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Variation in Appearance
• Can look very different in different skin types
• Still with loss of adnexal structures (no hair, dryer, more PIH)
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Scleredema Early Scleromyxedema
NOTE DISTRIBUTION
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Scleredema Scleromyxedema
NOTE DISTRIBUTION
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Eosinophilic
Fasciitis
Nephrogenic Systemic
Fibrosis
NOTE DISTRIBUTION
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Clinical Appearance
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Clinical Appearance
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Clinical Appearance
Arm
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“Groove sign”: linear depression overlying
vein
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“Groove sign”: linear depression overlying
vein
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Symmetric and spares hands, feet, face
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When Thinking Sclerosis Disorders
• LOOK AT THE HANDS
– Sclerosis or Puffiness?
– True Raynaud’s?
– Capillary Changes?
– Telangiectasias?
– Calcinosis
– Pterygium Inversa?
• LOOK AT THE DISTRIBUTION
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Thank you!
