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Connective Tissue Diseases& Dermatology
OUTLINE• Introduction• Lupus• Dermatomyositis/Polymyositis• Scleroderma
– Systemic– Localized
• Rheumatoid Arthritis
INTRODUCTION
Things to keep in mind… Things to keep in mind…
• Introduce you to the physical findings in connective tissue disease
• We’ll focus on PE findings, but remember CTD are complicated, multi-system disorders
• Sometimes there’s overlap among the CTD’s• These patients will need management by
Rheumatology, Dermatology, and other specialties
INTRODUCTION
DIAGNOSING CTD • Based on clinical picture• Antibody tests done to support diagnosis
– Provides subsets of disease– Gives information on prognosis
SLE Classification
• American College of Rheumatology– Having at least 4/11 is compatible with LE diagnosis– Mnemonic: SOAP BRAIN MD
• Serositis—pericarditis, pleuritis• Oral ulcers• Arthritis—non-erosive• Photosensitivity• Blood dyscrasias—low cell counts• Renal disorder• ANA abnormality• Immunologic disorder• Neurologic disorder—seizures, psychosis• Malar rash• Discoid rash
SLE Classification
• Serositis—pericarditis, pleuritis• Oral ulcers• Arthritis—non-erosive• Photosensitivity• Blood dyscrasias—low cell counts• Renal disorder• ANA abnormality• Immunologic disorder• Neurologic disorder—seizures, psychosis• Malar rash• Discoid rash
Lupus Erythematosus
• Cutaneous lupus has 3 major subsets– Acute Cutaneous LE (aka Systemic LE)– Subacute Cutaneous LE (SCLE)– Chronic Cutaneous LE (aka Discoid Lupus)
• Others– Neonatal lupus– Drug-induced
Clinical Spectrum of Lupus
Acute LE Subacute Cutaneous LE Chronic LE (Discoid)
Systemic disease Scarring
Acute Cutaneous (Systemic) LE
CLINICAL• Rash on sun-exposed areas:
– Face, chest, shoulders, extensor arms, dorsal hands– Malar rashMalar rash seen in only 10-50% of acute lupus
• Superficial to indurated, non-pruritic red to violaceous papules & plaques w/ fine scale
• Various combinations of systemic involvement
Malar rash of Lupus
SLESLE
Acute Cutaneous (Systemic) LE
• Hand of a lupus patient– Affects phalangeal Affects phalangeal
skinskin– Spares the knucklesSpares the knuckles
• In comparison, dermatomyositis has the opposite findings!– Spares the phalangeal
skin– Affects knuckles
Raynaud phenomenon (RP)Raynaud phenomenon (RP) • Occurs in 10-30% SLE• Precipitated by cold or emotional stress• Sx: Fingers and/or toes w/ pallor, cyanosis,
hyperemia Pain, burning, numbness/tingling, swelling, hyperhidrosis
Raynaud Raynaud PhenomenonPhenomenon
Mneumonic – Red, White, & Blue (really white, blue, red )
Other cutaneous signs of LE
• Telangiectasia– Palms, fingers– Periungual
• Alopecia (20%)– Non-scarring – Scarring—more common with DLE
• Urticaria-like plaques• Rheumatoid nodules
Subacute cutaneous lupus (SCLE)
• Two patterns:– Papulosquamous patterned lesions– Annular-polycyclic pattern lesions
• Lesions may last for months • No scarring• Most patients are white females• ~50% of SCLE pts progress to SLE~50% of SCLE pts progress to SLE
Subacute cutaneous lupus
CLINICAL• Distribution
– Trunk— most common– Sun-exposed skin
• Subtle hypopigmentation, atrophy, & telangiectasia
• Photosensitivity, periungual telangiectasies, vasculitis
• Systemic disease may occur, but usually not as severe as SLE
SCLE
Sun-exposed skin
SCLE
Trunk skin (not sun-exposed)
Chronic LE (Discoid Lupus)
• Low incidence of systemic disease – Only 5-10% evolve to SLE
• Females, 4th decade of life• UV light initiates &
exacerbates disease
Chronic LE (Discoid Lupus)
CLINICAL• FACE & SCALP – MOST COMMONFACE & SCALP – MOST COMMON• Round, sharply demarcated • Asymmetric• Elevated red to violaceous flat topped
plaques• Adherent scale “carpet tacking” • Atrophy• Scarring/scarring alopecia• Hypopigmentation• Follicular plugs
Discoid Lupus
• Plugging• Atrophy• Scale• Telangiectasia• Erythema
• Lesions endure for months • Resolve or become atrophic/scarred• Pigment alteration• Hypertrophic variant
• Conchal bowl of Conchal bowl of ears—commonly ears—commonly affected area!affected area!
Discoid LE
• Scarring alopecia with follicular plugging
Scalp hair-bearing areas
Dermatomyositis/Dermatomyositis/PolymyositisPolymyositis
DM/PM
• Epidemiology– Bimodal distribution
• Juvenile form•Adult form (>40 yrs)
– Males = females– Blacks to whites = 4:1
Dermatomyositis & Polymyositis
TYPES:(1) Dermatomyositis (DM)
– Both muscle weakness & skin changes
(2) Polymyositis (PM)– Muscle weakness, but NO skin changes
(3) Amyopathic Dermatomyositis– Skin changes only– NO muscle weakness
Muscle weakness in DM and PM
• Symmetric & proximal muscle weakness– Hips/thighs characteristic
• eg. Difficult rising from chair
– Neck commonly affected• eg. Drooped head
– Other muscles can be involved• Pharyngeal, respiratory chest wall
Skin manifestation of DM
• Heliotrope erythema of periorbit• Gottron’s sign• Gottron’s papules• Photosensitivity• Periungual erythema & telangiectases
Heliotrope rashHeliotrope rash• Pathognomonic• Periorbital edema• Periorbital violaceous discoloration
–Involves upper eyelids
Gottron’s signGottron’s sign• Symmetric, violaceous-to-erythematous
macules/patches over bony prominences– knuckles, but spares phalanges – Elbows & knees
• Gottron’s papules– smooth, violaceous papules– knuckles, side of fingers
Gottron’s papulesGottron’s papules
Photosensitivity / PoikilodermaPhotosensitivity / Poikiloderma• typically on sun-exposed skin on trunk
Dermatomyositis
• Periungual erythema– ragged cuticles
• Telangiectases– dilated/plump capillary loops
If you diagnose DM/PM in an If you diagnose DM/PM in an adult, you must search for adult, you must search for malignancymalignancy……
Malignancy in DM/PM
• ↑ incidence of cancer in DM > PM• >50 y/o are at greatest risk
Malignancy in DM/PM
• Which cancers?Which cancers?– Women
• Ovarian #1• Breast, lymphoproliferative
– Men• Prostate, colon, lymphoma, testicular
Work-up of DM/PM
• Diagnostic tests– Skin biopsy– Muscle enzymes—CK, ALT, AST, LDH, Aldolase– Muscle biopsy– MRI of muscles
• help locate muscles to biopsy, document flare-ups
– EMG– Antibody screen:
• 90% ANA+• Check Ro, La, Sm, nRNP, Jo-1, PM-1, Mi-2
– Presence can help define associated diseases
SclerodermaScleroderma
SCLERODERMA
MULTI-SYSTEM FORMS LOCALIZED FORMS
-Morphea-Systemic Scleroderma/Sclerosis-CREST Syndrome
Rarely shows evidence of visceral involvement
Consider systemic involvement if:
-Raynaud phenomenon
-Nail fold capillary change’s
-Both
PROGRESSIVE SYSTEMIC SCLEROSIS (SCLERODERMA)
Systemic Scleroderma
• Characterized by– sclerosis of the skin– sclerosis of visceral organs– vasculopathy (Raynaud’s)– autoantibodies
• 2-12 case/1 million each year
Scleroderma—subtypes
• Systemic– Diffuse scleroderma– CREST syndrome
• Localized– Morphea
Scleroderma—subtypes
• Systemic– Diffuse scleroderma
• Skin – more extensive involvement• Visceral – early appearance• Worse prognosis
– CREST syndrome• Skin – limited (face, fingers)• Visceral – delayed appearance• Better prognosis
Raynaud’s phenomenon
Raynaud phenomenon (RP)Raynaud phenomenon (RP) • One of the major criteria for scleroderma• Precipitated by cold or emotional stress• Sx: Fingers and/or toes w/ pallor, cyanosis,
hyperemia Pain, burning, numbness/tingling, swelling, hyperhidrosis
Raynaud Raynaud PhenomenonPhenomenon
Mneumonic – Red, White, & Blue (really white, blue, red )
Skin changes in Systemic Scleroderma
• Skin Changes– Begin distally
• fingers, hands• face
– Progresses proximally– 3 stages
• 1) Edematous• 2) Indurated/sclerotic• 3) Atrophic
Skin changes in Systemic Scleroderma
• Edematous phase– Skin thickened, swollen, tense– Non-pitting edema
• “mask-like facies”• “sausaging” of the fingers
Skin changes in Systemic Scleroderma
• Indurative/Sclerotic – Skin hardened, bound down– Restricted range of motion– Contractures – Appendageal atrophy hair
loss, anhidrosis – Ulcerations, telangiectasia,
and atrophy – Fingers narrow or taper
distally– Distal phalanges shortened
due to bone resorption
• Atrophic
Skin changes in Systemic Scleroderma
Skin changes in Systemic Scleroderma
• The face of scleroderma– Skin contracts, fixed to
bone– Beaked nose– Furrowed perioral skin– Smaller oral aperture– Thinned lips– Facial telangiectases
Skin changes in Systemic Scleroderma
• Telangiectasias– Flat/macular– Rectangular collections of uniform, tiny
vessels– Lips, face, palms, back of hands
• Calcinosis cutis (CC) can be a late sequela to skin inflam
• CC occurs at sites of trauma– Buttocks, elbows, knees, fingers, around shoulders
• Complications: local pain, contractures, infections• SKIN MORPHOLOGY: Hard, irregular nodules draining
whitish, chalky material Extrusion leads to ulcers, sinuses, or cellulites
Multi-organ involvement in Scleroderma
• Gastrointestinal– Esophagel dysfunction
• Dysphagia – most common• Other symptoms: GERD, strictures
Multi-organ involvement in Scleroderma
• Respiratory– Frequent cause of death– Dyspnea—most common symptom – Interstitial fibrosis– Pulmonary hypertension (33%)
Multi-organ involvement in Scleroderma
• Renal– Renal disease– HTN
• Cardiovascular– Myocardial fibrosis arrhythmias
• Salivary– Sclerosis of salivary glands Sjogren’s-like
CREST syndrome
• aka ““Limited cutaneous sclerodermaLimited cutaneous scleroderma””• Sx: -CCalcinosis
-RRaynaud Phenomenon-EEsophageal dysmotility-SSclerodactyly-TTelangiectasias
• Labs: Anti-centromereAnti-centromere Antibodies Antibodies• More benign, chronic, and localized • Better Prognosis!!!
CREST syndrome
• Calcinosis cutis– Palmar tips of fingers– Over bony prominences of knees, elbows,
spine, and iliac crests
Localized SclerodermaLocalized Scleroderma
SCLERODERMA
MULTI-SYSTEM FORMS LOCALIZED FORMS
-Morphea-Systemic Scleroderma/Sclerosis-CREST Syndrome
Rarely shows evidence of visceral involvement
Consider systemic involvement if:
-Raynaud phenomenon
-Nail fold capillary change’s
-Both
MORPHEA
MORPHEA• Localized• Asymmetrical• No Raynauds
phenomenon• No internal organs
involved• Complications:
– RARELY contractures, growth retardation
– No ↑ mortality
SCLERODERMA• Generalized• Symmetrical• (+) Raynauds phenomenon• (+) internal organs involved• Complications:
– Significant ↑ morbidity – Significant ↑ mortality
Clinical features distinguish the 2 entities
Scleroderma vs Morphea
• Scleroderma– bound-down, tight; minor skin color change
• Morphea:– Small purplish induration – Become discolored, thicker, firm, hairless, ivory-
colored – Smooth, dull, white, waxy surface– Violaceous or lilac-colored active inflammatory
border – Atrophy, mottled brown hyperpigmentation– Can have guttate form
Treatment
• Topical– Steroids– Calcipotriene ointment (Dovonex)
• Intralesional steroids• Systemic
– Hydrochloroquine– Oral calcitriole– Sulfasalazine
Rheumatoid ArthritisRheumatoid Arthritis
• Rheumatoid Nodules– Rare in kids w/ JIA
• If seen, M/C seen in (+)RF polyarthritis– M/C location near olecranon process on ulnar
forearm• Other common areas: dorsal hands, knees, ears, &
pressure pts– Firm, non-tender
Rheumatoid Arthritis - Cutaneous Features
• Cuticular telangiectasias– Can be seen in RA pts, but they are M/C &
characteristic in pts w/ LE, scleroderma, dermatomyositis
Ready for a QUIZ?
The findings are consistent with?
A. DermatomyositisB. PolymyositisC. LupusD. SclerodermaE. Sjogren’s
The findings are consistent with?
A. DermatomyositisB. PolymyositisC. LupusD. SclerodermaE. Sjogren’s
This 50 year old patient was recently diagnosed with a connective tissue disease. Which of the following is likely to be FALSE?
A. She has trouble running and climbing stairs.
B. She is photosensitiveC. She may develop a
malignancy within one year
D. She has renal disease
This 50 year old patient was recently diagnosed with a connective tissue disease. Which of the following is likely to be FALSE?
A. She has trouble running and climbing stairs.
B. She is photosensitiveC. She may develop a
malignancy within one year
D. She has renal disease
What the diagnosis?
• Systemic lupus erythematosus
What the diagnosis?
• Dermatomyositis
What the diagnosis?
• Discoid Lupus
What the diagnosis?
• Lupus
Lupus Dermatomyositis
What the diagnosis?
• Discoid Lupus
The findings are consistent with?
A. DermatomyositisB. PolymyositisC. LupusD. SclerodermaE. Sjogren’s
Diagnosis?
CRESTCREST
Diagnosis?Diagnosis?
MORPHEAMORPHEA
CREST/SclerodermaDiagnosis?Diagnosis?
FINISHED!!!FINISHED!!!