Transcript Derm_CTD
Connective Tissue Diseases & Dermatology OUTLINE • Introduction • Lupus • Dermatomyositis/Polymyositis • Scleroderma – Systemic – Localized • Rheumatoid Arthritis INTRODUCTION 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 Systemic disease Chronic LE (Discoid) Scarring Acute Cutaneous (Systemic) LE CLINICAL • Rash on sun-exposed areas: – Face, chest, shoulders, extensor arms, dorsal hands – Malar 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 SLE Acute Cutaneous (Systemic) LE • Hand of a lupus patient – Affects phalangeal skin – Spares the knuckles • In comparison, dermatomyositis has the opposite findings! – Spares the phalangeal skin – Affects knuckles Raynaud Phenomenon 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 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 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 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 ears—commonly affected area! Discoid LE • Scarring alopecia with follicular plugging Scalp hair-bearing areas Dermatomyositis/Polymyositis 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 rash • Pathognomonic • Periorbital edema • Periorbital violaceous discoloration –Involves upper eyelids Gottron’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 papules Photosensitivity / 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 adult, you must search for malignancy… Malignancy in DM/PM • ↑ incidence of cancer in DM > PM • >50 y/o are at greatest risk Malignancy in DM/PM • 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 Scleroderma SCLERODERMA MULTI-SYSTEM FORMS -Systemic Scleroderma/Sclerosis -CREST Syndrome Consider systemic involvement if: -Raynaud phenomenon -Nail fold capillary change’s -Both LOCALIZED FORMS -Morphea Rarely shows evidence of visceral involvement 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 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 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 scleroderma” • Sx:-Calcinosis -Raynaud Phenomenon -Esophageal dysmotility -Sclerodactyly -Telangiectasias • Labs: Anti-centromere 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 Scleroderma SCLERODERMA MULTI-SYSTEM FORMS -Systemic Scleroderma/Sclerosis -CREST Syndrome Consider systemic involvement if: -Raynaud phenomenon -Nail fold capillary change’s -Both LOCALIZED FORMS -Morphea Rarely shows evidence of visceral involvement MORPHEA Clinical features distinguish the 2 entities SCLERODERMA MORPHEA • • • • • • • • • • Generalized Symmetrical (+) Raynauds phenomenon (+) internal organs involved Complications: – Significant ↑ morbidity – Significant ↑ mortality Localized Asymmetrical No Raynauds phenomenon No internal organs involved Complications: – RARELY contractures, growth retardation – No ↑ mortality 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 Morphea Morphea Treatment • Topical – Steroids – Calcipotriene ointment (Dovonex) • Intralesional steroids • Systemic – Hydrochloroquine – Oral calcitriole – Sulfasalazine Rheumatoid Arthritis Rheumatoid Arthritis - Cutaneous Features • 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 • 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. B. C. D. E. Dermatomyositis Polymyositis Lupus Scleroderma Sjogren’s The findings are consistent with? A. B. C. D. E. Dermatomyositis Polymyositis Lupus Scleroderma 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 photosensitive C. 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 photosensitive C. 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. B. C. D. E. Dermatomyositis Polymyositis Lupus Scleroderma Sjogren’s Diagnosis? CREST Diagnosis? MORPHEA Diagnosis? CREST/Scleroderma FINISHED!!!