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Connective Tissue Diseases. Rick Lin, DO MPH. Lupus Erythematosus. Chronic Cutaneous LE DLE Verrucous LE Lichen Planus-LE overlap. Chiblain LE Lupus Panniculitis (LE profundus) With DLE With Systemic LE. Discoid LE. Young adults. F:M=2:1 Cat’s Tongue (Langue au chat) = carpet tacks

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Connective tissue diseases l.jpg

Connective Tissue Diseases

Rick Lin, DO MPH


Lupus erythematosus l.jpg
Lupus Erythematosus

  • Chronic Cutaneous LE

    • DLE

    • Verrucous LE

    • Lichen Planus-LE overlap.

    • Chiblain LE

    • Lupus Panniculitis (LE profundus)

      • With DLE

      • With Systemic LE


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Discoid LE

  • Young adults. F:M=2:1

  • Cat’s Tongue (Langue au chat) = carpet tacks

  • Lesions heal centrally first with atrophy, scarring, and dyspigmentation

  • Up to 24% will have mucosal involvement.

  • 95% of cases confine to the skin at the onset and will remain so.


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Discoid LE

  • Spontaneous involution with scarring is common

  • Progression to SLE is rare and may be identified by abnormal labs.

    • ANA – elevated

    • Leukopenia, hematuria, or abuminuria


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Histology

  • Thinned epidermis

  • Loss of normal rete ridges

  • Follicular plugging

  • Hydropic changes of basal layer

  • Lymphocytic perivascular infiltrate

  • Increase mucin

  • DIF is positive more than 75% of case with Igs located at DEJ


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Treatment

  • SUNSCREEN!!!!

  • Topical steroid, high potency with occlusion if needed.

  • Intralesional Injection with Kenalog

  • Antimalarias: safest and most beneficial system therapy.

    • Plaquenil for 3 month, if no response switch to Aralen.

    • If response is still incomplete, change to Quinacrine


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Verrucous LE

  • AKA hypertrophic LE

  • Resembling KA or hypertrophic LP

  • Treatment with TAC or Intralesional

  • Also can be treated with Accutane or Plaquenil.


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LE-LP Overlap syndrome

  • Large atrophic hypopigmented bluish-red patches and plaques.

  • Response to treatment is poor

  • Dapsone or Accutane maybe effective


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Chilblain LE

  • Chronic, unrelenting form of LE with fingertips, rims of ear, calves and heels in women.

  • Chilblain lesions are due to cold

  • Usual LE treatment


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LE Panniculitis

  • AKA LE Profundus

  • Deep subcutaneous nodules 1-4cm

  • Head, face, and upper arms

  • Woman age 20-45

  • Histology shows lymphocytic panniculitis, hyaline degeneration of the fat, hyaline papillary bodies. Over lying epidermis shows hydropic changes and follicular plugging

  • Treatment with Antimalarials.


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SCLE

  • Subacute cutaneous LE

    • Papulosquamous

    • Annular

    • Syndromes commonly exhibiting similar morphology

      • Neonatal LE

      • Complement deficiency syndromes


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SCLE

  • Psoriasiform, polycyclic annular lesions

  • Shawl distribution: V neck, upper outer and inner arms.

  • ¾ of the patients have arthralgia,

  • 20% have leukopenia

  • 80% have positive ANA

  • Associated with Ro/SSA and HLA-DR3-Positive.

  • Hydrochlorothiazide can induce SCLE


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Neonatal LE

  • Annular scaling erythematous macules and plaques

  • Appear on head and extremities

  • First few months of life in babies born to mothers with LE, RA, or other connective tissue disease

  • Resolve spontaneously by 6 month of age

  • HALF of the patient has associated congenital heart block, usually 3rd degree


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Acute Cutaneous LE

  • Characteristic butterfly facial erythema

  • May last from days to several weeks

  • Bullous lesion occur as single or grouped vesicle or bullae

  • Subepidermal bulla containing neutrophils.

  • HLA-DR2 positive

  • Minute telangiectases appear in time on the face or elsewhere and commonly appear about the nail folds.

  • Rowell Syndrome: EM-like lesion dominant in LE


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Systemic LE

  • Young to middle age women

  • Skin involvement occur 80% of the case

  • American Rheumatism Association has 11 criteria

  • If 4 or more of the criteria are satisfied, the patient is said to have SLE


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ARA SLE criteria

  • Malar Erythema

  • Discoid Lupus

  • Photosensitivity

  • Oral Ulcers

  • Arthritis

  • Serositis

  • Nephritis

  • Hematologic

  • CNS Changes

  • Immunologic disorder

  • ANA


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Systemic Manifestation.

  • Arthralgia is the earliest abnormality.

  • 95% of SLE patient will have arthralgia.

  • Avascular necrosis of femoral head.

  • Thrombosis in vessels secondaary to presence of lupus anticoagulant.

  • Renal involvement in nephritic or nephrotic type.

  • Mycocarditis, cardiomegly, EKG changes.


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Systemic Manifestation.

  • CNS involvement

  • Ideopathic throbocytopenic purpura.

  • Sjorgen’s syndrom

  • Mixed with dermatomyositis


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Treatment of SLE

  • Treatment of depending on the organ system(s) involved.

  • Skin, musculoskeletal, and serositis-type manifestations generally respond to treatment with hydroxychloroquine and nonsteroidal anti-inflammatory medications.

  • Porphoria cutaneous tarda may co-exist with LE, in this case, Plaquenil is TOXIC!!!

  • More serious organ involvement, such as CNS involvement or renal disease, often necessitates immunosuppression with high-dose steroids and cyclophosphamide.


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Dermatomyositis

  • Gratton's sign - flat-topped violaceous papules

  • Heliotrope - reddish -purple flush around the eyes

  • Over knuckle streak erythema

  • Shawl pattern

  • Calcinosis Cutis may occur in oer half of the children with DM

  • Associated with Malignancy


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Dermatomyositis

  • Symmetrical muscle weakness

  • assoc c malignant neoplasm when over 40

  • periungual telangiectasia

  • Prednisone 60mg until severity decrease. Sunscreen, antimalarial

  • Mechanics hand: hyperkeratosis, fissuring, scaling involvement in the palm of the hand.


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Muscle involvement

  • Symmetrical muscle weakness

  • Unable to raise arms to comb their hair

  • Cardiac involvement with cardiac failure in terminal phase

  • Amyopathic dermatomyositis or dermatomyositis sine myositis: DM without muscle changes


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Scleroderma

  • characterized by symmetric thickening, tightening, and induration of the skin of the fingers and the skin

  • These changes may affect the entire extremity, face, neck, and trunk (thorax and abdomen).


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Localized Morphea

  • Smooth, hard, somewhat depressed, yellowish white, or ivory-colored lesions.

  • Common on the trunk

  • Margins surrounded by light violaceous zone or by telangiectases.

  • Resemble pigskin

  • Slowly involute over a 3-5 year period.


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Generalized Morphea

  • Widespread hard indurated plaque.

  • No systemic involvement

  • Patient appear young because of the firmness of the skin.

  • Resolution less likely than the localized version.


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Atrophoderma of Pasani and Pierini

  • Reduction of thickness of derma connective tissue

  • Upperback and lumbar sacral area

  • Benign course, usually resolve after few months or few years.

  • No effect treatment

  • Variant of morphea.


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Linear Scleroderma

  • Linear lesions extend to length of arms or leg

  • Begin first decade of life

  • May also occur parasagitally down the forehead, known as en coup de sabre

  • Parry-Romberg syndrome: progressive facial hemiatrophy, epilepsy, exophalmos, and alopecia, maybe a form of linear scleroderma.


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CREST Syndrome

  • AKA Thibierge-Weissenbach Syndrome.

  • Systemic sclerosis may be limited to the hands, and is called acroslerosis.

  • Not as severe as PSS

  • ANA shows anticentromere antibody, and is highly specific.

  • Most favorable diagnosis


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Progressive Systemic Sclerosis

  • Raynaud’s is the first manifestation of PSS most of the time and is eventually nearly always present

  • Round fingerpad sign: loose the normal peaked contour and appear round from the side.

  • Pterygium inversum unguis: distal part of nailbed remains adherent to ventral surface of nail plate. Seen also in LE, or congenital


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Progressive Systemic Sclerosis

  • 75% have dilated nail fold capillary loops

  • Esophageal involvement in 90% of patients

  • Pulmonary fibrosis

  • Cardiac involvement

  • Articular pain, swelling, polyarthritis.


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Prognosis

  • Skin involvement after 1 year of diagnosis:

  • Group I – sclerodactyly alone – 71% 10 year survival rate

  • Group II - Skin stiffness above metacarpal-phalangeal joints but not involving trunk – 58% survival rate.

  • Group III – truncal involvement – 21% survival.


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LAB Finding

  • Topoisomerase I (formerly Scl–70) is present in 20-30% of patients with diffuse disease (absent in limited disease) and has an increased association with pulmonary fibrosis

  • Anticentromere antibodies are present in about 60-90% of patients with limited disease and 10-15% with diffuse disease.


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Histology

  • Increased collagen bundle and thickness of the derma.

  • Pilosebaceous units are absent. Eccrine glands and ducts are compressed by collagen.

  • Eccrine glands present at the mid dermis rather than at the junction of dermis/subQ fat.


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Eosinophilic Fasciitis

  • Patient engaging in strenuous muscular effort few days or week before acute onset of weakness. Follow by severe induration of the skin and subQ tissue of forearms and legs.

  • Coarse peau d’orange appearance.

  • Groove sign: depression follow the course of underlying vessles when arms are hold laterally.

  • Excellent response to corticosteroid.


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Comparison of deep morphea and eosinophilic fasciitis. A Note the ‘pseudo-cellulite’ appearance of the involved skin of the thigh in deep morphea. B In eosinophilic fasciitis, the level of fibrosis is also deep.


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Histology Note the ‘pseudo-cellulite’ appearance of the involved skin of the thigh in deep morphea. B In eosinophilic fasciitis, the level of fibrosis is also deep.

  • Patchy lymphocytic and plasma cell infilrate in the fascia and subfacial muscle and great thickening, 10-50 times normal of the fascia.


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Mixed Connective Tissue Disease Note the ‘pseudo-cellulite’ appearance of the involved skin of the thigh in deep morphea. B In eosinophilic fasciitis, the level of fibrosis is also deep.

  • Mixed features of scleroderma, SLE, and dermatomyositis

  • IgG deposition on the Direct IF is a distinctive finding in MCTD

  • Treatment with daily dose of prednisone 1mg/kg shows good improvement.


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Sjogren’s Syndrome Note the ‘pseudo-cellulite’ appearance of the involved skin of the thigh in deep morphea. B In eosinophilic fasciitis, the level of fibrosis is also deep.

  • AKA Sicca syndrome

  • Triad of keratoconjunctivitis sicca, xerostomia, and thrumatoid arthritis.

  • RF is usually positive

  • Elevated C-reactive Protein, IgG, IgA, and IgM

  • 80% has anti-Ro/SSA antibody.

  • >50% have anti-La/SSB antobodies

  • Only symptomatic treatment available.


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Rheumatoid Nodules Note the ‘pseudo-cellulite’ appearance of the involved skin of the thigh in deep morphea. B In eosinophilic fasciitis, the level of fibrosis is also deep.

  • 20-30% of RA patients

  • Subcutaneous nodules

  • Found anywhere on the body

  • Histologically shows dense foci of fibrinoid necrosis surrounded by histiocytes in palisaded arrangement.


Relapsing polychondritis l.jpg
Relapsing Polychondritis Note the ‘pseudo-cellulite’ appearance of the involved skin of the thigh in deep morphea. B In eosinophilic fasciitis, the level of fibrosis is also deep.

  • Intermittent episodes of inflammation of the articular and nonarticular cartilage eventualing in chondrolysis.

  • MAGIC syndrome = Behcet’s + Relapsing Polychondritis (Mouth And Genital ulcers with Inflamed Cartilage)

  • Treatment with Dapsone for few weeks, then maintenance for 4-6 asymptomatic months.