Connective Tissue Diseases

1. Connective Tissue Diseases Rick Lin, DO MPH

4. Lupus Erythematosus • Chronic Cutaneous LE • DLE • Verrucous LE • Lichen Planus-LE overlap. • Chiblain LE • Lupus Panniculitis (LE profundus) • With DLE • With Systemic LE

5. 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.

8. 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

9. 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

11. 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

12. Verrucous LE • AKA hypertrophic LE • Resembling KA or hypertrophic LP • Treatment with TAC or Intralesional • Also can be treated with Accutane or Plaquenil.

13. LE-LP Overlap syndrome • Large atrophic hypopigmented bluish-red patches and plaques. • Response to treatment is poor • Dapsone or Accutane maybe effective

14. 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

15. 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.

17. SCLE • Subacute cutaneous LE • Papulosquamous • Annular • Syndromes commonly exhibiting similar morphology • Neonatal LE • Complement deficiency syndromes

18. 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

22. 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

23. 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

26. 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

27. ARA SLE criteria • Malar Erythema • Discoid Lupus • Photosensitivity • Oral Ulcers • Arthritis • Serositis • Nephritis • Hematologic • CNS Changes • Immunologic disorder • ANA

29. 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.

30. Systemic Manifestation. • CNS involvement • Ideopathic throbocytopenic purpura. • Sjorgen’s syndrom • Mixed with dermatomyositis

31. 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.

34. 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

35. 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.

36. 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

45. 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).

46. 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.

47. 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.

50. 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.