Autoimmune and Connective Tissue Disorders<br />By:<br />Dr. Kazhan Ali Tofiq Kadir<br />April 2011<br />
Connective Tissue Disorders:<br />          The cardinal feature of these conditions is inflammation of the CT which leads...
Systemic Lupus Erythematosus<br />specific labs - native(Double stranded) DNA, SM antigen<br />ANA test positive in 95%<br...
SLE - Diagnostic Criteria <br />
SLE<br />Head and Neck Manifestations<br />Malar rash first sign in 50% (Butter Fly Rash)<br />Erythematous maculopapular ...
larynx and trachea involvement rare
Vocal Cord thickening, paralysis, cricoarytenoid arthritis, subglotticstenosis
acute parotid enlargement 10%</li></li></ul><li>-xerostomia occars-neuropathy 15%-discoid lupus<br />
SLE<br />Treatment<br />avoid sun exposure<br />NSAIDS<br />topical and systemic steroids<br />antimalarials<br />low-dose...
DLE: Discoid Lupus Erythematosus:<br />Benign<br />Face (most frequently)<br />Patches:disc like<br />various sized , redd...
Precipitating factors: in DLE<br />Acronym: (ITEM)<br />1. Infection 	<br />2. Trauma 	<br />3. Exposure to cold<br />4. M...
Histopathology of DLE: <br />a. Liquefaction degenerationof the basal cell layer<br />b. Hyalinization, Edema & Fibrinoid ...
Symptoms:<br /><ul><li>Rash (Patches) +
history of Raynaud’s phenomenon, chilblains or poor peripheral circulation </li></ul>Signs: <br /><ul><li>The Face is most...
Scale: when the scale is removed its undersurface on direct examination shows horny plugs (which have occupied dilated pil...
D. D. of DLE: - <br />1. Morphea	 <br />2. LSA ( Lichen Sclerosus et Atrophicus): <br />3. PLE (polymorphic light eruption...
Treatment of DLE: <br />1. General measures: <br />The patients should avoid excessive exposure to sunlight <br />They sho...
Morphea<br />Definitions:<br />Sclerosis:<br />is diffused or circumscribed INDURATION of the S. C. tissue<br />may also i...
Localized Morphea: <br />Prevalence: 3 Females to 1 male <br />Localized sclerosis of the skin of unknown cause; <br />3 c...
Systemic sclerosis: (Systemic scleroderma): <br />Face & Hands (most frequently) but the change may extend proximally to i...
Lichen sclerosis et atrophicans (L. S. A): -<br />* uncommon <br />*small, white areas on the skin associated with an atro...
Dermatomyositis:Is a disorder mainly of <br />skin, muscle & B.V.s <br />Characteristic Erythematous & Edematous changes i...
The rash in Dermatomyositis is diagnostic:<br />     - Face: A purplish- red heliotrope erythema occurs on the face, espec...
Sjogren’s Syndrome<br />General<br />immune mediated<br />destruction of exocrine glands<br />primary<br />sicca syndrome-...
Sjogren’s Syndrome<br />General<br />prevalence<br />1% population<br />10-15% of RA patients<br />9 to 1 F>M<br />onset 4...
Sjogren’s Syndrome<br />General<br />clinical manifestations<br />xerophthalmia, keratoconjunctivitis <br />xerostomia<br ...
Sjogren’s Syndrome<br />Head and Neck Manifestations<br />80% xerostomia, most prominent symptom<br />difficulty chewing, ...
Sjogren’s Syndrome<br />Head and Neck Manifestations<br />salivary quantification- salivary scintigraphy<br />salivary gla...
Sjogren’s Syndrome<br />eye complaints<br />dryness, burning, itching, foreign body sensation<br />keratoconjunctivitis si...
Sjogren’s Syndrome<br />Schirmer I<br />Schirmer II<br />nasal crusting, epistaxis, hyposmia<br />Diagnostic:<br />history...
Sjogren’s Syndrome<br />Treatment<br />symptomatic<br />oral fluid intake<br />saliva substitutes<br />artificial tears<br...
Sjogren’s Syndrome<br />Treatment<br />pilocarpine<br />clotrimazole/nystatin<br />close dental supervision<br />surveilla...
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Dermatology 5th year, 5th lecture (Dr. Kazhan)

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Dermatology 5th year, 5th lecture (Dr. Kazhan)

  1. 1. Autoimmune and Connective Tissue Disorders<br />By:<br />Dr. Kazhan Ali Tofiq Kadir<br />April 2011<br />
  2. 2. Connective Tissue Disorders:<br /> The cardinal feature of these conditions is inflammation of the CT which leads to dermal atrophy or sclerosis, arthritis and S.T. to abnormalities in other organs. Basic to them all is a complex array of immune responses that target or affect collagen or ground substances.<br />Lupus Erythematosus<br />General<br />autoimmune multisystem disease<br />prevalence 1 in 2,000<br />9 to 1; female to male (1 in 700)<br />peak age 15-25<br />immune complex deposition<br />photosensitive skin eruptions, serositis, pneumonitis, myocarditis, nephritis, CNS involvement<br />
  3. 3. Systemic Lupus Erythematosus<br />specific labs - native(Double stranded) DNA, SM antigen<br />ANA test positive in 95%<br />LE cells<br />
  4. 4. SLE - Diagnostic Criteria <br />
  5. 5. SLE<br />Head and Neck Manifestations<br />Malar rash first sign in 50% (Butter Fly Rash)<br />Erythematous maculopapular eruption after sun exposure<br />Oral ulceration<br /><ul><li>3-5% perforated nasal septum
  6. 6. larynx and trachea involvement rare
  7. 7. Vocal Cord thickening, paralysis, cricoarytenoid arthritis, subglotticstenosis
  8. 8. acute parotid enlargement 10%</li></li></ul><li>-xerostomia occars-neuropathy 15%-discoid lupus<br />
  9. 9. SLE<br />Treatment<br />avoid sun exposure<br />NSAIDS<br />topical and systemic steroids<br />antimalarials<br />low-dose methotrexate<br />azothioprine, cyclophosphamide restricted<br />symptomatic treatment<br />saliva substitutes, Klack’s solution<br />postprandial rinses with H2O2 and H2O<br />
  10. 10. DLE: Discoid Lupus Erythematosus:<br />Benign<br />Face (most frequently)<br />Patches:disc like<br />various sized , reddish, well- defined, scaly (adherent ) <br />with hyperkeratosis which tend to heal with atrophy, scarring& pigmentary changes. <br />Hematological & Serological changes in about half (½) the patients, <br />Female: male is 2: 1 <br />Age of onset is 15 – 40 years <br />
  11. 11. Precipitating factors: in DLE<br />Acronym: (ITEM)<br />1. Infection <br />2. Trauma <br />3. Exposure to cold<br />4. Mental stress. <br />* Sunlight is exacerbating factor. <br />
  12. 12. Histopathology of DLE: <br />a. Liquefaction degenerationof the basal cell layer<br />b. Hyalinization, Edema & Fibrinoid change below epidermis.<br />c. A patchy dermal lymphocytic infiltrate around the appendages<br />
  13. 13. Symptoms:<br /><ul><li>Rash (Patches) +
  14. 14. history of Raynaud’s phenomenon, chilblains or poor peripheral circulation </li></ul>Signs: <br /><ul><li>The Face is most commonly affected & </li></ul> the scalp, ears, nose, arms, legs & trunk to a lesser extent.<br />-The circumscribed or discoid type is the most frequent & occurs particularly on the cheeks, the bridge of the nose, the ears, the side of the neck & the scalp. <br />- Unilateral, may be bilateral. <br /><ul><li>Permanent alopecia + well defined erythematous patches, varying in size,
  15. 15. Scale: when the scale is removed its undersurface on direct examination shows horny plugs (which have occupied dilated pilosebaceous canals). </li></ul> Raynaud’s phenomenon<br />
  16. 16. D. D. of DLE: - <br />1. Morphea <br />2. LSA ( Lichen Sclerosus et Atrophicus): <br />3. PLE (polymorphic light eruptions).<br />4. Contact Eczema <br />5. Seborrheic. Ecz. <br />6. Psoriasis <br />7. Rosacea<br />8. Necrobiosis Lipoidica. <br />9. Lupus vulgaris (skin TB). <br />
  17. 17. Treatment of DLE: <br />1. General measures: <br />The patients should avoid excessive exposure to sunlight <br />They should wear a broad – brimmed hat & avoid short- sleeved shirts & shorts. <br />They should use a sun- screening cream & lotion. Sunscreens are not for treatment once a reaction has occurred. <br />Patients who note exacerbation of their lesions with the cold should warp up well in the winter<br />Nifedipine may be helpful in those with Raynaud’s phenomenon.<br />Small dose of sedative in mental stress.<br />2. Topical therapy: Moderately potent steroid cream twice daily.<br />3. Intralesional Cs injects are helpful in resistant cases.<br />4. Oral antimalarials: <br />chloroquine tab. ½ tab. twice a day for 4- 6 wks<br />
  18. 18. Morphea<br />Definitions:<br />Sclerosis:<br />is diffused or circumscribed INDURATION of the S. C. tissue<br />may also involve the dermis, when the overlying epidermis may be atrophic. <br />Scleroderma:<br />- ↑ed depostion collagen in interstitium of small arteries <br /> and CT<br />- sclerotic skin changes, often multisystem disease <br />- is sclerosis of the skin occurs in:<br />1. LE<br />2. Morphea (Localized or Generalized) <br />3. Systemic sclerosis <br />4. L.S.A<br />5. Dermatomyositis<br />
  19. 19. Localized Morphea: <br />Prevalence: 3 Females to 1 male <br />Localized sclerosis of the skin of unknown cause; <br />3 clinical types: Circumscribed, Linear & Frontoparietal: <br /> (en coup de saber) with or without hemiatrophy of the face.<br />Clinical Features: <br />The lesions: <br />-indurated purplish, <br /> spontaneous, usually multiple, round or oval, 2-15 cm <br />- lose their color, become -> thickened & waxy areas, ivory in <br />color with a characteristic lilac- colored edge, surface is usually smooth & shiny<br />- in the lesion the hairs are absent, there will be No sweating <br />- on the trunk & limbs, face & genitalia. <br />- activity is usually between 3 –5 years & spontaneous resolution <br />-can be generalized.<br />Treatment: local steroid, or Intralesional Steroid <br />
  20. 20. Systemic sclerosis: (Systemic scleroderma): <br />Face & Hands (most frequently) but the change may extend proximally to involve the forearms & upper arms, <br />Face:facial appearance: is characteristic: - <br /> - The forehead is smooth & shiny<br /> - The forehead lines are expressless.<br /> - The nose becomes small & pinched. <br /> - The mouth opening is constricted & radial furrows appear.<br /> - Small, mat – like telangiectases are frequently found on the face, occur in 75% of patients. <br />Hands & forearms<br />forearm’s skin will be thinner than normal. <br />Atrophy occurs 1st in the pulps of the fingers & small painful ulcers are formed, heal with scars, <br />the nails are curving over the atrophic phalanges. <br />Paronychia is common<br />Pigmentation occurs in a bout 50% of the patients. <br />Leg ulcers occur in 40% of the patients.<br />
  21. 21. Lichen sclerosis et atrophicans (L. S. A): -<br />* uncommon <br />*small, white areas on the skin associated with an atrophic condition of the vulva & perianal skin.<br />Clinical features:<br />1- Non – genital L S A: <br /> - on the trunk, axillae & flexor surface of the wrists. <br /> - small, ivory white, shiny & round macules or papules, <br /> (a few mms in diameter) <br /> - later atrophy & the surface of the lesions wrinkled. <br />2- Anogenital LSA in women between 45 – 60 <br /> - Irritation may be marked<br /> - on the vulva & around the anus.<br /> - ivory – colored atrophic papules with <br /> follicular hyperkeratosis on the vulva.<br />DDx: <br />1. Morphea 2. L.P. 3. DLE. <br />Treatment: topical Cs may be of help. <br />
  22. 22. Dermatomyositis:Is a disorder mainly of <br />skin, muscle & B.V.s <br />Characteristic Erythematous & Edematous changes in the Skin + Muscle Weakness & Inflammation. <br />in childhood<br />Calcinosis is frequent & is usually ass. with a more favorable prognosis for life. <br />In adults:commonly ass. with an underlying carcinoma or reticulosis<br /> - Usually the patient 1st notices aching & weakness of the muscles as difficulty in going upstairs or rising from a chair or in women difficulty in raising the arms high enough to comb the hair. <br />
  23. 23. The rash in Dermatomyositis is diagnostic:<br /> - Face: A purplish- red heliotrope erythema occurs on the face, especially involving the eyelids, the upper cheeks, the forehead & the temples associated with the edema of the eyelids & periorbital tissues that is why it should be differentiated from Contact Dermatitis (CD) of the face. <br /> - Hands: Erythematous changes occur on the backs of fingers especially over the backs of the Joints & the knuckles.<br />
  24. 24. Sjogren’s Syndrome<br />General<br />immune mediated<br />destruction of exocrine glands<br />primary<br />sicca syndrome- isolated d/o lacrimal and salivary glands<br />secondary<br />sicca complex- assoc. with other CTD<br />
  25. 25. Sjogren’s Syndrome<br />General<br />prevalence<br />1% population<br />10-15% of RA patients<br />9 to 1 F>M<br />onset 40-60 yrs<br />increased risk of lymphoma<br />perhaps 44 times risk<br />
  26. 26. Sjogren’s Syndrome<br />General<br />clinical manifestations<br />xerophthalmia, keratoconjunctivitis <br />xerostomia<br />other areas<br />skin, vagina, genitalia, chronic bronchitis, GI tract, renal tubules<br />diagnosis<br />minor salivary gland biopsy<br />labs<br />RF and ANA <br />SS-A/Ro 60%<br />SS-B/La 30%<br />
  27. 27. Sjogren’s Syndrome<br />Head and Neck Manifestations<br />80% xerostomia, most prominent symptom<br />difficulty chewing, dysphagia, taste changes, fissures of tongue and lips, increased dental caries, oral candidiasis<br />
  28. 28. Sjogren’s Syndrome<br />Head and Neck Manifestations<br />salivary quantification- salivary scintigraphy<br />salivary gland enlargement<br />
  29. 29. Sjogren’s Syndrome<br />eye complaints<br />dryness, burning, itching, foreign body sensation<br />keratoconjunctivitis sicca<br /> corneal abrasions - rose bengal staining<br />
  30. 30. Sjogren’s Syndrome<br />Schirmer I<br />Schirmer II<br />nasal crusting, epistaxis, hyposmia<br />Diagnostic:<br />history><br />SchirmerI>Schirmer II or rose bengal staining or other supportiveevidence:<br />minor salivary gland biopsy<br />
  31. 31. Sjogren’s Syndrome<br />Treatment<br />symptomatic<br />oral fluid intake<br />saliva substitutes<br />artificial tears<br />avoid<br />decongestants<br />antihistamines<br />diuretics<br />anticholinergic <br />
  32. 32. Sjogren’s Syndrome<br />Treatment<br />pilocarpine<br />clotrimazole/nystatin<br />close dental supervision<br />surveillance for malignancy<br />
  33. 33. Behcet’s Disease<br />Vasculitis with triad<br />oral, genital ulcers, uveitis or iritis<br />oral<br />aphthous-like<br />painful, clusters on lips, gingiva, buccal, tongue<br />less often palate, oropharynx<br />genital<br />similar in appearance<br />
  34. 34. Behcet’s Disease<br />occular<br />uveitis, iritis<br />hypopyon<br />healing in days to weeks some scarring<br />symptoms simultaneously, months apart<br />
  35. 35. Behcet’s Disease<br />other findings<br /> tinnitus, vertigo<br />nasal, laryngeal, tracheal mucosal ulceration<br />CNS involvement, bowel dysfunction, large vessel arteritis<br />treatment<br />azothioprine, methotrexate possibly, not documented<br />
  36. 36. Thank You<br />

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