1. Connective tissue diseasesBy
Dr.Alaa A. Naif
April 05, 2015

2. Lupus Erythematosus

3. Types of lupus erythematosus
Chronic cutaneous lupus erythematosus(DLE)
Subacute cutaneous lupus erythematous (SCLE)
Acute cutaneous lupus erythematous(SLE)
Involve epidermis and lower dermis
Involve epidermis and upper dermis
Don’t have systemic dis.(5-10% develop SLE)
Majority don’t have systemic disease
Systemic disease is present
Scarring , dyspigmentation, atrophy and follicular
plugging are prominent.
Skin lesions involve face, scalp and ears
Photosensitivity is
Prominent
Shawl distribution of skin lesions(sun exposed areas)
Dx requires four criteria out of eleven and exclusion of drug-induced SLE
F>M
Anti-Ro(SSA)
Anti-La(SSB)
ANA and anti-DNA and anti-smith

8. Pathogenesis
Infectious agent e.g viral cross react with self-antigen in person with genetic background
Perpetuating factors:
Ultraviolet light
Sex hormones
Stress

9. SLE criteria(4 out of 11) Malar(butterfly) rash Discoid lesions
Photosensitivity
Oral ulcer
Arithritis
Serositis e.g. pleuritis, pericarditis
Renal: proteinuria, casts
Neurological: psychosis, seizure
Hematological: decreased platelets, WBC or RBC
Immunological: anti-DNA, anti-Sm, antiphospholipid antibodies
ANA

10. Drug-induced SLE
It is different from idiopathic SLE by (1) less involvement of kidney and CNS and skin and (2) presence of anti-histone antibodies instead of ANA
Most commonly implicated drugs:
Procainamide
Hydralazine
Minocycline
INH
Penicillamine
TNF- inhibitors

11. Pathology Colloid bodies (damaged keratinocytes)
Vacuolar changes in basal layer
Epidermal atrophy
Thickenening of basement membrane
Peri-adnexal, upper and lower dermal lymphocytic infiltrate
Mucin deposition
DIF (direct immunofluorescence) show granular deposit at DE junction (lupus band)a and around adnexa

12. Treatment Topical: Sun protection, topical and intralesional steroids
Systemic: Antimalarial e.g. hydroxychloroquine, chloroquine
Others: retinoids, thalidomide, dapsone

14. Morphea Affect female more than male
Does not affect survival but can cause a disability especially the linear type
Fibroblast isolated from morphea lesion produce increased amount of collagen and this is thought to be due to production of IL-4 and TGF-β by T-cells
Some believes that Borrelia plays a role

15. Types
1. Plaque-type : present as white indurated plaque surrounded by lilac border
2. Deep morphea: invlove deep dermis, subcutis +/- fascia
3. Generalized morphea: plaques coaleasce affecting the entire trunk except nipple, can involves the extremities, it is disabiling causing difficulty in breathing, distinguished from systemic sclerosis by (1)absence of Raynauds phenomenon, (2)absence of internal organ involvement and (3)asymmetry of involvement

17. 4. Linear morphea: different from plaque morphea by (1) childhood onset, (2)high ANA titre and (3)disabling especially when involve joint or cause atrophy of the whole limb
Variants:
En coup de sabre type (sword hit): linear morphea of head, can involve muscle, bone
Parry-Romberg syndrome: hemifacial atrophy including eyes and tongue(the most severe form)

22. Diagnosis
Autoantibodies: ANA and anti-ssDNA are commonly seen in linear and generalized types
Pathology: hyalinized and eosinophilic collagen bundles with a little space in between and atrophy of hair follicles and sweat glands

24. Treatment Topical : Corticosteroid is ineffective
Vit D analogues e.g. calcipotriol
Systemic treatment:
Glucocorticoids, methotrexate ,PUVA (psoralen plus UVA) and UVA1
Others: penicillin, penicillamine, acitretin , calcitriol and IFN-γ

25. Systemic sclerosis(Scleroderma)
Diffuse SS
Limited SS
CREST syndrome
(Calcinosis, Raynauds phenomenon, Esophageal dysmotility, Sclerodactyly, Telengictasia)
Involve proximal extremities and trunk in addition to distal extremities and face
Involve distal extremities and face
Internal organ involvement is early and extensive
Internal organ involvement is late and limited
Worse prognosis
Better prognosis
Presence of anti-Scl 70 antibodies
Presence of anti-centromere antibodies
The same
The most common cause of death is lung involvement in both types
Possible internal organ involved: Lung(pulmonary hypertension, interstitial lung dis.), kidney(hypertensive renal crisis), GIT(GERD, dyphagia, diarrhea, constipation)

27. Dermatomyositis Classification(1): Dermatomyositis ( skin plus muscle)
Polymyositis (muscle only)
Amyopathic dermatomysitis (skin only)
Classification (2):
Juveile type: not associated with malignancy but associated with more calcinosis and vasculitis
Adult type: associated with malignancy especially ovarian, lung and breast

28. Clinical features Cutaneous:
Heliotrop rash(violaceous patch and edema around eyes)
Gottron papules: flat-topped violaceous papules on knuckles
Gottron sign: violaceous discoloration of knuckles, elbows and knee
Photodistribution of skin manifestations(shawl)
Nail fold telengictasia

35. Systemic :
Proximal myopathy: inability to comb, to walk upstair or to stand from sitting position
Lung: interstitial lung disease( restrictive lung disease)
Heart: conduction defects, arrhythmia
Calcinosis cutis: deposition of calcium in skin, subcutis and muscle

37. Diagnosis
Diagnosis of muscle involvement(which requires systemic steroid ): history, physical exam, muscle enzymes e.g. aldolase and CK, EMG, muscle biopsy, imaging e.g. MRI and ultrasound
Autoantibodies: ANA, anti-Jo1, anti-Mi2 antibodies

38. Treatment of cutaneous lesions: the same as cutaneous LE

39. Thanks For Listenening