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Connective Tissue diseases:

المرحلة الخامسة ا.د.محمد كاظم الحطاب المادة:الجلدية

Are groups of clinico pathological conditions involve connective tissue of most systems of the body, include mainly LE, scleroderma, systemic sclerosis, dermatomyositis, MCTD &others…

Lupus Erythematosus:

is an autoimmune disorder resulting from an interplay of genetic, environmental &hormonal elements with a heterogeneous clinical expression extending from a localized cutaneous form to a life threatening systemic form.

LE is a spectrum of diseases, in one end of spectrum: DLE is purely cutaneous LE, at the other end :SLE. In the middle of spectrum: sub acute LE, neonatal LE, complement deficiency LE& drug-induced LE .

Discoid Lupus Erythematosus(chronic DLE):

DLE is a relatively benign disorder of the skin, most frequently involving the face and characterized by various- sized ,reddish ,well-defined scaly patches which tend to heal with atrophy, scarring and pigmentary changes.

Etiology & pathogenesis: there should be genetic factors, these will cause somatic mutations in lymphoid stem cells during life, this will lead to forbidden clones of lymphocytes ,which synthesize autoantibodies. Normally ,an endogenous defense mechanism appears to be directed against the proliferating forbidden clones. Till now ,the patient is living with out any clinical or abnormal serological findings.

When these clones of lymphocytes become un forbidden, autoimmune disease appears .certain modifying factors in environment: uv light ,trauma, drugs , emotional tension & infection.

These factors interfere with immunological defense mechanism .so when some body exposed to these environmental factors ,the disease appears.

DLE is primarily a disease of adult life with the average time of onset in the fourth decade ,with a F:M ratio of about 2:1.

Factors trigger ,initiate or exacerbate DLE:

Trauma to skin(koebner s phenomena),sunburn, exposure to cold, pregnancy ,smoking…These factors only in 1/3 of patients, other 2/3 starts spontaneously without abnormal predisposing factors (idiopathic).

Clinical features:

DLE is a disease of signs. localized DLE lesions occur only on the head or neck ,where as generalized or disseminated DLE lesions occur both above or below the neck. DLE lesions below the neck most commonly occur on the extensor aspects of the arms, forearms &hands although they can occur at any site on the body.

Variants:

1.Classical DLE: early lesions, sharply-demarcated, coin-shaped discoid erythematous plaques covered by a prominent ,adherent scales that extend into the orifices of dilated hair follicles .when the adherent scale is lifted from more advanced lesions ,keratotic spikes ,similar in appearance to carpet tacks can be seen to project from the undersurface of the scale (Carpet tacks sign) finger-like projections in pilosebaceous follicles =horny plugs occupying dilated hair follicles.

2.Atrophic DLE: with time ,months or years ,early DLE lesions typically expand with erythema and hyper pigmentation at the periphery ,leaving atrophic central scarring ,telangiectasia &

hypopigmintation.

Sites of involvement :cheecks 76% ,nose 57%,scalp 48% with scarring alopecia in 42.5% & non- scarring hair loss in 5.5%, ears 46%,oral mucous membrane 11% whitish erythematous patches affect buccal mucosa ,soft &hard palate ,even lips may be affected.

Nasolabial folds are not affected . some lesions on the face have a butterfly distribution.

3.Hypertrophic DLE: warty- like plaques covered with massive hyperkeratosis at the extensor surfaces of the forearms.

4.Lupus panniculitis(Lupus profundus): presented as a tumor-like or big lumps felt under the skin rather than seen, as a result of inflammation of fatty layer of the skin ,with time ,involvement of the underlying skin with fibrosis ,atrophy & scarring.

The term LE profundus is used for patients who have both Lupus panniculitis& DLE lesions, where as LE panniculitis refers to those having only subcutaneous involvement.

Frequent sites of involvement include the soft tissues of the head& neck, upper arm, chest, back, breasts, buttocks & thighs.

5.Mucosal DLE: occurs in approximately 25%of the patients with chronic cutanous LE, the oral mucosa is most frequently affected ,however ,nasal ,conjuctival &genital mucosal surfaces can be targeted.

6. Lupus telangiectoids:persistant, reticulate telangiectasia occurs on the face, neck, ears ,dorsa of the hands, breast& sides of the feet.

Healing occurs with punctate atrophic scarring.

7.some times, vitiligo-like or leukoderma-like hypopigmentation after healing of the classical DLE lesions.

Laboratory abnormalities in DLE:

Anemia ,leukupenia or thrombocytopenia may be found in about 1/3 of patients, ESR is raised in 20%, serum globulin is raised in 29%.

Antinuclear Abs are found in 35% ,they are more common in older patients ,in those who have had the disease for a long time and when there is extensive skin involvement ,but the titers are lower than those in patients with SLE, Indirect ImmunoFlourescence (IIF) detects nuclear homogenous, rim(peripheral), speckled &nucleolar patterns.

The incidence of anti-DNA Abs in DLE varies from 0-27%.

Lupus band test: in both DLE&SLE, there is impaired cell- mediated immunity because of decrease suppressor T-lymphocytes. B-cells are normal ,HelperT-cell stimulate B-cells to form immunoglobulin circulating in blood ,they might be deposited in tissues like skin . The examination by fluorescence microscopy (DIF) of frozen section cut from lesional or non- lesional ,sun-exposed or non sun-exposed skin &incubated with fluorescein -conjugated Abs monospesific for IgG, IgA, IgM& complement constitutes LBT.

In DLE LBT is +ve in lesions or involved skin only & -ve in normal skin , while in SLE LBT is +ve in involved &un involved, sun-exposed or sun-protected skin.

Prognosis:

Complete remission in the course of years can be expected in over 50%. The risk of developing SLE is about 6.5%,the risk is higher in patients with disseminated DLE than in DLE confined to the head &neck. 40-50% of patients with lupus profundus have or will develop signs of systemic disease at sometimes during their course.

In Iraq ,the percentage of DLE change to SLE is 5.5%& no case of lupus profundus changed to SLE. SCCs and less commonly BCCs occasionally occur in the scars of DLE, particularly the scalp ,ears ,lips and nose. From 54 patients of Iraqi stydy,1 case showed development of SCC in a scar located on the upper lip with a duration of disease around 20years.

Treatment:

1.Avoid precipitating factors if possible, use sun-screen creams, B-carotene tablets,

2.Moderate –potent topical steroid ,

3.Intra lesional steroids with quick results.

4.Local freeing or cautery of resistant lesions.

mg twice per day 5.Oral antimalarias:chloroquine,250

or hydroxychloroquinone 200 mg twice per day for short course .If remission so stop it ,given in summer and stopped in winter to avoid retinopathy which is permanent.

6.Systemic steroids in cases not respond to other treatments ,10-20mg prednisolone per day for short time and tappering .

Systemic Lupus Erythematosis

Systemic disease with pathological changes affect connective tissues & vascular system .there is fever ,rash, involvement of kidney ,lungs, heart , joints & other organs. It is commonly seen by dermatologist because of rash or by rheumatologists because of joints involvement, F:M ratio is about 8:1, HLA-B8 is increased in SLE ,where as HLA-B7 in DLE.

Creteria for classification of SLE:

1.Malar rash :fixed erythema over malar eminences, sparing nasolabial folds.

2.Discoid rash :erythematous raised patches with adherent keratotic scaling &follicular plugging .

3.Photosensitivity :skin rash as a result of unusual reaction to sunlight .

4.Oral ulcer: usually painless.

5.Arthritis:non erosive arthritis involving 2 or more peripheral joints ,characterized by tenderness, swelling or effusion .

6.Serositis:pleurisy or pricarditis.

7.Renal disorder: persistent proteinuria >0.5g/day or cellular casts.

8.Neurological disorder: seizure or psychosis .

9.Hematological disorder: Hemolytic anemia ,leucopenia ................
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