SCLEROSIS LOCALIZED SCLERODERMA SYSTEMIC SCLERODERMA lichen sclerosis et sclerodermiform S YNDROMES Sclerosis includes all these pictures.

The term "sclerosis" means "hardening of the skin," so we're talking about a diverse group of processes in which essentia lly is the hardening of the skin. SCLERODERMA It is a chronic disease that do not yet know the etiology. It is characterized b y hardening of the skin and can acompañarse1 multiorgan some alterations. PATHOG ENESIS There are two main factors: a) immune disorders: it seems that there are autoantibodies and autoimmunity b) If it is very clear that there are vascular abnormalities. These are varied: the re is a decrease in skin vessels, dilated vessels and other alterations characteristic o f vessels in other vascular thickening, intimal proliferation and decreased vasc ular caliber vascular obstruction → → necrosis by ischemia that this produces c) All this leads to increased fibroblast activity resulting → fibrosis of the dermis. The latest theories say they are common immunological fi broblasts select those that will produce a much denser collagen fibers than norm al, resulting in fibrosis. CLINICAL There are two possible forms of presentation : A) B) Systemic Scleroderma: it will present with cutaneous and visceral involvement. L ocalized scleroderma: there is only skin involvement A) Systemic Scleroderma Within it there are two main types that differ in the presentation of skin lesio ns: - Diffuse or proximal starts affecting the proximal parts (the trunk and roots Member) - Limited = acrosclerosis. Start with the acrosclerosis members in parts progressing distal centripetal B) Localized scleroderma is a hardening of the sk in, but in which we can also find several ways: Localized: drop, plaques, genera lized linear A) Systemic scleroderma Skin involvement is centripetal or centrifugal accompanied by lesions in the sys temic and visceral injuries are: d esophagus and other parts of the gastrointest inal tract accompanied by dysphagia. They can also occur: intestinal polyps, hia tus hernia and gastric disorders. Systemic involvement is common for the two for ms which we have quoted above: Esophageal stricture + liver cirrhosis portal hyp ertension (esophageal varices, hematemesis) pulmonary fibrosis, heart disease, k idney disorders (nephrosis), polymyositis, arthralgia. Keratoconjunctivitis "sic ca" All of this systemic disease affects the severity and prognosis for survival as it affects the quality of life very difficult to lead a normal life. But is the skin involvement that gives the key to the diagnosis of one form or another. It mainly affects women (4 / 1) and middle-aged 40-60 years. • Scleroderma skin inv olvement fuzzy system (5%) is less frequent. Start with hardening of the skin on the trunk and roots of the members (proximal) to the knees and elbows.

• Systemic Scleroderma LIMITED (95%): acrosclerosis. You can start with acrosclerosis, ie hardening of the skin from the metacarpopha langeal joints to elbow in the upper extremities and from the MTP to the knee in the lower extremities, ie begin in the distal parts of the limbs and progress c entripetal way. And it appears the CREST syndrome. This name corresponds to the acronym for calc inosis, Raynaud, esophageal sclerosis, sclerodactyly, telangiectasia. The skin involvement is the key to the diagnosis of these two forms. Let's see t he features of both forms, linking them together to see the fundamental differen ces: sclerotized. SYSTEMIC S. Visceral Sclerosis Raynaud ANA Forecast Evolution DIFFUSE <1 years Trunk, proximal Early, severe Anti-Scl-70 Quick Malo acrosclero sis> 1 year or absent Acral anticentromere Well Slow Delayed These two forms of systemic scleroderma input differ in prognosis, much worse th at of diffuse scleroderma than the form of acrosclerosis. Diffuse scleroderma of ten begins with a Sd. Raynaud. Moreover, when it appears we know that within one year will be established scleroderma. However, in the acrosclerosis we have a h istory of Sd. Raynaud's years of evolution. In the diffuse visceral involvement is early and severe, while the acrosclerosis is late. The table also can see the ANA (antinuclear antibody) that will be present in every form. And evolution, l ogically related to both forms,€acrosclerosis in life to be easier because the e volution is slower. CLINIC It starts with a Sd. Raynaud (90% of cases). It is di fficult to diagnose the patient. To a cold environment when the patient hands dipped in cold water or to any exte rnal cause that promotes vasoconstriction, there is a very sudden and intense va soconstriction in the acral parts. Therefore, initially there will be pallor (For vasoconstriction) of the parties acral (hands and feet), the skin becomes really white. Once past the pallor, cyanosis appears the tissue ischemia caused by this vasoco nstriction, get little blood and becomes purple or violet skin. After all this h appens a reactive hyperemia resulting in an intense BLUSH in affected areas (com pensatory vasodilation). Most often it is triggered by cold, but sometimes can be triggered by emotional stimuli. Early stages: Apart from this Sd. Raynaud's in the forms of acrosclerosis going to see intense that it leaves no pitting edema in the extremities (fingers). These are called

"FINGERS IN SAUSAGE", begin to be swollen but rigid, is difficult both to extend and flex. In the images we saw a shining fingers, with some wrinkles that should not appear on fingers extending into the diffuse form affects mainly the trunk and proximal areas. In picture is difficult to perceive this, it is felt necessary to note that skin thickening o f the skin. The tightening of the skin will determine the movement of the joints (the teacher says it's like an armor that does not let you move) hindering the extension of the hand being this semi-flexed. As the disease progresses: - There are no sausage fingers quite the contrary, those fingers are edematous s uffering a number of other changes, such as sharpening, it thins the epidermis a nd dermis is sclerotic. - The nails are being extremely sclerotic narrowing, it is as if we played a "fi nger of wood." - Some patients come to be practically unable to move their finge rs. And some of them even produced Ulcers pressure of the bones with the skin an d the continuous contact with various objects that are managed in everyday life. They are very torpid ulcers, and when healed often leave a scar. - In addition, there is necrosis of the distal parts of fingers due to obstructi on of cutaneous vessels and may even lose the distal phalanx in extreme cases. T hese lesions eventually heal, leaving a depressed scar in the distal fingers, sc ar punctate in the fingertip, which is very characteristic. Patients are useless , often can not even dress. - The face is a FACIES Expressionless, by hardening of the skin that also affect s the face. The wrinkles are less noticeable in both the face and neck, there is a kind of rejuvenation, disease course, which lasts a short time. - At the mouth can also be observed alterations. The skin around the mouth loses elasticity, like the rest of the face, which will result in the appearance of a radial folds around the mouth (as a sort of gathering around the lips) and also decreased mouth opening, patients have more difficulty opening the mouth, so th at they become low-expression by affecting the mimic musculature. - Also are numerous telangiectasia (red spots) for dilation of dermal vessels. - In addition, excessive collagen production is "choking" the skin appendages, s o it is possible to see the disappearance of these appendages: ▪ In the pilo-seb aceous follicles: the appearance of alopecia (hair follicles and is constrained sebaceous glands affected by the abundance of sclerotic tissue) ▪ In the sweat g lands: the emergence of anhidrosis diagnostic criteria must meet a major criteri on or two minor: ■ Mayor: ■ proximal skin sclerosis Juvenile Sclerodactyly: sharpening of pinpoint scars fingers in pads (due to necrosis of the distal ends) bilateral pulmonary fibrosis TREATMENT Treatment is difficult, frustrating and inefficient. Sclerosis progresses we see that although we can influence it. a) Symptomatic:

A) Rehabilitation articulate: this is to try to avoid linking the effect of anky losis already involves the hardening of the skin, the effect of ankylosis which produc es immobile joint. 2) Protection from cold, this is very important 3) Vasodilators: this especially if Sd. Raynaud's could even pentoxicilina that helps improve peripheral circulation. (the latter is the comm ittee last year but the teacher did not mention it) 4) therapeutic measures for: treat gut disorders such as esophagitis, hypertensi on, renal failure, etc. 5) local treatment of ulcerative lesions (this does not say) b) To improve the process sclerosus: Different drugs have been tried: D-penicillamine: increases the solubility of dermal collagen, but with few resul ts Colchicine: interrupts the activation of fibroblasts corticosteroids, immunos uppressants (such as methotrexate, cyclosporine) B) LOCALIZED SCLERODERMA E. L. Localized IN PLATES This form of scleroderma is a fundamentally different problem to the patient that suffers, it is much more bearable. It is also more c ommon in women. It consists of a hardening of the skin in a localized area of the body. Plates a ppear more or less rounded or oval. The lesion begins with a rash, which often g oes unnoticed and then appears in the central zone plate sclerosis, hardening of the skin. It is an area where the skin is difficult to tweak, and this differen ce is clearly manifest if after trying to pinch pinch an area of healthy skin. S ometimes it is even possible to see in this central zone some depression. In this central area the skin is more clear, white or yellow (also can give us t he impression that it is somewhat brighter), leaving a more violet beltway known as RING LILAC. All these characteristics are typical of Morpheus in place. But these morphs into plaque, which are located, may be appearing throughout the yea rs, and go on overlapping plates and get existing ones to join together with oth er leading to enormous plates that affect large areas of skin. The LS can be gen eralized leading to a form very similar to systemic scleroderma, generalized mor phea, with hardening of the body, limited joint mobility, and even alopecia. Alt hough this is very rare situations. E. L. LINEAR - The "coup de saber" (en coup de saber) is a hardening of the skin of linear morphology. By observing this injury gives the impression that the pa tient had received a saber in the face and has a scar from it. It is an injury-l ooking and somewhat depressed scar that extends from the scalp through the face of all, first on the forehead, up to and including the chin. At the end can prod uce a symmetry of the face. It affects the skin and the fascia below and may even reach the cartilage and bone atrophy resulting from under all these structures. Although it may seem, it is not so rare injury.

Monomiélica Scleroderma is a rare lesion. It affects a single limb (asymmetry), eg one leg. Looking at the two legs together shows the difference in volume of t he affected leg to heal. The leg, but may also start presenting sclerotic phenom ena, is twice the diameter of the patient, which is much smaller. By touching th e diseased leg seems almost to be playing a piece of wood. 40% of patients with localized scleroderma have a positive ANA (+), possibilities of phenomena Raynaud, and even nephritis. HlSTOPATHOLOGY This section comprises the slides two slides which are histological sections. In one of them puts localized scleroderma: morphea, and puts the other systemic sc leroderma. But the teacher in class said nothing of this distinction, but was co mmenting on the histopathology rather commenting on the photographs. The epidermis is atrophied. Below the dermis is observed epidermis homogenized b y excessive production of thick collagen fibers resulting in a smooth and homoge neous dermis. There is also a reduction of skin appendages. On the slide sweat. At first this sclerosis is not so impressive. Shown inflammatory infiltrates aro und appendages and as it is establishing sclerosis will be disappearing these ap pendages, even to disappear dermohipodérmico limit (in the histological section we recognize the place where are the outlines of the annexed). The hypodermis is slowly being engulfed by the dermis, until it reaches the point of being comple tely flooded by the excessive production of fibers. you can see some outlines of what had been glands Inflammatory infiltrate is squeezing the skin attached to that eventually disapp ears. In another slide you can see remnants of hair and a gland on a small island of f atty tissue,€and circling around thick collagen fibers. Phenomena are also observed vascular obstruction: intimal thickening causing a decrease in the size of the vessels and inflammatory infiltration arou nd the vessels leading to fibrosis. Here is a cut to a much greater increase in which we can see thick hyalinized co llagen fibers Sclerodermiform SYNDROMES Receive this designation because of its similarity with scleroderma. It consists of a progressive hardening of the skin more or less diffuse. Toxic syndrome Rap eseed oil 1981 Because of an adulterated oil syndrome occurred in the residual phase which was a very intense sclerosis of the skin: Table ▫ very severe multisystem which many people died and others suffered a: ▫ Phase chronic graft versus host disease sc lerodermiform . immunocompetent (BMT) versus-host immunosuppressed: chronic phas e Graft Not all have sclerodermiform sclerodermiform phase, but some of them together an

d then given in the late phase of GVHD. It occurs when cells in the graft that h ave been introduced to the subject immunosuppressed structures act against the l atter and also he is unable to defend themselves. We saw a series of images of t his phase sclerodermiform of graft versus host disease: amyotrophic intense imag es in the trunk, with a narrow band around the trunk completely hard; images atr ophic areas and plaques on the arms fully hard (the teacher said that that patie nt could not wear, even button his shirt) and also a patient with hands totally full of sclerotic, unable to move anymore. Sclerodermiform Syndromes professionals: For various toxic: PVC, perchlorethylene, pesticides, fumes from epoxy R IMPORTA NT: All sclerosis lesions need to be touched because it is perceived as perfectl y. So it is very important to palpation of the lesions. Lichen sclerosis et It is also a picture of multiple sclerosis but is poorly understood etiology. It is not often like the above. Etiology is unclear. Is believed to be due to: Previous infection: autoimmunity is unsubstantiated but we do know that previous infections due to hormonal factors: in fact when vulvar lichen sclerosus affect s girls at puberty (in the wake hormone) improves greatly. Even pregnant women a re also improved in what happens after pregnancy to recur. Diabetes also is a factor to consider. In women is more common in because they a re more frequent genital infections: candidiasis ... Immunologic factors Start with punctate lesions or lenticular hypochromic. In these lesions is diffi cult to explore sclerosis because they are very small, although we could notice a difference to the feel of consistency, feel a hardness. Sclerosis can be seen pinching the skin. These lesions are grouped in one area of the skin: forearms, trunk ... They start with small bumps in the form of white spots, sometimes surr ounded by an erythematous ring. It may also appear as plaques with indurated areas in central and scar appearanc e in the most external. Sometimes there is an increase of pores and comedones ap pear as inside the plates before they disappear sclerosis. As we see lichen sclerosus may have skin involvement, although it can affect the mucosa, especially in the genital mucosa. Male genitalia: usually begins by aff ecting the glans. Plates appear yellowish, waxy and very hard consistency. And t hen, as it will affect the penis, there is a hardening of the foreskin, which of ten leads to paraphimosis. These injuries are very annoying and may eventually l ead to squamous cell carcinoma. In the following photo we see the male genitalia : foreskin and glans. Coloration appears white / yellow, with intense induration Female genitalia: In this case affects both adult women and girls. In the case o f girls can be autorresolutivas lesions may disappear as they approach puberty a re pearlescence injuries, which often come to producing disappearance of the lab ia majora, and retracting long occluding the vaginal opening. Typically these in juries occur with severe itching, and difficult to treat. Besides affecting the vulva may also affect the inguinal folds. Treatment often do with testosterone t opical (cream) which greatly improves sclerosis, but girls in menopause or proge sterone.€It is also frequently administered: potent topical corticosteroids

Girl: pearly white or color. Cracks may also occur. Plates inguinal regions with a purple halo and a white center / pearly. They are accompanied by intense itching (especially women). This lack of flexibility of the skin makes it harder disparemia sexual activity. As they move it changes the morphology vulva clitoral hood disappears and is con stricted. Until today it seemed kind of short but not ... Greetings to all my girls they k now who they are, children too of course.