Natural Scleroderma Relief

Natural Scleroderma Relief

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Natural Scleroderma Relief Summary

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Systemic Sclerosis And Localized Scleroderma

The scleroderma spectrum of disorders encompasses a wide variety of disparate conditions whose unifying feature is the clinical presence of hard, tight skin, and pathological deposition of excessive collagen. Children and adolescents with scleroderma make up a very small subgroup of patients seen in a general rheumatology clinic, and the type and pattern of scler-oderma is different to that seen in adults. Localized scleroderma is far more common in pediatrics than systemic sclerosis, by a ratio of at least 4 1 (94). There is a significant association with trauma, not seen in adults (94). Childhood-onset scleroderma resembles adult disease in its female predominance, racial predisposition, histological findings, and heterogeneity of clinical expression, but differs in a number of ways. In contrast to the adult disease, childhood onset scleroderma is associated with normal parameters of vascular activation (such as von Willebrand factor, angio-tensin-converting enzyme, endothelial-1...

Localised And Systemic Scleroderma

Although patients suffering scleroderma do not show compromised D synthesis (Matsuoka et al 1991), vitamin D3 has been investigated as a therapeutic agent to moderate the excessive proliferation and collagen production typically seen in this condition. An in vitro study assessing the action of vitamin D3 on the behaviour of affected fibroblasts has confirmed a non-selective antiproliferative action (Boelsma et al 1995). To date, clinical studies have produced mixed results. Clinical trials focusing on generalised scleroderma have involved small numbers and produced promising Vitamin D 1317

Localized Scleroderma

Wasting Tongue

Localized scleroderma (morphea or linear scleroderma) involves the skin and subcutaneous tissue in one area, although there may be involvement of other organ systems. Presentation tends to be indolent, starting with superficial erythema of the skin that gradually spreads. There may be central clearing with a lilac ring appearance. With time, there is hardening of the skin or subdermal structures, leading to an ivory-like appearance of the tissue (95). Loss of hair and anhydrosis is common along with hypo-pigmentation or hyperpigmentation. Involvement of the deeper structures varies. Classification of localized scleroderma is based on clinical morphological findings and the depth of tissue involved (96,97). The terms localized scleroderma and morphea have often been used interchangeably, leading to confusion. Morphea is used by most rheuma-tologists to describe a subgroup of localized scleroderma that presents with oval-shaped patches or plaques characterized by thickening and...

Sclerodermatomyositis

Sclerodermatomyositis is an overlap disorder in which there is a band of tight skin that appears to be linear scleroderma, but also the muscle weakness, elevated muscle enzymes, and heliotropic rash (a rash in areas exposed to sun, often the face and eyelids) typical of dermatomyositis (see Chapter 14). The simultaneous occurrence of manifestations of two separate diseases in these children is unexplained. Laboratory abnormalities found in children with sclerodermatomyositis include a positive test for ANA. The routine blood tests may show a mild anemia, elevation of the ESR, and elevated muscle enzyme levels (CK and aldolase see Chapter 22). RF is rare, and anti-Scl-70 is normally absent. Although diffuse sclerodermatous changes, rash, and elevated muscle enzymes may occur in mixed connective tissue disease, children with MCTD do not have linear bands of tight skin, and the laboratory findings are different. Complications of sclerodermatomyositis are rare. The muscle inflammation and...

Linear Scleroderma en Coup de Sabre and Parry Romberg Syndrome

Linear scleroderma en coup de sabre is a particular form of linear scleroderma that may not truly be related to the other forms. Children with linear sclero-derma en coup de sabre have an area of thickened abnormal skin involving one side of the forehead and extending along the scalp toward the back. They usually do not have significant underlying problems. Parry-Romberg syndrome is a gradually progressive facial hemiatrophy. In a full-fledged case, there is significant deformity, with one entire side of the face smaller than the other. This is in sharp contrast to typical linear scleroderma en coup de sabre, where the abnormality is confined to the forehead. While the changes of linear scleroderma en coup de sabre are usually confined to the skin, in Parry-Romberg syndrome there is an increased frequency of underlying changes in the brain. The difference between linear scleroderma en coup de sabre and Parry-Romberg syndrome is best exemplified by involvement of the tongue in...

Systemic Forms Of Scleroderma Progressive Systemic Sclerosis

The form of scleroderma called progressive systemic sclerosis (PSS), also referred to as diffuse scleroderma, is the most severe form of the disease, with the subtle onset of skin tightening and in many cases shortness of breath. Children and Some children with scleroderma are first seen by their physicians because of chest pain. The distal esophagus is frequently involved in scleroderma, and the valve (sphincter) that keeps stomach acid out of the esophagus often fails to work properly. As a result, the chest pains are due to irritation of the esophagus by acid reflux. Occasionally, children present with chronic diarrhea and weight loss because the intestinal tract is affected by the scleroderma. In all of these cases, the key to the diagnosis is consideration of the possibility of scleroderma by a knowledgeable physician. The diagnosis of PSS is based on the characteristic findings on examination of the skin. In some cases, it is necessary to biopsy the skin for confirmation, but...

Scleroderma

Scleroderma Facial Features

As in SLE, changes in the skin are the most obvious manifestations of scleroderma in the head and neck. In systemic sclerosis, the skin changes can begin with a sometimes pruritic, edematous phase, as inflammatory cells and fibroblasts are activated and cytokines are released. The skin in the affected areas tightens, loses flexibility, and thickens. In systemic sclerosis, characteristic facies may be noted, with a pursed-lip appearance and reduction in the oral aperture (Fig. 4). After several years, thinning and atrophy occur. Pigmentation changes may occur as well, giving a salt and pepper appearance. The dramatic en coup de sabre lesion (Fig. 5) is rare and is a manifestation of localized scleroderma. A nondermatomal fibrotic band infiltrates skin, subcutaneous fat, fascia, and muscle in the midline of the face, giving the appearance of a sword wound. Telangiectasias may be prominent on the head and neck, including involvement of the palate, oral mucosa, and tongue (Fig. 6)....

Linear Scleroderma

Linear scleroderma differs from morphea in that the areas of involved skin form linear bands instead of irregular ovals. Its cause remains unknown. A typical case of linear scleroderma in a child presents as a tight band of skin on the top of one foot extending up onto the leg. It may also begin on the hand and arm. These areas appear pinkish at first and don't hurt. Later they become pale and harden. A small area of involvement may be of little concern, but if a large area is involved, tightening of the skin may cause the leg (or arm, toes, etc.) to become bent. We do not understand the patterns of linear scleroderma. Some children have one patch on one arm or leg. Others have several patches on one arm. Still others may have involvement of both the arm and the leg on one side, and the lesions may extend onto the trunk. Involvement of both sides of the body is unusual and should prompt careful investigation. Laboratory abnormalities in children with linear scleroderma are minimal....

Mixed Connective Tissue Disease

Mixed connective tissue disease is characterized by a combination of overlapping features of systemic lupus erythematous, scleroderma, and polymyositis. Typical presentation is with Raynaud's phenomenon, arthralgias, inflammatory myopathy, lymphadenitis, skin or mucosal lesions, and serositis. A key distinguishing factor of the disorder is a high titer of antibody to ribonucleoprotein, a finding absent in any of these three disorders (SLE, scleroderma, and polymyositis). Neurologic dysfunction is present in approximately 10 to 15 of cases, usually presenting with facial pain, facial paresthesias, or aseptic meningitis (96,97). Facial nerve involvement, although far less common than trigeminal nerve involvement, has been described and is felt to be an early manifestation of the disorder (98). Although CSF analysis has suggested an inflammatory involvement of

Pulmonary Function Testing

Obstructive lung disease occurs when something is getting in the way of the air flow. This is rare in children with rheumatic disease. Restrictive lung disease describes when the lungs themselves are not moving well. This can be due to weakness, but more often it means the lungs themselves are stiff. Most often, this happens when the lungs are involved by scleroderma, but it can happen with other rheumatic diseases.

TNF Blockade An Inflammatory Issue

Tumor necrosis factor (TNF), initially discovered as a result of its antitumor activity, has now been shown to mediate tumor initiation, promotion, and metastasis. In addition, dysregulation of TNF has been implicated in a wide variety of inflammatory diseases including rheumatoid arthritis, Crohn's disease, multiple sclerosis, psoriasis, scleroderma, atopic dermatitis, systemic lupus erythematosus, type II diabetes, atherosclerosis, myocardial infarction,

Pathogenic Autoantibodies

The putative role of antibodies to the acetylcholine receptor as a mediating of autonomic neuropathy in pSS was investigated by Waterman and colleagues.104 IgG autoantibody found in patients with both primary and secondary SS induces cholinergic hyper-responsiveness and detrusor instability on passive transfer to normal mice potentially through, compensatory up-regulation of postsynaptic M3R receptor number, consistent with the hypothesis that the overactive bladder in pSS is an autoantibody mediated disorder.101 Proof of a pathogenic role for anti-M3R antibody in autonomic dysfunction in pSS will require purification and characterization of the antibody. Conventional immunologic techniques have not confirmed detectable binding of pSS IgG to M3R, and studies to date have relied on biologic assays that have proven difficult to replicate.105 The most compelling evidence for functional M3R antibody in pSS remains a bioassay capable of detecting circulating antibody at concentrations...

Muscle Enzymes Creatine Kinase and Aldolase

Creatine kinase (CK) is an enzyme produced by skeletal muscles, the heart muscle, and the brain. Although it may be elevated in adults who have recently suffered a heart attack, the most frequent cause of elevations in childhood is muscle inflammation. This may occur in children with dermatomyositis, scleroderma, or mixed connective tissue disease. Sometimes the CK is elevated with extensive exercise, but in these children the level goes back to normal after two weeks' rest. The level of CK is also elevated after some viral infections. Some children have elevated CK levels that persist despite rest and for which no explanation can be found see Chapter 14, on dermatomyositis.

RLS and rheumatic diseases

In the rheumatoid arthritis (RA) patient population, increased prevalence of RLS has been reported by several investigators. Reynolds et al studied hospitalized RA patients employing a 'control' group of osteoarthritis (OA) patients, and found a 30 prevalence of RLS in RA compared to 3 in OA (Reynolds G et al, 1986). A subsequent study again comparing RA and OA found comparable prevalence rates for RLS of 25 in RA and 4 in OA (Salih AM, et al, 2004). Auger et al in their study of MS patients employed a RA patient group for contrast, finding a prevalence rate for RLS of 31 in their RA group (Auger C, et al, 2005). A more recent study employing the 2003 IRRLSG criteria for RLS found a prevalence of RLS of 27.7 in RA and a prevalence of 24.4 in OA patients (Taylor-Gjevre, et al, 2009). Increased frequency of RLS has also been reported in other rheumatic disease populations, including Sjogren's Syndrome, scleroderma and fibromyalgia patients (Taylor-Gjevre, et al, 2011). A recent study of...

Immunosuppressive Drugs

Or one of the newer biologics discussed below (although the biologics are classified separately, they are in fact immunosuppressive). The majority of children with arthritis do not require immunosuppressive drugs, but children with more severe arthritis will do much better if they are appropriately treated and their disease is brought under good control. Stronger immunosuppressive drugs are commonly used for children with illnesses such as systemic lupus erythematosus, dermatomy-ositis, scleroderma, polyarteritis nodosa, and Wegener's granulomatosis.

Partial Mastectomy and Axillary Dissection

The majority of patients with intraductal carcinoma or Stage I and II invasive cancer are candidates for a partial mastectomy. Absolute contraindications to the procedure are multicentric carcinoma, prior irradiation to the breast region, first or second trimester pregnancy, diffuse indeterminate microcalcifications on a mammogram, and the inability to achieve negative margins after an adequate number of surgical attempts. Relative contraindications are a large tumor-to-breast ratio and scleroderma or systemic lupus. Patients with invasive carcinoma who are clinically node negative should have their axillary nodal status determined by sentinel node biopsy or axillary dissection. Those who are clinically node positive require axillary dissection.

Intestinal Pseudoobstruction

Intestinal pseudo-obstruction encompasses several intestinal motor disorders characterized by episodes that suggest intestinal obstruction because defecation stops and abdominal distension, pain, and vomiting occur, but in which no mechanical obstruction is found. It may be due to primary abnormalities of the visceral muscle or nerves or be secondary to chronic renal failure, hypothyroidism, diabetes mellitus, amyloidosis, scleroderma, or muscular dystrophy. There is no effective treatment that is specific for intestinal pseudo-obstruction. If the patient has bacterial overgrowth, this should be treated with antibiotics. If nutrition is impaired, administration of liquid, low-residue feeds enterally is required rarely, parenteral (intravenous) feeding is necessary.

Other Overlapping Conditions

Somehow all of these diseases that look so different in their typical forms are interrelated, but we clearly do not understand the connections. I have seen several children who simultaneously have skin lesions of morphea on their backs and abdomens, indentations typical of linear scleroderma en coup de sabre on their foreheads, ocular inflammation such as that seen in children with juvenile arthritis, and areas of linear scleroderma on their legs. Other overlap conditions may also occur.

Differential Diagnosis Of Mctd And Laboratory Findings

As noted, the clinical manifestations of MCTD overlap with dermatomyositis, SLE, JA, and early scleroderma. Consequently, physicians must rely more heavily on laboratory findings to confirm this diagnosis. The source of much of the confusion in diagnosing MCTD results from the unclear nature of the disease itself. Indeed, it is quite possible that we will ultimately realize that at least two different diseases have been grouped together as MCTD. As previously mentioned, children with MCTD often have many findings that are also seen in dermatomyositis and scleroderma. In more severe cases, children with MCTD often have Gottren's papules (sores over the knuckles) and nail fold capillary abnormalities. Although children with MCTD have elevated muscle enzymes and rash, they do not tend to evolve into dermatomyositis. However, MCTD may develop into scleroderma over a period of years. The presence of antibodies to Scl-70, or anticentromere antibodies, strongly suggests the diagnosis of...

Complications Of Raynauds

It is also important to recognize that children with Raynaud's phenomenon may have increased reactivity in other blood vessels. This is a rare complication, but I have cared for children with scleroderma who had chest pain and cardiac abnormalities because their coronary arteries constricted whenever their fingers blanched because of Raynaud's phenomenon. If the internal organs are being deprived of blood flow, the Raynaud's must be treated aggressively to prevent damage to these organs.

Rheumatoid Arthritis

It is important to recognize that discrete rheumatologic diseases share clinical features, and the individual patient may have evidence of more than one disease at a time. Thus, patients with SLE or scleroderma may have inflammatory myositis. Such overlap syndromes require diagnostic vigilance, but often result in therapeutic choices that are particular to the disease manifestation rather than the original diagnosis.

Treatment

Autoimmune disorders such as those discussed in this chapter are generally treated with immunosuppressive therapy. Corticosteroids are a mainstay of therapy in all of these disorders, but doses may vary widely, depending on the severity of disease manifestations. Among other immunosuppressives, those with more serious potential side effects are reserved for more severe disease manifestations. Often, however, the dermatologic manifestations of SLE and DM can be treated by hydroxychloroquine. This is a long-acting anti-inflammatory agent, not generally considered immunosuppressive, whose precise mechanism of action remains unclear. Hydroxychloroquine is frequently used alone or in combination with immunosuppressive therapy when skin rash is present however, it is not effective for the skin changes of scleroderma or the myositis of DM PM.

Summary

Systemic rheumatic disease may present with a variety of manifestations in the head and neck regions. Especially important among these are the dermatologic findings of SLE, DM, and scleroderma. Rashes characteristic of these disorders may also arise later in the course of the diseases. Some of the more frequently reported manifestations to be aware of are autoimmune hearing loss, especially in SLE esophageal dysmotility in scleroderma oropharyngeal and esophageal involvement in DM PM and keratoconjunctivitis sicca and cervical spine involvement in RA.

Overlap Syndromes

Children and adolescents can present with signs and symptoms that are characteristic of more than one connective tissue or inflammatory disorder. This occurrence is more common in children and adolescents compared to adults. In our experience, children's signs and symptoms may evolve over time from those of one connective tissue disease to another. For example, combinations of features of JIA, systemic lupus erythematosus, juvenile dermatomyositis, scleroderma, and vasculitis are seen. These children are best described as having overlap syndromes or undifferentiated connective tissue disease. Treatment should be tailored to organ involvement and underlying pathophysiology rather than be disease specific.

Immunity

However, there is also preliminary evidence that iron may be implicated in the pathogenesis of auto-immune disorders, including SLE, scleroderma, type 1 diabetes, Goodpasture syndrome, multiple sclerosis and RA (Bowlus 2003). Current evidence suggests that moderately elevated iron stores may be associated with an overall increased risk for cancer, especially colorectal cancer (McCarty 2003). Additionally, it has been proposed that iron may increase HIV replication and the rate of progression of HIV infection, although doses of 60 mg of elemental iron twice weekly for 4 months did not appear to affect HIV-1 viral load in clinical studies (Olsen et al

Systemic Sclerosis

While 84 had classical focal sialadenitis with focus score of 1 or more, only 18 had concomitant fibrosis. In terms of complications, primary Sjogren's syndrome showed a somewhat increased prevalence of cryoglobulinemia, while Sjogren's syndrome associated with systemic sclerosis more often was complicated by peripheral neuropathy and additional autoimmune disease or autoantibodies, not typical for either primary Sjogren's syndrome or systemic sclerosis. On the other hand, Sjogren's syndrome may be protective against systemic sclerosis-associated pulmonary fibrosis, a finding in two recent French studies.11,29 Limited systemic sclerosis was predominantly associated with Sjogren's syndrome in these studies (81 and 95 respectively).11,29 In summary, the restricted data on Sjogren's syndrome in systemic sclerosis indicate that symptoms of dryness are due mainly to fibrosis of the glands and thus due to scleroderma itself, while a limited number of patients...

Motor Disorders

Gastric stasis refers to the delayed emptying of gastric contents and this results in patients experiencing early satiety, bloating, nausea, and vomiting. Endo-scopy can confirm the presence of gastric stasis by finding retained food after an overnight fast. Systemic disorders such as scleroderma and diabetes mellitus can have neuromuscular effects that can affect the stomach. Neurological disorders can likewise cause gut dysmotility.

Specialized Tests

The pattern of ANA test results can be significant. Homogeneous pattern ANA occurs in many different people and is not disease-specific. Speckled pattern ANA is also common at low titer, but at higher titer it may be associated with mixed connective tissue disease or scleroderma. Rim pattern (also called shaggy pattern) is almost always a sign of active lupus. Anticentromere and anti-Scl-70 antibodies are associated with forms of scleroderma. Anticentromere antibodies are often present in children with CREST syndrome, and anti-Scl-70 antibodies may be present in children with systemic scleroderma. These antibodies are very significant if they are present. Most children who test positive for the antibodies will ultimately turn out to have the disease. However, I have seen false positive results and low-titer positive results that have turned out not to mean anything. Adult-onset rheumatoid arthritis (RA) by definition occurs after sixteen years of age. However, since the disease does...

Epidemiology

There is an estimated 2589 incident cases of rheumatic conditions per 100,000 population in the 0-15 age group, and 8935 per 100,000 population in the 16-24 year old age group (1). However, this estimate is not exhaustive, and only covers 10 musculoskeletal conditions selected as the most common or characteristic of their group. Specific adolescent data (10-19 year old age group) are lacking. If this group of 10 conditions is restricted to those requiring continuing secondary health care, (childhood arthritis, SLE, gout, and scleroderma) there is an estimated 129 and 188 prevalent cases per 100,000 population in the 0-15 and 16-24 year old age groups respectively, according to data available (1). This chapter aims to provide an overview of adolescent rheumatolo-gical conditions not represented in other chapters in this book, including vasculitis, juvenile dermatomyositis, scleroderma, musculoskeletal infections, systemic diseases presenting to rheumatology and musculoskeletal...

Laboratory Tests

When the laboratory reports the WBC count, it also reports the percentage of each different type of cell neutrophils, lymphocytes, monocytes, basophils, and eosinophils. Normally, the neutrophils make up the greatest percentage. In young children (under the age of five), lymphocytes are often increased. Eosinophils are often increased in people with allergies. They may also be increased in children with parasitic infections. Certain uncommon rheumatic diseases (such as eosi-nophilic vasculitis, some cases of scleroderma, and Churg-Strauss syndrome) are also associated with a significantly increased number of eosinophils. Significantly increased numbers of monocytes or basophils are very rare.

Of North America

Sjogren's syndrome (SS) is atypical among the major autoimmune rheumatic diseases encountered by the rheumatologist. The predominance of ocular, oral mucosal, and exocrine gland pathology accompanied by the copresence of multiple organ-specific autoimmune disorders results in a somewhat unfamiliar diagnostic and therapeutic landscape. The lack of universally accepted diagnostic, classification, and outcome criteria, as well as the need for close collaboration with ophthalmologic, dental, and otolaryngologic subspecialists for optimal patient management, all contribute to challenges in management. Nevertheless, SS affords rheumatologists and immunologists an opportunity to understand the pathogenesis, long-term evolution, and outcome of an autoimmune disease that possesses both organ-specific and systemic features. In addition, the frequent co-occurrence of SS with other major rheumatic diseases, such as rheumatoid arthritis, systemic lupus erythematosis, and scleroderma, affords a...

Treatment Of Mctd

For some children with MCTD, it is not possible to reduce the corticoster-oid dosage to a satisfactory level. Methotrexate seems to be an effective steroid-sparing agent for these children. Most often it is necessary to treat them with higher doses of methotrexate, as are used in dermatomyositis and scleroderma (i.e., up to 1 mg kg week, 50 mg maximum). The key to successful treatment of MCTD is early recognition of the children who are at high risk for progressive disease. Children in this group who are treated aggressively generally do well. Stronger immunosuppressive agents rarely are necessary for the treatment of children with MCTD unless the condition is evolving toward scleroderma. For children who do not respond to or tolerate methotrexate, azathioprine or mycophenolate mofetil may be considered. In the small subset of children who develop kidney damage, treatment with intravenous cyclophosphamide may be necessary and is often helpful. Children who progress to scleroderma are...

Morphea

Morphea is the most common form of scleroderma in childhood. Morphea consists of areas of thickened skin commonly on the body (trunk), but sometimes on arms or legs. Its cause is unknown. Families usually notice a patch of pink and irritated skin that looks like many common skin conditions. If the patch is due to morphea, it typically does not itch or hurt. Often the area of reddened skin does not attract any particular attention until it has persisted for several weeks. It will not improve with treatment for skin infections, but it might briefly look better if it is treated with corticosteroid creams. Many parents worry that morphea lesions may be the first indications of systemic scleroderma. This is rarely, if ever, true. In most children who develop systemic scleroderma, there is strong evidence suggesting systemic disease from the beginning. If there are no such indications, I recommend only periodic monitoring once the initial evaluation is complete. The long-term outlook for...

Penicillamine

D-penicillamine clearly affects the course of JA, morphea, and scleroderma, but it has a very slow onset of action and a high frequency of toxic reactions (routine blood test abnormalities, skin rashes, kidney irritation, and neurologic abnormalities). Because of these drawbacks, D-penicillamine was rapidly replaced by methotrexate for the treatment of children with arthritis. D-penicillamine may still have a role for children who have not responded to other medications, but it is rare to see it used for arthritis in childhood. D-penicillamine has long been the standard recommended therapy for children with morphea and scleroderma (progressive systemic sclerosis). Although some physicians report good effects, most physicians with extensive experience find D-penicillamine to have a high frequency of toxicity and only minimal efficacy. The right therapy for scleroderma remains controversial, but I prefer methotrexate. For most children with scleroderma or morphea, methotrexate has...

Cyclophosphamide

Intravenous cyclophosphamide has been used for the treatment of children with systemic-onset JA who failed all other therapies. Although there are a few reports in the literature describing success, it is not generally utilized because the treatment is difficult for the child and family and it is not universally successful. With the current availability of many new therapies, the role of cyclophospha-mide in the treatment of children with arthritis is extremely limited. However, it remains a mainstay of therapy for children with vasculitic diseases, including SLE, dermatomyositis, and scleroderma. The future of cyclophosphamide in the therapy of rheumatic diseases may well lie in combining it with newer biologic agents, allowing a significant reduction in the total amount of cyclophospha-mide given.

CREST Syndrome

CREST syndrome is a variation of systemic scleroderma that has several peculiar aspects. The name CREST is an acronym that comes from the findings of calcinosis (pieces of calcium under the skin), Raynaud's phenomenon, esophageal problems, sclerodactyly (tight skin on the fingers), and telangiectasias (small red spots due to abnormal blood vessels in the skin). The key findings that distinguish CREST from PSS are the presence of telangiectasias and anticentromere antibodies.