Natural Scleroderma Relief
Diffuse scleroderma is an uncommon systemic disease of unknown etiology that is characterized by a long course of progressive disability due primarily to lack of mobility of the areas and the organs that are affected. There are two clinical forms of this disease. In the diffuse form the skin becomes hidebound, the esophagus and the gastrointestinal tract semirigid, and the lungs and the heart fibrosed. In the CREST form (calcinosis, Raynaud's phenomenon, esophageal involvement, sclerodactyly, and telangiectasis), the course is relatively benign because of minimal internal organ involvement.
Scleroderma, or systemic sclerosis, is a connective tissue disorder whose hallmark is tissue fibrosis. Systemic scleroderma is characterized by progressive fibrosis of the skin, lungs, heart, gastrointestinal tract, and kidneys. An association of limited skin involvement and late visceral involvement is known as CREST syndrome (calcinosis, aynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasias). A localized form of scleroderma, known as linear scleroderma or morphea, exists when fibrotic changes are localized to the skin it does not involve the GI tract.30 As with many other connective tissue diseases, scleroderma affects women three times as often as men, and the incidence in the United States is estimated to be 1 100,000 persons per year. The incidence peaks in women between the fifth and sixth decades of life.31 Clinically, patients with systemic involvement present earlier in the disease course with mostly skin complaints. The histopathologic features...
The partial or complete absence of sweating, seen in ichthyosis, extensive psoriasis, scleroderma, prickly heat, vitamin A deficiency, one form of ectodermal dysplasia and other diseases. Partial anhidrosis is produced by many antiperspirants. Annular atrophic plaques of the face (Christianson's disease). Rare sclerotic annular plaques mainly on the face. Chronic, progressive, recalcitrant to treatment with unknown cause. May be a variant of scleroderma.
Cutaneous lesions are prominent in porphyria cutanea tarda and include facial plethora, hyperpigmentation of exposed areas, hypertrichosis (this may be the presenting complaint in women, who sometimes are then treated with estrogen ), blisters, erosions mainly over the dorsal hands (secondary to very fragile skin), milia, and localized areas of scleroderma (later may occur in nonexposed areas). Acute abdominal crises or other neurologic attacks do not occur in PCT, even after drugs such as barbiturates and sulfonamides. Such crises may occur, however, in the less common inherited disorder, variegate porphyria. The skin lesions in variegate porphyria are very similar to those-in PCT.
Mononeuropathies are rare in children compared with adults. Median neuropathies at the wrist may be associated with idiopathic carpal tunnel syndrome, but may also occur in the setting of acute trauma, chronic trauma via sports, mucopolysaccharidosis (Hurler Scheie, Hunter, and Maroteux-Lamy), mucolipidosis (types II or III), or scleroderma. In some cases, the median neuropathy may be the first specific finding that indicates a systemic condition. Thus, the possibility of a mucopolysaccharidosis or mucolipidosis should always be considered if a child is diagnosed with a distal median neuropathy. Proximal median neuropathies seem to occur at least as often as median neuropathies at the wrist, and are typically caused by trauma, such as a fracture of the humerus or a laceration. The classic clinical picture of a median neuropathy occurs in some of these patients but is not universal. A careful needle EMG is important in cases of median neuropathy to investigate the possibility of a...
Examples of causes of diffuse sensory neuropathy include a dysproteinemic state (IgM monoclonal gammopathies with antimyelin-associated glycoprotein), amyloidosis (generally small fiber), hereditary, diabetes mellitus, uremia, hypothyroidism, immunological (scleroderma, sarcoid), and toxins. A generalized peripheral neuropathy can show selective involvement of certain fiber types such as large myelinated fibers. In these circumstances there will be a dissociated sensory loss with a deficit of vibration and proprioception while sparing pain and temperature on sensory examination. Examples of these conditions include Friedreich's ataxia, Charcot-Marie-Tooth disease, uremia, and Guillain-Barre syndrome.
Most mothers have or will have signs of lupus erythematosus or Sjogren's syndrome. scleroderma Figure 25-4. Scleroderma. Diffuse scleroderma of hands. (Burroughs Wellcome Co.) Figure 25-5. Scleroderma. (Ortho Pharmaceutical Corp.) Figure 25-5. Scleroderma. (Ortho Pharmaceutical Corp.) Figure 25-6. Scleroderma. (Ortho Pharmaceutical Corp.) Figure 25-6. Scleroderma. (Ortho Pharmaceutical Corp.) As in lupus erythematosus, there are two forms of scleroderma that are clinically unrelated, except for some common histopathologic changes in the skin. Localized scleroderma (morphea) is a benign disease. Diffuse scleroderma (systemic sclerosis) is a serious disease.
PCI can also be classified as primary (idiopathic) or secondary, the latter being associated with a wide variety of both gastrointestinal and nongastrointestinal lesions ( Table 32-1 ). PCI of the left side of the colon is usually idiopathic, whereas PCI of the small bowel or ascending colon is usually secondary. 1 I 1 Secondary PCI accounts for about 85 of reported cases and has been described in association with chronic obstructive pulmonary disease, high positive-pressure ventilation, necrotizing enterocolitis, pseudomembranous enterocolitis, diverticulitis, intestinal strangulation, appendicitis, gallstones, volvulus, pyloric stenosis, peptic ulcers, Crohn's disease, ulcerative colitis, esophageal stricture, tuberculous enteritis, scleroderma, blunt abdominal trauma, diaphragmatic hernia, jejunoileal bypass, steroid use, chemotherapy,
The primary causes of fibrosis are diverse and include toxic vapors, inorganic dusts, drugs, and radiation (4,6). Physical or chemical injuries and immunologic disorders can lead to cutaneous fibrosis such as keloids, hypertrophic scars, and scleroderma (systemic sclerosis) (4). Alcohol and viral infections are major causes of hepatic fibrosis, and glomerulonephritis, diabetic mellitus, and hypertension are major causes of renal scarring (4,6). Diffuse cardiac fibrosis is one of the major complications of hypertension usually associated with progressive heart failure (7). A number of drugs such as bleomycin, cisplatin, cyclosporine, and gentamicin can also induce fibrosis of the lung and kidney and have been instrumental in inducing fibrosis in certain experimental models (7,8). A relatively newly described immune cell, the fibrocyte, has been implicated in fibrotic disease, as well as in normal wound repair, granuloma formation, and antigen presentation (3,5,30-33). Evidence from...
Certain physiologic skin changes occur. Perspiration is increased. Hyper-pigmentation of the abdominal midline, nipples, vulva, and face (chloasma) is seen, and, in some brunettes, nevi and freckles also become more prominent and more pigmented. Malignant melanoma is not more common in pregnancy. Hypertrichosis of the scalp may be unnoticed until the excess hair begins to be shed after delivery. Striae of breasts, abdomen, and thighs appear. The skin diseases of pregnancy are herpes gestationis (see Fiig.M 26-11D-E), impetigo herpetiformis, vulvar pruritus (often due to candidal infection), palmar erythema, spider hemangiomas, pyogenic granulomas, rarely erythema multiforme, and pedunculated fibromas. The following dermatoses are usually better, or disappear, during pregnancy psoriasis, acne (can be worse), alopecia areata, and, possibly, systemic scleroderma.
The incidence and the severity of delayed GI tract toxicity following allogeneic hematopoietic stem cell transplantation are related to the cumulative radiation dose used in the conditioning regimen, the presence of GVHD, or, a combination of both. Whereas GI tract toxicity is frequent in the acute transplant setting, chronic late problems affecting GI tissues are relatively uncommon. Xerostomia may result from sclerodermatous changes of the mucous membranes and salivary glands and predispose to accelerated tooth decay and periodontal disease 9 . Esophageal stricture formation is the most common form of delayed GI toxicity and may require dilation procedures to maintain satisfactory oral intake 58,59 . Affected tissues show characteristic web-like intraluminal membranes that form strictures some patients also demonstrate perimuscular fibrosis similar to that seen in scleroderma 58,59 . GVHD of the small bowel may result in chronic diarrhea from malabsorption (Fig. 12.3). Steatorrhea...
As skin disorders that can lead to a scarring hair loss one should include discoid lupus erythematosus, scleroderma, lichen planus (lichen planopilaris), fungal infections, and prolonged inflammatory tinea. Metastatic carcinoma and trauma of various types can cause scarring hair loss. A skin biopsy and a fungal culture are indicated to help establish the diagnosis in cases of scarring alopecia ( Fig 27.16).
A moderately rare idiopathic atrophy in older adults, particularly women, characterized by the presence of thickened skin at the onset, with ulnar bands on the forearm, changing into atrophy of the legs below the knee and of the forearms. In the early stages this is to be differentiated from scleroderma. High doses of penicillin may be effective. Late stage of Lyme disease. Poikiloderma atrophicans vasculare (Jacobi). This rare atrophic process of adults is characterized by the development of patches of telangiectasis, atrophy, and mottled pigmentation on any area of the body. This resembles chronic radiodermatitis clinically and may be associated with dermatomyositis or scleroderma. May precede the development of a lymphoma. Hemiatrophy. May be localized to one side of the face or may cover the entire half of the body. Vascular and neurogenic etiologies have been proposed, but most cases appear to be a form of localized scleroderma. Atrophoderma,...
Erythematosus, scleroderma, and dermatomyositis. Most of these diseases are associated with autoantibodies, including antinuclear antibodies. Clinical presentation, serology, and histology are used to classify the disorders. Overlap syndromes combining one or more of these illnesses are common.
SCLERODERMA. (See Fig, 25-4, Fig 2.5.-5, Fig 25z6 and Fig 26 17.C.) Scleroderma is a chronic systemic disease of unknown origin that affects the connective tissue and the vasculature. The disease is characterized by fibrosis and obliteration of the vessels in the skin, lungs, heart, gastrointestinal tract, and kidneys. The localized form of scleroderma, morphea, is confined to the skin. In systemic scleroderma, the clinical manifestations depend on the sites involved.
Raynaud's disease has been divided into primary and secondary forms. Primary Raynaud's is more common than the secondary form, occurring in 3 to 16 of the general population.27 Secondary Raynaud's disease is far less common, developing in only 3 to 9 of patients it is defined as Raynaud's phenomenon associated with the development of a connective tissue disease (most commonly scleroderma).
Thrombocytopenia, pernicious anemia, cyclic or periodic neutropenia), immunocompromised conditions (such as the acquired immuno-deficiency syndrome, organ transplants, lymphomas), collagen diseases (lupus erythematosus and scleroderma), pigmentary diseases (e.g., Addison's disease, Peutz-Jeghers syndrome), and autoimmune diseases, which cross over in several categories but include pemphigus and pemphigoid, and possibly benign mucosal pemphigoid.