Natural Scleroderma Relief
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
Skin changes were first clearly described in the patient series reported by Thannhauser, who distinguished changes in WS patients from those commonly seen in scleroderma patients or in patients with RothmundThomson syndrome (Thannhauser, 1945). The histologic appearance of skin biopsies from WS patients reveals an interesting mix of atrophic and proliferative changes. There is epidermal atrophy that extends to include skin appendages (e.g., hair follicles, sweat and sebaceous glands), in conjunction with focal hyperkeratosis and basal hypermelanosis. Dermal subcutaneous connective tissue atrophy is common, and often is found in conjunction with dermal fibrosis. Muscle, adipose, and connective tissue underlying the skin is often atrophic. This constellation of changes gives the skin a tight, white, and shiny or contracted appearance, with a loss of normal elasticity. Figure 80.1 Skin and soft tissue changes in Werner syndrome patients. A. Scleroderma-like skin changes,...
Scleroderma can involve limited or widespread changes in the skin, with a host of associated internal organ manifestations. In cases of limited skin involvement, the acronym CREST has been widely used to denote the characteristic features of calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasias. In cases of widespread skin involvement, the term systemic sclerosis may be used. Additional features include fibrotic lung disease, pulmonary hypertension, and renal failure.
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
Skin alterations after surgery The retraction of the skin and the scarring of the cutaneous and breast tissue may assume the mammographic appearance of a tumor recurrence. In addition, therapeutic radiation leads to skin thickening and the parenchyma may develop some radiation fibrosis this makes mammographic evaluation of the treated breast extremely difficult (Fig. 12.19). Contrast-enhanced MRI of the breast may be of use in this situation and is being evaluated as an adjunct tool to mammography.
Two diagnostic aids to help radiologists interpret breast lesions. The first aid was a checklist containing a set of features that a radiologist must assess and record. The second aid was an estimated probability of malignancy provided by a computer classifier that analyzed the features reported by radiologists. The process of developing the checklist of features was remarkably involved. It started with interviews with five mammography specialists. These interviews generated several dozen features. Then, this extensive set of features was reduced to 29 through mathematical multidimensional scaling and hierarchical clustering, and through a series of consensus-seeking group discussions. Finally, the 29 features were reduced to 13 by means of linear discriminant analysis. The final set of 13 features was believed to be a necessary and important set of features for classifying malignant and benign breast lesions. The final set included six features describing masses, three features...
The clinical course of patients with nonresectable metastatic carcinoid is highly variable and largely unpredictable (1-4). Patients with high tumor burden can remain relatively asymptomatic for years, totally oblivious to their disease. Others with minimal residual (but nonresect-able) disease in the small-bowel mesentery can suffer all the symptoms of the carcinoid syndrome without having disease in the liver (vide supra). However, the majority of patients with carcinoid metastatic to the liver will exhibit at least some, if not all of the symptoms of the carcinoid syndrome (1-4). These symptoms include facial and sometimes torso flushing which may progress to rosacea and scleroderma (5), and may include the cutaneous manifestations of pellagra (5). Other symptoms include diarrhea, breathlessness, and wheezing and may be precipitated by certain foods including alcohol and chocolate. The exact hormonal mechanism of these symptoms remains unknown.
Deletions in the elastin gene (located at 7q11.23) have been found in approximately 90 of subjects with Williams syndrome, a developmental disorder affecting connective tissue and the central nervous system. The mutations, by affecting synthesis of elastin, probably play a causative role in the supravalvular aortic stenosis often found in this condition. A number of skin diseases (eg, scleroderma) are associated with accumulation of elastin. Fragmentation or, alternatively, a decrease of elastin is found in conditions such as pulmonary emphysema, cutis laxa, and aging of the skin.
In its more aggravated forms diffuse scleroderma is one of the most terrible of all human ills. Like Tithonus to 'wither slowly' and like him to be 'beaten down and marred and wasted', until one is literally a mummy, encased in an ever shrinking, slow contracting skin of steel, is a fate not pictured in any tragedy.
Conditions such as systemic lupus erythematosus, systemic sclerosis, and insulin autoimmune syndrome (Ito et al., 1993 Crow et al., 1994 Kuwana et al., 1995a). In both lupus and scleroderma, T-helper cells mediate antigen-specific autoanti-body production by B cells (Adams et al., 1991 Mohan et al., 1993 Kuwana et al., 1995b).
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.
This was a 41 year old woman with a history of scleroderma, who presented with painful ulcers in the fingers and heels. She was diagnosed with scleroderma when she experienced shortness of breath and esophageal dysmotility. Previously, she had been admitted to the hospital with bilateral hand and foot pain, sclerodactyly and Raynaud's syndrome. On that admission, an echocardiogram showed right atrial and ventricular dilatation, and moderate to severe tricuspid regurgitation. Recently, she was unable to ambulate due to bilateral heel ulcers. She had also developed constipation, abdominal pain and mild dysuria. Her social history included smoking 25 packs a year and previous cocaine abuse (for which she received rehabilitation).
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
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.