1/10. A pediatric case of sclerodermatous chronic graft-versus-host disease.We report a rare case of sclerodermatous chronic graft-versus-host disease (GVHD) in a 6-year-old boy that occurred after bone marrow transplantation for his aplastic anemia. The clinical manifestation and histopathologic findings were typical of scleroderma. Although various kinds of treatment have been tried for scleroderma, no established therapy exists. Furthermore, treating this disease is even more difficult in children. In the future, clarification of the pathogenesis of chronic GVHD and establishment of therapy will be necessary.- - - - - - - - - - ranking = 1keywords = scleroderma (Clic here for more details about this article) |
2/10. Continuous regional anesthesia before surgical peripheral sympathectomy in a patient with severe digital necrosis associated with Raynaud's phenomenon and scleroderma.BACKGROUND AND OBJECTIVE: Digital ischemia and necrosis caused by Raynaud's phenomenon in patients with connective tissue diseases may not respond to medical therapy and may have major adverse effects on quality of life. We describe the use of continuous ambulatory regional anesthesia for diagnosis and treatment before peripheral sympathectomy in a patient with secondary Raynaud's phenomenon. CASE REPORT: A 55-year-old man with progressive systemic sclerosis and secondary Raynaud's phenomenon presented with severe pain and digital necrosis that were refractory to maximal medical treatment and thoracic sympathectomy. Continuous ambulatory regional analgesia increased digital temperature from 32.3 degrees C at baseline to 34.4 degrees C after 80 minutes. An increase in digital flow was documented by Doppler ultrasound measurements made ventrally at the point of greatest pulsation of the radial artery. Subsequent peripheral sympathectomy resulted in restoration of nutrient flow with healing of ulcers and alleviation of pain. CONCLUSIONS: Continuous ambulatory regional anesthesia appears effective as a treatment bridge for vasospasm and ischemia associated with secondary Raynaud's phenomenon. The enhancement of peripheral blood flow achieved with the regional anesthetic technique suggests that surgical peripheral sympathectomy may provide long-term benefits.- - - - - - - - - - ranking = 0.57142857142857keywords = scleroderma (Clic here for more details about this article) |
3/10. Bilateral breast cancer associated with diffuse scleroderma.There is a strong evidence to suggest the association between breast cancer and diffuse scleroderma, though it is an infrequent occurrence. We describe the clinical and radiological findings in a patient who, over a period of 2 years, was diagnosed initially of diffuse scleroderma, next with right breast cancer and finally with left breast cancer. A review of the literature evidencing the relation between these two pathologies is provided. We suggest that special vigilance for tumoral pathology of the breast should be performed in patients with systemic scleroderma.- - - - - - - - - - ranking = 1keywords = scleroderma (Clic here for more details about this article) |
4/10. Keloidal scleroderma.Progressive systemic sclerosis (PSS) can involve the skin diffusely or in a limited way. Rarely cutaneous involvement can assume a hypertrophic form, described in the literature as keloidal scleroderma. The authors describe a PSS patient who presented cutaneous manifestations compatible with the diagnosis of this clinical situation. The clinical features, pathogenic mechanism and therapeutic approach are discussed and a broad review of the literature is given.- - - - - - - - - - ranking = 0.71428571428571keywords = scleroderma (Clic here for more details about this article) |
5/10. Gemcitabine-associated scleroderma-like changes of the lower extremities.Gemcitabine is a nucleosid analog approved for use in the treatment of metastatic urothelial carcinoma of the bladder. We describe an unusual case of scleroderma-like changes of the lower extremities after treatment by gemcitabine for metastatic carcinoma of the bladder. The patient developed initial inflammatory edema (3 kg) restricted to the lower extremities and subsequent scleroderma-like changes after 2 cycles of gemcitabine. Cutaneous biopsy specimen revealed diffuse sclerosis without involvment of the fascia or muscle. Discontinuation of gemcitabine resulted in dramatic removal of the edema, softening of the skin, and partial reversibility of the fibrotic process. This is the first case report of a scleroderma-like reaction associated with gemcitabine. This antineoplastic agent must be added to the very limited number of cytostatic agents capable of giving rise to scleroderma-like features.- - - - - - - - - - ranking = 1.1428571428571keywords = scleroderma (Clic here for more details about this article) |
6/10. Ischaemic stroke in progressive systemic sclerosis.Progressive systemic sclerosis (PSS) or scleroderma is a multisystem disease affecting the skin, lungs, myocardium, kidneys and gastrointestinal tract. Primary involvement of cerebral arteries in PSS has been reported but is very rare. A 61-year-old woman suffering from scleroderma for six years was hospitalised for two subsequent episodes of transient acute dysarthria and left hemiparesis. After five hours from the first onset of symptoms, she was submitted to brain magnetic resonance (MR) protocol that showed a right subinsular ischaemic lesion and whole right middle cerebral artery (MCA) territory hypoperfusion. Intracranial and epiaortic MR angiography reported a focal stenosis in the M2 portion of MCA. She was immediately treated with i.v. high dose steroids and oral acetylsalicylic acid. At one-month follow up, MR findings were confirmed. We have documented a cerebral infarct in a PSS patient. In our opinion, the ischaemic stroke was caused by a localised autoimmune angiopathy.- - - - - - - - - - ranking = 0.28571428571429keywords = scleroderma (Clic here for more details about this article) |
7/10. Chemotherapy-induced scleroderma: a pleiomorphic syndrome.A scleroderma-like disease has recently been described in association with taxanes. We present the first case of diffuse scleroderma occurring in a woman treated with doxorubicin and cyclophosphamide for breast cancer. The clinical pattern of skin involvement and histological alterations were identical to those found in the classical form of scleroderma. skin involvement progressed to affect 80% of total body area, and subsequently remained unchanged despite progression of the underlying cancer, making a paraneoplastic aetiology of the scleroderma unlikely. Specific chemotherapeutic agents might be directly responsible for the clinical manifestations and the parameters of progression. Analysis of all similar case reports defines the particular features and clinical course of this phenomenon.- - - - - - - - - - ranking = 1.1428571428571keywords = scleroderma (Clic here for more details about this article) |
8/10. Late recurrence of scleroderma renal crisis in a renal transplant recipient despite angiotensin ii blockade.Scleroderma renal crisis is the most severe renal manifestation of scleroderma and has been reported to recur rarely early after renal transplantation. angiotensin ii blockade is critical in preventing and treating scleroderma renal crisis, but some concern exists as to whether angiotensin ii receptor blockers are clinically equivalent to angiotensin-converting enzyme inhibitors. The current case indicates that late recurrences of scleroderma renal crisis are possible in renal transplant recipients and that angiotensin-converting enzyme inhibitors, rather than angiotensin ii receptor blockers, may be the superior drugs for such patients.- - - - - - - - - - ranking = 1keywords = scleroderma (Clic here for more details about this article) |
9/10. multiple myeloma with scleroderma-like changes.We present a case of a patient who presented concomitantly with generalized pruritus, brownish sclerodermatous plaques, sclerodactyly and a monoclonal band for IgG-kappa. The patient was diagnosed as having multiple myeloma by bone marrow examination. The rapidly progressive evolution with acute anuric renal failure, malignant hypertension and the skin sclerosis seem to be related to the neoplastic disorder. The scleroderma-like changes have to be differentiated from systemic scleroderma.- - - - - - - - - - ranking = 1keywords = scleroderma (Clic here for more details about this article) |
10/10. Ischemic scleroderma wounds successfully treated with hyperbaric oxygen therapy.Hyperbaric oxygen therapy (HBOT) has been used to treat refractory wounds for the last several decades, with the majority of research focusing on wounds secondary to arterial insufficiency. We describe 2 patients with scleroderma with intractable bilateral extremity ulcers. Local ischemia was identified using transcutaneous oximetry. Each patient then underwent 30 treatments of HBOT at a relative depth of 2.4 ATA with resulting wound healing. This is the first reported successful use of HBOT to treat scleroderma ulcers, and may represent an unrecognized treatment option for these notoriously difficult chronic wounds.- - - - - - - - - - ranking = 0.85714285714286keywords = scleroderma (Clic here for more details about this article) |
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