1/93. Isolation of Borrelia afzelii from circumscribed scleroderma.A 45-year-old man presented with circumscribed scleroderma (CS) on the extremities. histology of lesional skin showed the typical manifestations of scleroderma including a perivascular and interstitial infiltrate of lymphocytes and plasma cells; in one of the biopsies spirochaetes could be detected. Despite treatment with penicillin, progression of CS was observed and spirochaetes were isolated from skin cultures obtained from active scleroderma lesions. These spirochaetes were identified as Borrelia afzelii by sodium dodecyl sulphate-polyacrylamide gel electrophoresis of outer surface proteins and polymerase chain reaction (PCR) analysis of their chromosome. After two courses with ceftriaxone the lesions stopped expanding and sclerosis of the skin was diminished. At this time cultures for spirochaetes and PCR of lesional skin for Borrelia afzelii dna remained negative. The pathogenetic role of Borrelia afzelii in the development of CS is discussed.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
2/93. bleomycin-induced scleroderma: report of a case with a chronic course rather than the typical acute/subacute self-limiting form.We report a case of bleomycin-induced scleroderma in a 35-year-old woman treated with chemotherapy for Hodgkin's disease. Approximately 6 months after the first chemotherapy cycle, the patient developed skin sclerosis in both arms. The lesion showed no signs of spontaneous clinical amelioration and treatment with steroids was unsuccessful. A partial remission of the skin sclerosis was instead obtained by the administration of D-penicillamine. A family history revealed other cases of autoimmune diseases and HLA typing showed the presence of antigens associated with scleroderma. The association between bleomycin therapy and scleroderma is discussed.- - - - - - - - - - ranking = 2keywords = sclerosis (Clic here for more details about this article) |
3/93. Scleroderma-like lesions in insulin-dependent diabetes mellitus.We report on two patients (one female 42 years, one male 47 years) suffering from insulin-dependent diabetes mellitus (IDDM) for more than 20 years. Both patients exhibited sclerodactyly and sclerosis of the hands and lower arms as well as swelling and slight contracture of the distal interphalangeal joints. Interestingly, internal organs were not involved and autoantibodies characteristic for scleroderma were missing. Poor utilization and excess of glucose seem to be responsible for the activation of fibroblasts to produce abundant matrix proteins in the skin. Significant therapeutic improvement of the glucose metabolism was able to improve joint contractures or at least to stop the progression of skin changes in our patients. These skin changes should not be misdiagnosed as systemic sclerosis.- - - - - - - - - - ranking = 2keywords = sclerosis (Clic here for more details about this article) |
4/93. Post-stripping sclerodermiform dermatitis.BACKGROUND: Cutaneous sclerosis, a process that results in hardening of the skin, is the hallmark of scleroderma and sclerodermoid disorders. Cutaneous sclerosis is usually classified as secondary or primary, depending on the presence or absence of underlying diseases. Primary cutaneous sclerosis is a feature of idiopathic inflammatory processes that are often associated with autoimmune disorders, whereas secondary cutaneous sclerosis arises in the context of many pathological processes of varying causes, including chronic graft-vs-host disease, defined metabolic or genetic disorders, and exposure to certain infectious organisms, drugs, or chemicals. OBSERVATIONS: Three patients had localized cutaneous sclerosis overlying the site of a surgically removed (stripped) great saphenous vein. In all 3 patients, lesions were clinically characterized by multiple hypopigmented and indurated plaques distributed linearly along the path of the preexisting vein. Extensive history, physical examination, and diagnostic tests did not reveal known predisposing factors for cutaneous sclerosis. CONCLUSIONS: Although the observed association of sclerodermiform dermatitis and venous stripping in these 3 patients does not imply a causal relationship, the absence of other identifiable predisposing factors and the striking linear distribution of the cutaneous lesions along the path of the preexisting vein are suggestive. This poststripping sclerodermiform dermatitis may be a rare late complication of saphenous vein stripping.- - - - - - - - - - ranking = 6keywords = sclerosis (Clic here for more details about this article) |
5/93. Lipodermatosclerosis - report of three cases and review of the literature.We report 3 cases of lipodermatosclerosis (LDS) and discuss the nosology of similar disorders caused by venous insufficiency of the legs. These cases are characterized by (1) occurrence in middle-aged or aged woman, (2) painful, indurated erythema with hyperpigmented scleroderma-like hardening on the lower leg, (3) lobular panniculitis with membranocystic fat necrosis and various degrees of septal fibrosis. Although the designation LDS has been used particularly in the UK and in the USA, this entity is not familiar in other countries including japan. LDS clinically represents a wide spectrum from an acute, inflammatory phase to a chronic, fibrotic state. The clinicopathologic findings of LDS are similar or identical to the disease previously reported as chronic indurated cellulitis, hypodermitis sclerodermiformis, stasis panniculitis or sclerosing panniculitis. These diseases are probably related conditions, which depend upon the various stages.- - - - - - - - - - ranking = 5keywords = sclerosis (Clic here for more details about this article) |
6/93. A case of generalized morphea with a high titer of anti-borrelia burgdorferi antibodies.A 69-year-old male had noticed pruritus on the back for the previous 3-4 years and cutaneous sclerosis with swelling of the dorsum of the neck had developed in the last one and a half years. However, he had never complained of Raynaud's phenomenon of the fingers, dry mouth, or dry eyes. At this first visit to our hospital, he complained of erythematous cutaneous sclerosis with swelling of the dorsum of the neck. Histopathological findings biopsied from the neck showed epidermal hyperplasia with elongation of rete ridges and homogeneous and fibromatous changes of the dermis with dense perivascular cell infiltration consisting of mononuclear cells or lymphocytes with several nests of incontinentia pigmenti. However, there were no sclerotic changes in blood vessels in the upper dermis biopsied from the forearm skin, although slightly homogeneous and fibromatous changes of the dermis were seen. In the clinical course, the cutaneous sclerotic change enlarged to extend to the bottom of the cheek, forearm, and lower legs. These clinical features and histopathological findings led to the diagnosis of generalized morphea. Hematologic examination showed positive anti-borrelia burgdorferi IgM antibodies, although there were no positive anti-borrelia burgdorferi IgG antibodies. These results revealed that there can be a close association of localized scleroderma with borrelia burgdorferi and that generalized morphea may also represent a Borrelia infection.- - - - - - - - - - ranking = 2keywords = sclerosis (Clic here for more details about this article) |
7/93. Primary atrophic profound linear scleroderma. Report of three cases.We present 3 unusual cases of deep linear, primary atrophic scleroderma, not preceded by inflammatory reaction and sclerosis, involving the subcutis and deeper tissues. These cases differ in the course and prognosis from typical profound scleroderma since they do not lead to disfiguration and crippling deformities. In contrast to the atrophies left after regression of morphea or linear scleroderma, they do not involve the dermis, which does not show discoloration or changes in texture. Infiltrates in the endomysium, involvement of deeper tissues and the progressive character of the disease argue for atypical primary atrophic profound scleroderma. The coexistence in one case of primary facial hemiatrophy appears to indicate also its relationship with primary linear atrophies of the limbs. copyright (R) 2000 by S. Karger AG, Basel- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
8/93. Postirradiation morphea of the breast presentation of two cases and review of the literature.The advent of radiation therapy as a common modality in the treatment and palliation of breast cancer has led to the observation of morphea developing months to years after supervoltage radiation therapy, in and around the site of treatment. We report 2 new cases of morphea at the site of previous supervoltage radiation therapy for breast cancer. The time period between irradiation and onset of morphea in our 2 patients were an atypically long 6.5 years and 32 years, the latter being the longest reported such interval. With conservative treatment, the inflammatory component of the lesions resolved over an approximately 1-year period, leaving residual sclerosis. These patients are compared to those previously reported in the medical literature so as to summarize the range of clinical presentation and course. Recognition of postirradiation morphea is important in distinguishing it from infectious cellulitis, recurrent carcinoma, metastatic carcinoma or development of a second primary carcinoma. copyright (R) 2000 S.Karger AG, Basel- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
9/93. The CRST syndrome (calcinosis, Raynaud's phenomenon, sclerodactyly and telangiectasia).The CRST syndrome is defined, and its relation to systemic sclerosis described. The literature referring to the condition is reviewed and a further case reported. Clinical and diagnostic features of the telangiectasia are emphasised, particularly in comparison with those of the similar condition, hereditary haemorrhagic telangiectasia.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
10/93. Brachial plexopathy associated with diffuse edematous scleroderma.peripheral nervous system (PNS) involvement is rare in systemic sclerosis (SSc), usually restricted to peripheral nerve entrapment. To our knowledge, only one case of scleroderma with brachial plexus involvement has been reported previously. We report here on a 61-year-old woman with past history of limited cutaneous SSc who developed motor deficiency in the left arm concomitant with diffuse edematous scleroderma without evidence for trauma or compression of the brachial plexus. After six months intravenous pulse cyclophosphamide therapy, dramatic improvement of skin and neurological involvement was observed.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
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