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Results for “Scleroderma, Localized”

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PubMed1977

Cellular infiltrates in scleroderma skin.

The purpose of this study was to determine the frequency, distribution, and nature of cellular infiltrates in 108 skin biopsies from patients with systemic scleroderma (SS) and localized scleroderma (LS). Cellular infiltrates, perivascular or diffuse, were noted in 49% of SS and 84% of LS patients and consisted of lymphocytes, plasma cells, and macrophages. No correlation was noted between the presence or severity of skin cellular infiltrates and serum serologic abnormalities.

Adolescent↗
PubMed1995

Increased expression of lysyl oxidase in skin with scleroderma.

Lysyl oxidase initiates cross-linkage of collagen and elastin by catalysing the formation of a lysine-derived aldehyde. In order to study cross-linking in scleroderma, we used monoclonal antibodies to lysyl oxidase to determine the localization of this enzyme in systemic and localized scleroderma, and compared the distributions obtained with that in normal skin. Using an indirect immunofluorescent antibody method and an avidin-biotinylated enzyme complex method, 11 cases of diffuse type of systemic scleroderma and seven cases of localized scleroderma were studied. In the oedematous stage of systemic scleroderma, intracellular and extracellular lysyl oxidase were remarkably increased in the dermis, particularly in groups around blood vessels. In the sclerotic stage of systemic scleroderma, lysyl oxidase was detected intracellularly in fibroblasts and extracellularly among collagen bundles between the lower dermis and the subcutaneous fat tissue. In localized scleroderma, a marked increase in lysyl oxidase was observed in mononuclear cells and fibroblasts near blood vessels in the lower dermis and in the subcutaneous fat tissue, in addition to the extracellular deposits between collagen bundles. The increase in lysyl oxidase in localized scleroderma was much more common than in the oedematous stage of systemic scleroderma. These findings indicated that intracellular and extracellular expression of lysyl oxidase expression was greater in sclerodermatous skin than in normal skin.

Adolescent↗
PubMed1995

A case of linear type of lichen sclerosus et atrophicus?

We report a case of the linear type of lichen sclerosus et atrophicus (LSA). The patient had linear atrophic lesions from the left upper back to the left hand. Histological findings obtained from the upper back were those of typical LSA. Histology of the forearm showed mild changes suggestive of LSA. There were no histological features suggesting any association with localized scleroderma in either specimen. The relation between LSA and localized scleroderma is unclear at present. This case suggests that there is a linear form in LSA as already recognized in localized scleroderma.

Adult↗
PubMed2001

Anti-U1RNP antibodies in patients with localized scieroderma.

Antibodies to U1 ribonucleoproteins (RNP) have been detected in serum from patients with various autoimmune diseases. However, the presence of anti-U1RNP antibodies in patients with localized scleroderma has not been reported. In this study, we examined the frequency of anti-U1RNP antibodies using immunoprecipitation of U small nuclear RNAs and determined the antigen specificity by immunoblotting. Of 70 serum samples from patients with localized scleroderma, 2 (3%) immunoprecipitated U1 small nuclear RNA. Indirect immunofluorescence using HEp-2 cells as substrate showed coarse speckled nuclear fluorescence without nucleolar staining in both of the samples positive for anti-U1RNP antibodies. In addition, the presence of anti-U1RNP antibodies in each serum sample was confirmed by immunodiffusion against HeLa cell extracts. Immunoblotting analysis showed anti-70 kDa antibodies in each serum sample. This reaction against 70 kDa protein in the patients with localized scleroderma was analogous to that in patients with systemic sclerosis or mixed connective tissue disease. Both patients with positive serum were diagnosed as having linear scleroderma, but neither had evidence of Raynaud's phenomenon or sclerodactyly. These results indicate that the presence of anti-U1RNP antibodies is one of the serological abnormalities in localized scleroderma, and that the mechanism of induction of anti-U1RNP antibodies in patients with localized scleroderma might be similar to that in patients with systemic sclerosis and mixed connective tissue disease.

Autoantibodies↗
PubMed1995

Incidence of cancer among patients with systemic sclerosis.

BACKGROUND: To determine cancer risk among patients with systemic sclerosis and localized scleroderma, a population-based retrospective cohort study was performed. Patients in Sweden with a discharge diagnosis of systemic sclerosis or localized scleroderma were obtained from the computerized database of hospital discharge diagnoses for the years 1965-1983. Nine hundred seventeen patients with systemic sclerosis and 102 with localized scleroderma were identified. METHODS: Using record linkage analysis with data from the Swedish National Cancer Registry, standardized incidence ratios (SIR)s (the ratio of observed to expected incidence) were calculated for specific cancer sites. RESULTS: The SIR for developing cancer in the cohort with systemic sclerosis was 1.5 (95% CI, 1.2-1.9). For specific cancer sites, risks were elevated for lung cancer (SIR, 4.9; 95% CI, 2.8-8.1), nonmelanoma skin cancers (SIR, 4.2; 95% CI, 1.4-9.8), and primary liver cancer (SIR, 3.3; 95% CI, 1.1-7.6). There was a suggestive increase in hematopoietic cancers (SIR, 2.3; 95% CI, 0.9-4.8). In contrast, cancer risks in the similarly ascertained cohort with localized scleroderma were no different from those of the general population. CONCLUSIONS: This study confirms earlier reports of an association between systemic sclerosis and an increased risk of cancer. Specific tumor sites correspond to the sites commonly affected by fibrosis such as the lung and skin.

Adult↗
PubMed1984

Blood flow of morphoea plaques as measured by laser-Doppler flowmetry.

Blood flow measurements by laser-Doppler flowmetry were performed on 15 patients with localized scleroderma (morphoea). Thirty morphoea plaques were studied by means of measurements of the sclerotic centre, the perilesional area, and regional control of the normal-appearing skin of the individual patients. Blood flow was increased (P less than 0.01) in the sclerotic area of all patient material and in the 10 patients having one or a few plaques (P less than 0.01). Five patients with generalized morphoea showed the same tendency. Blood flow was also increased in the perilesional area (P less than 0.01) of the total material, and in the patients with one or a few plaques (P less than 0.05), particularly in the case of a 'lilac ring'. 133Xenon washout measurements in 3 patients showed parallel results. The blood flow of plaques with clinically 'advanced' scleroderma was higher (P less than 0.01) as compared to plaques with 'slight' scleroderma. Measurements in pigmented spots after previous morphoea showed normal blood flow.

Adolescent↗
PubMed1983

Collagen synthesis in generalized morphea.

Synthesis of collagen and non-collagenous proteins was measured in fibroblast cultures derived from different layers of the dermis from a patient with an early stage of localized scleroderma. Increased synthesis of collagen was found in fibroblasts grown from the subcutaneous fat of this patient, whereas cells obtained from the papillary dermis revealed normal metabolism. These data agree with the results obtained in previous experiments with cells derived from patients with progressive systemic sclerosis in primary culture, thus indicating that the two diseases have a common pathomechanism. Several subcultures of the activated fibroblast populations were also studied. Normal collagen synthesis in these cultures was observed after the fifth passage, probably indicating selection of cell populations or loss of the previous phenotype.

Adipose Tissue↗
PubMed1990

Cutaneous manifestations of the L-tryptophan-associated eosinophilia-myalgia syndrome: a spectrum of sclerodermatous skin disease.

The natural history of the clinical and pathologic features of skin disease was reviewed prospectively in 30 patients with the L-tryptophan-associated eosinophilia-myalgia syndrome. Overall, cutaneous manifestations developed in 26 patients (87%). Early lesions were nonspecific and characterized predominantly by an erythematous macular eruption on the trunk and extremities. The most characteristic abnormality noted was the spectrum of sclerodermatous disease in 15 patients (50%) often after a subacute stage of peripheral or truncal edema. Clinical and/or biopsy evidence of eosinophilic fasciitis was seen in nine patients (30%). Findings consistent with diffuse, limited, or localized scleroderma were subsequently observed in nine patients (33%). Small mucinous papules, similar to those seen in scleromyxedema, were found in five patients (17%). Alopecia, frequently a late sequela, developed in 11 (37%). Common histologic features included papillary dermal fibrosis, dermal and fascial infiltrates consisting of mononuclear cells and eosinophils, deposition of glycosaminoglycans in the dermis, and, in some patients, numerous mast cells.

Adult↗
PubMed1982

Morphea of the eyelids.

Morphea, or localized scleroderma, of the eyelids is an uncommon disease. Morphea usually involves the thorax, trunk, lower and upper extremities, face, and genitalia. In the present report a patient with a biopsy-proven morphea of both upper eyelids is described. The salient histopathologic features included thinning of the epidermis with thickening and sclerosis of the collagen fibers in both the papillary and reticular dermis. There was a marked decrease in the fibrocytes. The eccrine sweat glands were entrapped by sclerotic collagen fibers. The pilosebaceous units were markedly decreased in number. There was a moderate lymphocytic infiltration in the dermis and a prominent lymphocytic perivasculitis. The clinical and histopathologic features of morphea are compared with those of lichen sclerosus et atrophicus.

Adolescent↗
PubMed1984

Enophthalmos: a clinical review.

Twenty-six cases of enophthalmos were reviewed. The causes in order of frequency were: orbital asymmetry (8); trauma (5); orbital metastasis (4); microphthalmos (2); orbital varix (2); maxillary mucocele (2); localized scleroderma (1); absence of sphenoid wing (neurofibromatosis) (1); post irradiation atrophy (1). Only six of the patients (23%) were referred with the diagnosis of enophthalmos, suggesting the sign maybe subtle and is frequently missed or misdiagnosed. The nature of the causes underscore the need for careful and thorough diagnosis. In particular, the therapeutic implications of diagnosing metastatic disease, maxillary mucocele, and orbital varices is noted. A review of etiology and mechanisms of enophthalmos point to the diversity, importance and conditions causing this sign.

Adolescent↗
PubMed1988

Generalized morphea and idiopathic thrombocytopenia.

A patient with generalized morphea and thrombocytopenia is reported. The thrombocytopenia responded promptly to corticosteroid and immunosuppressive therapy but recurred when the steroids were discontinued. The occurrence of thrombocytopenia in generalized morphea suggests a common immune mechanism and emphasizes the need to look for concomitant autoimmune hematologic disorders in patients with systemic, as well as localized, scleroderma.

Biopsy↗
PubMed2001

Effects of low dose ultraviolet A-1 phototherapy on morphea.

AIM: The effects of low dose ultraviolet A-1 (UVA-1) phototherapy on different clinical stages of morphea (localized scleroderma) were analyzed in this case study. Based on these data, the different types of phototherapy described in the literature and currently used for treatment of morphea are compared. METHODS: Three patients with severe plaque type morphea in different stages were studied: one patient with late-stage lesions having stable sclerotic plaques; another patient with active inflammatory lesions; and a third patient with late-stage lesions associated with overlying lichen sclerosus et atrophicus (LSA). The treatment given was low dose UVA-1 phototherapy with single doses of 20 J/cm2 administered four times a week for 6 weeks, and once a week for another 6 weeks. RESULTS: Following UVA-1 phototherapy, the sclerotic plaques resolved, leaving smooth and soft tanned skin with normal structure, consistency and folding capability. In morphea with overlying LSA the elastic fibers did not completely return to the superficial papillary dermis despite the clinical clearance of both morphea and LSA. These data suggest that low dose UVA-1 phototherapy may improve, but not completely reverse, the histopathological changes of LSA. No side effects were observed during or after treatment. CONCLUSION: Our observations show in three patients that low-dose UVA-1 phototherapy is highly effective for treatment of all stages of morphea, including early inflammatory and late sclerotic lesions, and morphea with overlying lichen sclerosus et atrophicus. Because of its safety and efficacy, low dose UVA-1 phototherapy appears to be the treatment modality of choice.

Adult↗
PubMed2003

Ultraviolet A1 phototherapy.

Long-wavelength ultraviolet A (340-400 nm; UVA1) therapy is currently available in only a few dermatology departments. Equipment capable of delivering this waveband has been available since 1981, but it is only over the past decade that increasing numbers of studies assessing the potential of this as a therapy have been published. High-dose UVA1, which requires expensive and space-occupying apparatus, is effective as a monotherapy for acute flares of atopic dermatitis, but it has not yet been formally assessed as an adjunct, rather than as an alternative to conventional therapies including potent and very potent topical corticosteroids. Low-dose (which can be administered using a standard phototherapy cubicle fitted with appropriate lamps) and medium-dose UVA1 may be less effective for this indication. Another condition for which UVA1 is effective, and is particularly promising because we have no reliably effective treatment already, is localized scleroderma. It also appears to be effective in systemic lupus erythematosus (although it is not yet clear when it is indicated, and its safety needs to be assessed in more patients) and in polymorphic light eruption (although there have been no studies suggesting that UVA1 will have any advantages over standard prophylactic phototherapies). Open studies and case series suggest that UVA1 may prove beneficial for various other diseases, including cutaneous T-cell lymphoma, lichen sclerosus, keloids, systemic sclerosis and hand dermatitis. In the centres where it is available, UVA1 has already proved a useful addition to the range of phototherapies previously available. However, much more research is needed to confirm its efficacy for many of its potential indications, and to determine when and how it should be used.

Controlled Clinical Trials as Topic↗
PubMed2003

Guttate morphoea in human T-cell lymphoma/lymphotrophic virus type-1 (HTLV-1) infection.

A 62-year-old Japanese man presented with multiple small atrophic macules on the trunk and extremities. The lesions were discrete, oval in shape and enclosed by lilac ring. They were distributed in a Christmas tree distribution, reminiscent of pityriasis rosea. Skin biopsy showed increased collagen fibres in the dermis and invading subcutaneous tissue. The clinico-pathological features were consistent with guttate morphoea, a rare variant of localized scleroderma. Serological tests revealed a positive reaction to human T-cell lymphoma/lymphotropic virus type-1 infection.

Biopsy↗
PubMed2003

Ulcers caused by bullous morphea treated with tissue-engineered skin.

Bullous morphea is an uncommon form of localized scleroderma. The exact pathogenesis is unknown and treatment of the accompanying ulcers is problematic. We report a patient with bullous morphea with long-standing ulcers whom we successfully treated with the tissue-engineered skin Apligraf (Organogenesis Inc., Canton, MA). The patient experienced rapid improvement in granulation tissue and the ulcers healed 4 months after a single application. The rationale for the use of Apligraf is based on experience with patients with venous ulcers who have surrounding peri-ulcer fibrosis. This condition, termed lipodermatosclerosis, has been reported as a poor prognostic factor for healing, yet many ulcers associated with lipodermatosclerosis may respond to treatment with tissue-engineered skin. Taken in concert, these results suggest a role for tissue- engineered skin in the treatment of chronic wounds with surrounding fibrosis.

Collagen↗
PubMed2001

Morphoea in three siblings.

Atrophoderma of Pasini and Pierini (APP) is an uncommon form of localized morphoea that occurs as superficial, hyperpigmented plaques distributed mainly on the trunk and proximal part of the limbs. There is little information about the influence of genetic and environmental factors on disease susceptibility and expression for localized scleroderma, although APP familial cases have been reported. We report three siblings without a family history of autoimmune disease presenting cutaneous lesions suggesting morphoea (APP variant).

Adult↗