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PubMed1999

Trauma-induced linear scleroderma.

Linear scleroderma (linear morphea) is a form of localized scleroderma characterized by sclerotic lesions distributed in a linear, band-like pattern. Despite its benign course, the disease can cause severe cosmetic, orthopedic, and psychologic problems. The cause is unknown. Many cases are preceded by a history of trauma. We describe a case in which linear scleroderma occurred following a laceration to the affected site. We review the treatment options and discuss the current theories regarding the pathogenesis of the disease.

Adult↗
PubMed2002

Treatment of scleroderma.

The treatment of systemic sclerosis (scleroderma) is difficult and remains a great challenge to the clinician. Because the cause is unknown, therapies are directed to improve peripheral blood circulation with vasodilators and antiplatelet aggregation drugs, to prevent the synthesis and release of harmful cytokines with immunosuppressant drugs, and to inhibit or reduce fibrosis with agents that reduce collagen synthesis or enhance collagenase production. The purpose of this review is to critically analyze conventional and new treatments of systemic sclerosis and localized scleroderma. The therapeutic options discussed for the treatment of systemic sclerosis include the use of (1) vasodilators (calcium channel blockers [nifedipine], angiotensin-converting enzyme inhibitors [captopril, losartan potassium], and prostaglandins [iloprost, epoprostenol]), (2) immunosuppressant drugs (methotrexate, cyclosporine, cyclophosphamide, and extracorporeal photopheresis), and (3) antifibrotic agents (D-penicillamine, colchicine, interferon gamma, and relaxin). The treatment options reviewed for localized scleroderma include the use of corticosteroids, vitamin D analogues (calcitriol, calcipotriene), UV-A, and methotrexate. Preliminary reports on new therapies for systemic sclerosis are also considered. These include the use of minocycline, psoralen-UV-A, lung transplantation, autologous stem cell transplantation, etanercept, and thalidomide.

Antineoplastic Agents↗
PubMed2000

Recent advances in phototherapy.

This synopsis reviews recent developments in dermatological phototherapy. UVA1 phototherapy (340-400 nm) is effective in the treatment of inflammatory skin diseases such as acutely exacerbated atopic dermatitis, localized scleroderma, urticaria pigmentosa and disseminated granuloma annulare. Narrowband UVB radiation (311-313 nm) is used successfully as monotherapy or combined with dithranol, oral retinoids or 8-MOP in psoriasis, atopic dermatitis (AD) or photosensitivity disorders such as polymorphic light eruption. Bath water delivery of 8-methoxypsoralen and subsequent UVA-irradiation (PUVA bath therapy) for the treatment of psoriasis as well as for mycosis fungoides, localized scleroderma, urticaria pigmentosa or lichen planus is an effective alternative to its systemic application. The combination of salt water brine baths in different concentrations and subsequent UVA/B irradiation is used increasingly for the treatment of psoriasis or AD. Extracorporeal photopheresis (ECP) has proven to be a very effective treatment modality for cutaneous T cell lymphoma, chronic graft-versus-host disease and certain autoimmune diseases such as systemic scleroderma or pemphigus. However, despite the documented benefits of these new treatment modalities, little data exist as of yet on potential long-term side effects, thus the indications for these therapies should be considered carefully and patients should be followed up at regular intervals.

Female↗
PubMed2005

Phototherapy and photochemotherapy of sclerosing skin diseases.

The treatment of sclerosing skin diseases [systemic sclerosis, localized scleroderma, lichen sclerosus et atrophicus, sclerodermoid graft-vs.-host disease, scleredema adultorum (Buschke), scleromyxedema and necrobiosis lipoidica] is difficult and remains a great challenge. Numerous treatments, some with potentially hazardous side effects, are currently used with only limited success. The introduction of phototherapy and photochemotherapy for sclerosing skin diseases has considerably enriched the therapeutic panel and proven useful in a number of sclerosing skin diseases especially in localized scleroderma. Two phototherapeutic modalitites are used for the treatment of sclerosing skin diseases, long-wave ultraviolet A and psoralen plus ultraviolet A (PUVA). This article reviews current knowledge about the application of phototherapy and photochemotherapy to various sclerosing skin disorders.

Humans↗
PubMed1997

Epidemiology of systemic sclerosis and related diseases.

Epidemiologic studies of scleroderma can provide insight into the dynamics of disease expression over time, over different geographic areas, and over diverse ethnic populations. Although such population studies cannot establish cause and effect relationships, they can reveal associations previously obscured by the relative rarity and clinical diversity of this disease. The incidence rate of systemic sclerosis has been stable over the past 20 years at 19 new cases per million per year. The incidence rate of localized scleroderma or morphea is reported at 27 new cases per million per year and has a benign prognosis overall. Disease expression of systemic scleroderma is influenced by genetic, ethnic, and environmental factors. A cluster of scleroderma cases among Choctaw Native Americans in Oklahoma provides an opportunity to investigate the interaction of genetic and environmental influences. Twin studies suggest a definite but relatively weak genetic component. Case-control studies regarding environmental and occupational exposures have yet to identify risk factors that could serve as triggers for this disease.

Cluster Analysis↗
PubMed2026

Causal Relationships Between Oral Microbiota and Inflammatory Skin Diseases.

INTRODUCTION AND AIMS: The oral microbiome has been increasingly linked to systemic inflammation and immune dysregulation, but whether specific oral bacteria causally contribute to inflammatory skin diseases remains unclear due to confounding and reverse causation. This study aimed to assess the causal effects of 43 oral microbiota taxa on the risk of five inflammatory skin diseases using a Mendelian randomization (MR) approach. METHODS: We performed a two-sample MR analysis using genetic instruments for oral microbiota derived from publicly available genome-wide association studies and outcome data from the FinnGen consortium. Causal effects of oral taxa on systemic lupus erythematosus, vitiligo, pemphigus, localized scleroderma, and dermatitis herpetiformis were estimated. The inverse-variance weighted method served as the primary analysis, complemented by sensitivity analyses to evaluate horizontal pleiotropy, heterogeneity, and reverse causality. RESULTS: MR analyses identified several putative causal associations between oral microbiota and inflammatory skin diseases. Genus Granulicatella and an unknown Streptococcus species (ASV0009) showed causal effects on systemic lupus erythematosus. Family Lachnospiraceae_[XIV] and an unknown Rothia species (ASV0016) were associated with vitiligo. Five oral microbiota taxa demonstrated causal associations with pemphigus. Actinomyces species micronuciformis was linked to localized scleroderma. Order Fusobacteriales and an unknown Neisseria species (ASV0004) were associated with dermatitis herpetiformis. No significant heterogeneity or horizontal pleiotropy was detected in sensitivity analyses. CONCLUSION: This MR study provides genetic evidence supporting a causal role of specific oral bacteria in the development of several inflammatory skin diseases, highlighting the oral microbiome as a potential contributor to cutaneous autoimmunity and inflammation. CLINICAL RELEVANCE: Our findings highlight the putative role of the oral microbiome as a plausible candidate for mechanistic and clinical investigations into the prevention or adjunctive management of selected inflammatory skin diseases. However, oral hygiene improvement, targeted antimicrobials, and other microbiota-directed interventions were not directly tested in this MR study and remain hypothetical strategies requiring validation in experimental and clinical studies.

Humans↗
PubMed1991

Quantitative nailfold capillary microscopy in cutaneous and systemic lupus erythematosus and localized and systemic scleroderma.

Quantitative television microscopy of nailfold capillaries of the fingers was performed in 12 patients with cutaneous lupus erythematosus (six with discoid type and six with disseminated type), in six patients with localized scleroderma (two with circumscribed type, two with linear types, and two with atrophic type), in 10 patients with systemic lupus erythematosus, and in eight patients with systemic scleroderma. The following features were analyzed and compared with a control group (n = 15) of similar age: venous plexus visibility; density of capillaries; avascular fields; hemorrhages; giant capillaries; diameters of the transitional segment, the arterial, and the venous limbs; loop width; and flow stop caused by local cooling test. The patient groups with cutaneous lesions only showed no essential differences as compared with the controls. Patients with systemic scleroderma differed in almost every finding from the controls and from patients with localized scleroderma. Patients with systemic lupus erythematosus exhibited significant differences in several findings as compared with the controls and the cutaneous lupus erythematosus group, but there was overlap.

Adolescent↗
PubMedDate not supplied

Physical injury as a provoking factor in three patients with scleroderma.

A 51-year-old female developed linear-like scleroderma in the left thigh following a linear wound caused by a car accident. 27 years later she also developed a typical diffuse cutaneous systemic sclerosis with extensive skin involvement and bibasilar pulmonary fibrosis. The second case is a 39-year-old female who had a history of Raynaud's phenomenon since early childhood. She developed a morphea following a burning injury of the left thigh. 17 years later she also developed a typical limited cutaneous systemic sclerosis with sclerodactyly, skin ulcers and subcutaneous calcinosis. The third case is a 43-year-old female who developed a typical morphea of the right elbow around the site of a previous local corticosteroid injection. The two remarkable points of these 3 cases are the possible role of physical injury in the provocation of localized scleroderma and in the first 2 cases the unusual later development of a systemic form of scleroderma.

Adult↗
PubMed2002

Low-dose ultraviolet A1 phototherapy for extragenital lichen sclerosus: results of a preliminary study.

BACKGROUND: Lichen sclerosus (LS) is a chronic inflammatory skin disease in which numerous therapies have been used, with only limited success. Because low-dose UVA1 phototherapy has been shown to be an effective treatment option for localized scleroderma, which shares several similar clinical and histologic features with LS, we initiated a clinical trial with this phototherapeutic modality in patients with LS. METHODS: Ten patients suffering from extragenital LS were treated with low-dose UVA1 phototherapy 4 times weekly with single UVA1 doses of 20 J/cm(2). Forty treatment sessions were performed within 10 weeks, resulting in a cumulative UVA1 dose of 800 J/cm(2). RESULTS: Low-dose UVA1 phototherapy resulted in a marked reduction of the clinical score and a significant (P <.05) decrease of ultrasonographically measured skin thickness as well as a highly significant (P <.001) increase of dermal density. The patients reported a remarkable softening and repigmentation of the affected skin. CONCLUSION: Analogous to the treatment results in localized scleroderma, low-dose UVA1 phototherapy seems to be an effective and well-tolerated treatment option for extragenital LS.

Adult↗
PubMed1990

[The treatment of scleroderma with the enzyme preparation collalysine].

Examinations of 42 patients with local scleroderma have revealed increased hydroxyproline and glycosaminoglycane excretion and impaired cellular and humoral immunity, these shifts correlating with the type and severity of the disease. Collalysin therapy resulted in a good clinical effect and improvement of the immune and metabolic parameters.

Adolescent↗
PubMed1990

[Bilateral circumscribed scleroderma of the face in children].

Two cases of local scleroderma in children are described with bilateral symmetrical localization of the foci on the face, including a most rare site--round the eyes. A long follow-up of children with such a localization of the disease is necessary, because they may develop Romberg's hemiatrophy.

Child↗
PubMed2003

Scleroderma en coup de sabre with central nervous system and ophthalmologic involvement: treatment of ocular symptoms with interferon gamma.

Scleroderma en coup de sabre, a variant of localized scleroderma, is a disorder of unknown origin characterized by fibrosis of connective tissue. Rare complications of scleroderma en coup de sabre are orbital and intracerebral involvement. We describe a patient with scleroderma en coup de sabre in whom intracerebral and orbital lesions developed after 2 decades of disease duration. Clinically, she had epilepsy, impaired vision, and retro-ocular pain of the affected eye. A 12-month course of interferon-gamma stopped progression of visual symptoms caused by orbital fibrous tissue. To our knowledge, this is the first patient with scleroderma en coup de sabre complicated by orbital involvement who was successfully treated with interferon-gamma.

Adult↗
PubMed1987

[Functional follow-up of pulmonary localization of scleroderma].

Pulmonary lesions of scleroderma are frequent, but little is known of their course, especially where lung function is concerned. We report the results of a longitudinal study performed in 32 patients with generalized scleroderma (ARA criteria) explored on at least 2 occasions with an interval of at least 24 months between the two examinations. Functional alterations seemed to be more pronounced in patients with severe Raynaud's syndrome, but in view of major individual variations no pathognomonic functional profile could be described.

Adult↗