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PubMed2000

Low-dose broad-band UVA in morphea using a new method for evaluation.

Until recently, various therapies for localized scleroderma have been used with limited success. Recently, phototherapy, with or without psoralen, was proposed as a successful treatment modality. The aim of this study was to evaluate the effect of broad-band low-dose ultraviolet A (UVA) phototherapy in patients with localized scleroderma, using a new method for evaluation. Twelve patients complaining of morphea were exposed to UVA irradiation at a dose of 20 J/cm2 3 times per week for 20 sessions. Selected covered plaques served as internal controls. The efficacy of therapy was judged clinically by sequential inspection and palpation. In biopsy specimens from exposed and covered plaques stained with hematoxylin and eosin (H & E) and Masson trichrome stains, the concentration of collagen per dermal surface area was measured with the use of a computerized image analyzer. All patients reported remarkable softening of skin lesions, confirmed by sequential palpatory assessment. A significant reduction in the mean concentration of collagen per surface area was detected in the plaques exposed to UVA (the P value being 0.007, P<0.01), whereas in the covered plaques the difference was not statistically significant (the P value being 0.10, P>0.05). The conclusion is that low-dose broad-band UVA phototherapy is a very effective and safe treatment modality for localized scleroderma.

Adolescent↗
PubMed1986

[Autoantibody profile in collagen diseases. A clinical-serological study of 250 patients from Tuebingen clinics].

Sera from 250 patients with connective tissue diseases were tested for antinuclear antibodies by immunofluorescence (IFL) and immunodiffusion. In patients with systemic lupus erythematosus (SLE, n = 49), progressive systemic sclerosis (PSS, n = 30) and Sjögren's syndrome (n = 20), IFL showed antinuclear antibodies with a frequency of 70 to 100%. Patients with localized scleroderma (n = 16) and discoid LE (n = 38) had antinuclear antibodies in 31 and 21% of cases, respectively. In patients with vasculitis (n = 32), fluorescent antinuclear antibodies were only rarely detected (0-6%). In the immunodiffusion LE-specific anti-Sm antibodies were demonstrated in 10%, anti-nRNP nRNP antibodies in 20%, and anti-Ro antibodies in 37% of patients with SLE. In Sjögren's syndrome, anti-Ro antibodies were found in 45% and anti-La antibodies in 35% of patients. 57% of patients with PSS had the disease-specific anti-Scl-70 antibody, while only 19% had antinucleolar antibodies as detected by IFL. In patients with localized scleroderma, dermatomyositis (n = 6) or vasculitis, no precipitating antibodies were detectable. The demonstration by immunodiffusion of nuclear antibodies in patients with discoid LE could be connected with a transition to a systemic course of the disease.

Adolescent↗
PubMed1997

Distribution of anti-p80-coilin autoantibody in collagen diseases and various skin diseases.

Anti-p80-coilin antibody produces a unique pattern of immunofluorescence staining called nuclear dots characterized by the presence of up to six discrete nuclear bodies in interphase cell nuclei. The distribution of this antibody and its clinical relevance have not been established. We studied the prevalence of anti-p80-coilin antibody in the sera from 810 patients with collagen diseases and various skin diseases. Five sera showed the nuclear dot pattern by indirect immunofluorescence on an HEp-2 cell substrate, and reacted by immunoblotting with 80-kDa protein in a nuclear extract from HeLa cells. Four of these sera were from patients with localized scleroderma, and the other was from a patient with primary Raynaud's phenomenon. The patients with localized scleroderma who were positive for anti-p80-coilin antibody had all been classified as having linear scleroderma. They had only one or two lesions, and were negative for antihistone and anti-single-stranded DNA antibodies. Our data indicate that anti-p80-coilin antibody is uncommon in skin diseases: however, this antibody is present in patients with a milder form of linear scleroderma, although the incidence of positivity may not be high.

Autoantibodies↗
PubMed1990

[Electrocardiographic disorders in diverse clinical variations of scleroderma].

The electrocardiograms of 130 patients affected by scleroderma, 117 with systemic sclerosis (67 with diffuse and 50 with limited form) and 13 with localized scleroderma were analyzed. ECG was normal in 44.6% of cases, respectively in 39.3% with systemic sclerosis and in 92.3% with localized scleroderma. The more frequent electrocardiographic abnormalities were supraventricular and ventricular premature beats, right bundle branch block, left anterior hemiblock, low QRS voltages, ST-T wave abnormalities and Q or QS aspects. All the abnormalities above resulted to be more frequent in the patients with diffuse systemic sclerosis in comparison to the patients with limited systemic sclerosis. Our results confirm that the cardiac involvement appears to be much more serious in the diffuse systemic sclerosis.

Adolescent↗
PubMed1990

[Anti-Borrelia burgdorferi antibodies in chronic erythema migrans, benign lymphadenosis cutis, scleroderma and scleroatrophic lichen].

Fiftyone patients with a diagnosis of erythema chronicum migrans (ECM), lymphadenosis cutis benigna (LABC), systemic progressive sclerosis, localized scleroderma and lichen sclerosus et atrophicans were investigated in order to obtain serological evaluation of Borrelia burgdorferi circulating antibodies. In addition sera from 9 patients with pellagroid erythema, prurigo and panniculitis were performed: in these dermatoses there was no evidence of a possible borrelia relationship. Indirect immunofluorescence assay was used for serologic testing. Elevated IgG antibody titers were detected in one female patient with localized scleroderma (1:128) and in one male patient with LABC (1:64). Serologic testing was not positive in the other 58 patients. The very small number of patients with positive antibodies in our study would indicate that Borrelia burgdorferi infection is sporadic in Lazio.

Antibodies, Bacterial↗
PubMed1995

Lymphoproliferative responses to Borrelia burgdorferi in patients with erythema migrans, acrodermatitis chronica atrophicans, lymphadenosis benigna cutis, and morphea.

BACKGROUND AND DESIGN: Specific humoral and cell-mediated immune responses play an important role in the pathogenesis of Lyme borreliosis. Several previous studies demonstrated that a specific cellular immune response to Borrelia burgdorferi can occur independently of a diagnostic humoral response. Little is known about T-cell reactivities against B burgdorferi in early and late cutaneous manifestations of Lyme borreliosis. We studied the lymphoproliferative response of peripheral blood mononuclear cells to B burgdorferi antigen from 99 patients (25 with erythema migrans, 16 with acrodermatitis chronica atrophicans, 13 with lymphadenosis benigna cutis, and 45 with localized scleroderma) and 21 control subjects. The results are expressed as a stimulation index (SI) (mean count per minute of triplicate cultures with stimulant divided by mean count per minute without stimulant). The serum samples from all patients and control subjects were tested for antibodies to B burgdorferi by indirect immunofluorescence assay. RESULTS: The 21 healthy seronegative controls had an SI of 3.3 +/- 2.0 (mean +/- SD). Compared with that of control subjects, the SIs were significantly elevated in patients with erythema migrans (9.8 +/- 9.1), acrodermatitis chronica atrophicans (11.8 +/- 8.2), and lymphadenosis benigna cutis (7.2 +/- 6.2). The 45 patients with localized scleroderma had elevated proliferative responses, with an SI of 6.5 +/- 7.3, but these responses did not significantly differ from those of controls. Elevated titers of antibodies to B burgdorferi were present in six (24%) of 25 patients with erythema migrans, five (38%) of 13 patients with lymphadenosis benigna cutis, and 13 (29%) of 45 patients with localized scleroderma. All 16 patients with acrodermatitis chronica atrophicans had markedly elevated antibody titers. CONCLUSIONS: Our findings show that a significant lymphoproliferative response to B burgdorferi occurs in the majority of patients with cutaneous manifestations of Lyme borreliosis. The lymphocyte proliferation assay may be of diagnostic value in patients in whom Lyme borreliosis is strongly clinically suspected and who have nondiagnostic levels of antibodies against B burgdorferi.

Acrodermatitis↗
PubMed1998

Demonstration of increased levels of type I collagen mRNA using quantitative polymerase chain reaction in fibrotic and granulomatous skin diseases.

Collagen changes occur in localized scleroderma, scleredema and sarcoidosis. Previous biochemical, immunohistochemical and in situ hybridization studies have revealed increased collagen synthesis in these diseases. In the present study, we measured by pro alpha 1 (I) collagen and beta-actin mRNA levels in skin punch biopsy specimens from lesional and healthy skin using a quantitative polymerase chain reaction (PCR). In this method, the targeted mRNA and a synthetic RNA as a internal standard are co-amplified together with the same primers. The amount of pro alpha 1 (I) collagen mRNA in cutaneous sarcoidosis lesions was found to be increased about two- to threefold compared with the values obtained for the healthy skin of the same two patients. In lesional skin of three patients with localized scleroderma the number of pro alpha 1 (I) collagen molecules was increased about two-fold. The beta-actin mRNA values were at the same level in the affected and unaffected skin of all the patients studied. In conclusion, a marked increase in type I collagen gene expression was seen in localized scleroderma and scleredema, leading to fibrosis of the skin, and in a granulomatous skin disease, cutaneous sarcoidosis.

Actins↗
PubMed1999

[Scleroderma in children: a retrospective study of 70 cases].

BACKGROUND: Scleroderma is uncommon in childhood. The aim of our study was to analyze the frequency of different clinical forms, their prognostic significance, biological features, and co-morbidities and to assess the pertinence of therapeutic options. PATIENTS AND METHODS: The files of 70 children with primary scleroderma seen from 1980 to 1997 were retrospectively reviewed. RESULTS: Localized scleroderma was observed in 56 children and diffuse lesions in 14. Localized scleroderma (44 girls, 12 boys) began early at a mean age of 7 years 2 months. The lesions presented as isolated bands (39 p. 100), associated with morphea (36 p. 100), or multiple morphea (5 p. 100). Mean duration of the clinical course was longer in cases with more and deeper lesions. Eosinophilia was observed at onset in 38 p. 100 of the cases and antinuclear antibodies were found in 28 p. 100. Local corticosteroid therapy (level I or II) appeared to be useful in the superficial and active lesions (morphea) but did not halt progression to deep scleroderma. General corticosteroid therapy (1 mg/kg/24 h) did not prevent the development of sequelae in cases with bands (16/16). Diffuse scleroderma corresponded to systemic scleroderma (6 cases), dual morbidity (dermatomyositis, mixed connective tissue disease) (6 cases), or scleroderma after eosinophil fasciitis (2 cases). Age at onset was around 9 years with female predominance. A particular gloves and socks form was observed and cardiac involvement was common, but there was no case of renal involvement. The therapeutic problems were similar to those in adults. DISCUSSION: Our findings emphasize that scleroderma occurs readily in childhood, unlike what has been reported 10 years ago. Prognosis depends on functional impairment resulting from major sequelae particularly important in localized forms and the life-threatening situations occurring in systemic forms.

Adrenal Cortex Hormones↗
PubMed1983

Progressive hemifacial atrophy (Parry-Romberg syndrome) report with review of genetics and nosology.

We describe a boy with mild hemifacial atrophy (Parry-Romberg syndrome); he had localized scleroderma on a leg and his trunk, and antinuclear antibodies in his serum. These findings support suggestions that this disorder could be a variant of localized scleroderma rather than a developmental anomaly or dysplasia. A review of the literature does not support assertions of autosomal dominant inheritance of the condition.

Adolescent↗
PubMed2006

[Diagnostic and therapeutic problems of scleroderma].

Scleroderma is the autoimmunologic disease with induration and fibrosis of the skin, subcutaneous tissue; sometimes refers to muscles, bones and other internal organs. Pathogenesis of morphea is still unknown. There are two main types of scleroderma: the first type is related only to the skin (localized scleroderma--morphea), while the second is connected with fibrosis and induration of the skin, lungs, heart and other organs (systemic sclerosis). There are various clinical forms of scleroderma circumscripta: linear scleroderma (the most frequent in children), morphea en plaque, generalized morphea, nodular and keloidea like, morphea guttata, scleroderma circumscripta with blisters on the surface-bullous morphea. Morphea profunda is the most severe type of scleroderma localized on the skin. Although the diagnosis of morphea is not so difficult, the treatment is problematic and not very effective. It is very important to initiate the proper treatment as soon as possible. There are three main ways of morphea treatment: medication (receiving local or per os), physical methods (for example phototherapy) and balneotherapy or climatic treatment.

Adolescent↗
PubMed2006

Simultaneous occurrence of linear scleroderma and homolateral segmental vitiligo.

Localized scleroderma and vitiligo only rarely have been reported to occur simultaneously. Here we report a case of a 21 year old man affected with both linear scleroderma of the left upper limb and homolateral segmental vitiligo of the trunk. Since the two diseases appeared during the same period, involved the same side of the body and their progression paralleled, a possible non-coincidental association between these two diseases is discussed.

Abdomen↗
PubMedDate not supplied

Long-term evaluation of colchicine in the treatment of scleroderma.

Fifteen patients with PSS, 4 with CRST syndrome, and 4 with localized scleroderma have been treated with colchicine at the maximum tolerated individual doses for an average of 39 months (range 19--57). All but 2 of the 19 patients with generalized scleroderma and 3 of those with localized scleroderma improved. Patients who had had scleroderma for less than 5 yr when begun on colchicine, and those who received total doses of colchicine of more than 1,438 mg, scored a significantly higher mean response index than those with longer disease duration, or those who received less colchicine.

Adult↗
PubMed1990

[Autoantibodies to 5 types of collagen in patients with circumscribed scleroderma].

Elevated levels of autoantibodies to types I-V collagens were detected in local scleroderma patients. In 75 percent of cases the levels of autoantibodies to types I and III collagens were elevated, in 30 percent to type II collagens, in 25 percent to type IV collagens, and in 20 percent to type V collagens. Anticollagen autoantibody levels did not differ from the normal values in the patients with a prolonged (for more than a year) remission. A reduction of these antibody levels almost to the normal values was achieved in 10-12 months after tigason therapy during a prolonged clinical remission. The ratio between autoantibodies to types I and II collagens was found to be a constant both in the donors and patients with local scleroderma.

Adult↗
PubMed1990

In vivo study of scleroderma by non-invasive techniques.

Sixteen patients with localized scleroderma (LS) and 11 with progressive systemic sclerosis (PSS) were studied using non-invasive techniques to determine skin thickness, skin extensibility, transcutaneous PO2 and cutaneous blood flow and were compared with normal controls. LS was characterized by a decreased skin extensibility with thickening of the skin in progressive lesions. There was an increase in the cutaneous microcirculation with a decrease in the PO2. In PSS, there was a decrease in the skin extensibility caused by thickening of the skin, but no change in cutaneous microcirculation or PO2 values. These results indicate that skin thickness and extensibility are the most useful parameters in the study of the progression of LS and PSS.

Adult↗
PubMed1987

Association of acrodermatitis chronica atrophicans and morphea.

A patient developed successively acrodermatitis chronica atrophicans (ACA) and localized scleroderma. ACA was demonstrated to be a tick-borne disease, whereas morphea is only suspected to be one. As morphea appeared after successful treatment of ACA, it suggests that localized scleroderma is not directly an infectious disease but might be rather the consequence of spirochetal infection.

Acrodermatitis↗
PubMed2001

Localized and systemic scleroderma.

Scleroderma is a broad term encompassing both localized and systemic sclerosis. Localized scleroderma is a cutaneous limited fibrosis that manifests as plaque morphea, generalized morphea, linear scleroderma, and deep morphea. Systemic scleroderma (sclerosis) can manifest as either limited or diffuse disease. Limited systemic sclerosis is typically preceded by Raynaud's phenomenon, involves cutaneous sclerosis distal to the elbows, with gastrointestinal and pulmonary fibrosis, and anticentromere antibody positivity. Diffuse systemic scleroderma is characterized by simultaneous Raynaud's phenomenon, cutaneous skin involvement proximal to the elbow with gastrointestinal, pulmonary, renal and cardiac fibrosis, and positive serology for antitopoisomerase and anti-RNAP III antibodies. This article discusses the classification, epidemiology, pathogenesis, clinical manifestations, treatment, and prognosis of the scleroderma.

Adult↗
PubMed1998

Histopathologic differentiation between localized and systemic scleroderma.

It is widely accepted that a histopathologic differentiation between localized scleroderma (LS) and systemic scleroderma (SS) is not always possible. With the objective of identifying differentiating histopathological features between them, 32 cases of LS and 19 cases of SS were reviewed. Histological features such as the distribution, composition, and density of the inflammatory cell infiltrate, thickness of the dermis, alterations of dermal collagen, and the presence of calcification were evaluated in each case. On the basis of our observations, LS and SS can be differentiated usually by the distribution and density of the inflammatory infiltrate and by the involvement of the papillary dermis. Inflammatory changes are more prominent in LS than in SS. Sclerosis of the papillary dermis is frequently seen in LS but is absent in SS.

Adolescent↗
PubMed1996

[Should watermelon stomach be considered to be an unrecognized localization of scleroderma?].

We reported a typical case of watermelon stomach which occurred during the course of a limited cutaneous systemic sclerosis. Watermelon stomach is an important source of upper gastrointestinal bleeding which requires endoscopic treatment. Such an association has already been described and we suggest that watermelon stomach could be an unrecognized localization of scleroderma stomach involvement. Because gut involvement may precede skin manifestations. A search of progressive systemic sclerosis should be done with clinical examination, antinuclear antibodies research (especially anticentromere antibodies) and nailfold capillaroscopy when a such endoscopic appearance is noted.

Gastrointestinal Hemorrhage↗