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PubMed1996

Bronchiolitis obliterans in a patient with localized scleroderma treated with D-penicillamine.

D-penicillamine-associated bronchiolitis obliterans (BO) is a rare but well-known pulmonary complication in patients with rheumatoid arthritis or progressive systemic sclerosis. It has been assumed that in most, if not all cases, BO is a complication of the underlying disease rather than a side-effect of treatment. We report the case of a 46 year old man with scleroderma localized to his lower legs (morphea), who received a daily dose of 750 mg D-penicillamine. During the treatment of 1 yr duration, he developed progressive shortness of breath due to a worsening obstructive ventilatory defect suggesting BO, which was confirmed by surgical lung biopsy (constrictive BO). Bronchial obstruction progressed over the next 5 yrs and did not respond to corticosteroids. The patient finally underwent a successful single left lung transplantation. The histological features of constrictive BO were confirmed in the explanted lung. This observation suggests that D-penicillamine may induce bronchiolitis obliterans in the absence of a systemic connective tissue disease.

Antirheumatic Agents↗
PubMed2006

Congenital localized scleroderma.

OBJECTIVES: Juvenile localized scleroderma (JLS) usually has its onset during later childhood. This report describes the clinical and serologic features of six children with congenital localized scleroderma (CLS). STUDY DESIGN: A large, multinational study was conducted among pediatric rheumatology and dermatology centers by collecting information on demographics, family history, triggering environmental factors, clinical features, laboratory reports, and treatment of patients with JLS. Patients with onset at birth were carefully examined. RESULTS: Among 750 patients with JLS, 6 patients (0.8%) had scleroderma-related lesions at birth. Female-to-male ratio was 2:1. All patients had linear scleroderma, in four involving the face with en coup de sabre appearance. Two patients were misdiagnosed as having skin infection, one nevus, one salmon patch, and two undefined skin lesions. The mean diagnostic delay was 3.9 years. In comparison with the group of 733 patients with late-onset JLS, CLS presented a significantly more prolonged disease duration at diagnosis and a higher frequency of en coup de sabre subtypes. CONCLUSIONS: Congenital localized scleroderma is a rare and probably underestimated condition in neonates. The linear subtype was the exclusive manifestation of the disease. CLS should be included in the differential diagnosis of infants with cutaneous erythematous fibrotic lesions to avoid functional and aesthetic sequelae and to allow prompt therapy.

Atrophy↗
PubMedDate not supplied

Topical photodynamic therapy for localized scleroderma.

Therapy of localized scleroderma is unsatisfactory, with numerous treatments being used that have only limited success or considerable side-effects. The aim of this trial was to determine whether topical photodynamic therapy would be effective in patients with localized scleroderma. Five patients with progressive disease, in whom conventional therapies had failed, were treated by application of a gel containing 3% 5-aminolevulinic acid followed by irradiation with an incoherent lamp (40 mW/cm2, 10 J/cm2). The treatment was performed once or twice weekly for 3-6 months. In all patients the therapy was highly effective for sclerotic plaques, as measured by a quantitative durometer score and a clinical skin score. The only side-effect was a transient hyperpigmentation of the treated lesions. These cases document the beneficial effect of topical photodynamic therapy in localized scleroderma. Controlled trials are now necessary to confirm these preliminary results.

Adult↗
PubMed1999

Morphea and localized scleroderma in children.

Localized scleroderma refers to a diverse spectrum of disorders that involve fibrosis of the skin. Children are more likely than adults to develop localized forms of scleroderma. This condition may have devastating effects on growth and development such as limb asymmetry, flexion contractures, and psychological disability. The pathogenesis of localized scleroderma is unknown but its possible relation to Borrelial infection is discussed. This article reviews associated laboratory and radiologic abnormalities, and discusses implications for monitoring disease activity. There is no universally effective therapy for this idiopathic condition and therapy is limited. A rationale for treatment based on disease subtype and severity is provided.

Adrenal Cortex Hormones↗
PubMed1994

Localized scleroderma: imaging features.

Localized scleroderma is distinct from the diffuse form of scleroderma and does not show Raynaud's phenomenon and visceral involvement. The imaging features in 23 patients ranging from 2 to 17 years of age (mean 11.1 years) were reviewed. Leg length discrepancy and muscle atrophy were the most common findings (five patients), with two patients also showing modelling deformity of the fibula. One patient with lower extremity involvement showed abnormal bone marrow signals on MR. Disabling joint contracture requiring orthopedic intervention was noted in one patient. In two patients with "en coup de sabre" facial deformity, CT and MR scans revealed intracranial calcifications and white matter abnormality in the ipsilateral frontal lobes, with one also showing migrational abnormality. In a third patient, CT revealed white matter abnormality in the ipsilateral parietal lobe. In one patient with progressive facial hemiatrophy, CT and MR scans showed the underlying hypoplastic left maxillary antrum and cheek. Imaging studies of areas of clinical concern revealed positive findings in half our patients.

Adolescent↗
PubMed2005

[Nodules on localized scleroderma or morphea].

BACKGROUND: Localized scleroderma or morphea usually appears as flat or depressed lesions. OBSERVATIONS: We report 3 cases of morphea with atypical appearance, alternating pigmented and depigmented patches with nodules or sclerous bands, occurring in adult men. DISCUSSION: The occurrence of nodular elements on generalized or localized scleroderma, although rare, was first reported in the literature by Addisson in 1884. These nodules usually appear during evolution. These scleroderma are then described as being keloidal or nodular. We report 3 cases of nodules on localized scleroderma which appeared at the beginning of the dermatosis and where the scleroderma had a similar unusual irregularly pigmented appearance.

Adult↗
PubMed2006

Localized scleroderma.

PURPOSE OF REVIEW: Localized scleroderma, also known as morphoea, has a variety of clinical manifestations that can include systemic involvement. Early recognition, diagnosis and treatment may improve the long-term outcome. RECENT FINDINGS: A large multicentre study coordinated by the Pediatric Rheumatology European Society has yielded important information on the epidemiology and clinical manifestations of juvenile localized scleroderma, especially as it pertains to systemic manifestations. Previous results using methotrexate and corticosteroids have been confirmed. Studies on phototherapy have also demonstrated efficacy. A new immunomodulator, imiquimod, has shown promise in an initial case series. SUMMARY: Studies over the past year highlight the wide range of extracutaneous manifestations and different forms of localized scleroderma and suggest that treatment may be beneficial.

Clinical Trials as Topic↗
PubMed1996

Autoantibodies to mitochondrial 2-oxo-acid dehydrogenase complexes in localized scleroderma.

Sera from patients with localized scleroderma frequently produce cytoplasmic staining by indirect immunofluorescence, although the antigen remains to be determined. We studied the prevalence, antigen specificity and associated clinical characteristics of anti-cytoplasmic antibodies in localized scleroderma. Serum samples from 60 patients with localized scleroderma were examined by indirect immunofluorescence analysis and immunoblotting. By immunofluorescence analysis on HEp-2 cell substrate, seven of 60 (12%) patients were shown to be positive for anti-cytoplasmic antibodies. Among these, six patients with generalized morphea had anti-mitochondrial antibodies as shown by immunoblotting: they showed reactivity with the E2 component of pyruvate dehydrogenase complex (PDC), with protein X, and with the E2 component of alpha-oxo-glutarate dehydrogenase complex, while two of them showed reactivity with PDC-E1 alpha. One of these patients who was positive for anti-PDC-E1 alpha antibody showed laboratory abnormalities, suggesting the presence of primary biliary cirrhosis. The age of disease onset was significantly higher in these six patients than in those without anti-mitochondrial antibodies. Furthermore, five of them were classified into generalized morphea with multiple plaque lesions but without linear lesions (multiple plaque type). These observations suggest that major antigens for anti-cytoplasmic antibodies in patients with localized scleroderma are mitochondrial enzymes, 2-oxo-acid dehydrogenase complexes. Patients with anti-mitochondrial antibodies may comprise a unique subset of localized scleroderma designated multiple plaque type of generalized morphea of older onset.

Adolescent↗
PubMed1984

Clinical appearance of skin lesions and disturbances of pigmentation in localized scleroderma.

Skin manifestations of localized scleroderma were assessed clinically in 58 patients presenting 214 circumscribed scleroderma lesions (23 patients with localized morphoea plaques, 15 with generalized morphoea, 11 with linear scleroderma of trunk and extremities, 9 with scleroderma en coup de sabre). Characteristic differences between different types of localized scleroderma with respect to age at debut, regional distribution, symmetry and linearity, degree of sclerosis of the lesions, and the presence of inflammatory change of colour, pigmentation and visible atrophy of underlying subcutaneous tissue were found. Separate brown-pigmented spots resembling atrophoderma. Pasini-Pierini were observed in 49% of the patients. A regional distribution chart of linearity in localized scleroderma was elaborated. The frequent finding of disturbances of pigmentation, the character of the linear distribution-pattern, and affections of underlying anatomical structures is discussed to indicate a predisposing defect in the migration of crest cells during embryonal life.

Adolescent↗
PubMed2000

PUVA-cream photochemotherapy for the treatment of localized scleroderma.

BACKGROUND: The efforts to treat localized scleroderma, including therapies with potentially hazardous side effects, are often unsatisfactory. Recently, PUVA-bath photochemotherapy has been proven highly effective in the treatment of localized scleroderma. Another form of topical PUVA therapy, 8-methoxypsoralen (8-MOP) containing cream or gel preparations, has been proven to be as effective as PUVA-bath therapy for palmoplantar dermatoses. OBJECTIVE: We sought to assess the efficacy of PUVA-cream photochemotherapy in patients with localized scleroderma. METHODS: Four patients with localized scleroderma were included in the study. Diagnosis was confirmed by 20 MHz ultrasound assessment as well as pretreatment skin biopsy specimens from lesional skin. PUVA-cream therapy was performed 4 times a week; all patients received 30 treatments. RESULTS: PUVA-cream photochemotherapy induced significant clinical improvement or clearance of localized scleroderma in all patients. Clearance was documented by clinical features as well as by 20 MHz ultrasound and histopathologic analysis. CONCLUSION: PUVA-cream phototherapy can be highly effective in patients with localized scleroderma even if previous therapy was unsuccessful.

Adult↗
PubMedDate not supplied

Topical tacrolimus in the treatment of localized scleroderma.

Although the cause of localized scleroderma is unknown, an autoimmune mechanism is suspected. We describe two patients with localized scleroderma treated with topical tacrolimus, an immunosuppressive macrolide antibiotic. Topical tacrolimus 0.1% ointment applied twice daily under occlusion led to a significant clinical improvement of late sclerotic lesions and complete clearance of early inflammatory skin lesions in 3 months. These were the first cases of successful topical tacrolimus therapy in localized scleroderma and should be regarded as a promising treatment option for LS, especially on account of its high tolerability that permits prolonged use without side-effects.

Administration, Topical↗
PubMed1998

Low-dose UVA phototherapy for treatment of localized scleroderma.

BACKGROUND: For treatment of localized scleroderma numerous treatments, including ones with potentially hazardous side effects, are currently used with only limited success. OBJECTIVE: We attempted to determine the efficacy of low-dose UVA1 irradiation in patients with severe localized scleroderma. METHODS: Patients were irradiated with 20 J/cm2 UVA1 for 12 weeks (total number of treatments: 30; cumulative UVA1 dose: 600 J/cm2). RESULTS: Low-dose UVA1 irradiation induced significant clinical improvement (clearance of > 80% of lesions) in 18 of 20 patients. Clearance was documented by clinical score as well as by 20 MHz ultrasound and histopathologic analysis. CONCLUSION: Low-dose UVA1 phototherapy can be highly effective for sclerotic plaques, even in patients with advanced localized scleroderma and with lesions rapidly evolving despite conventional therapy.

Adolescent↗
PubMed1986

Antinuclear antibodies and anti-DNA antibodies in scleroderma. A possible relationship between joint manifestations and increased antibodies in localized scleroderma.

Antinuclear antibodies (ANA), including anti-DNA antibodies, and rheumatoid factors (RAT, Waaler-Rose) were determined prospectively during a 3-year period in 40 patients with localized scleroderma (LS) compared with 77 patients with generalized scleroderma (GS). ANA were increased in 26% of patients with LS, and in 47% with GS, anti-DNA antibodies in 23% of patients with LS, and in 34% with GS. Thus, the anti-DNA antibody level was lower compared with the known level in systemic lupus erythematosus. Rheumatoid factors were present in 6-7% of patients with LS, and in 14-15% of patients with GS. Increased antinuclear antibodies were not associated with any specific type of localized scleroderma, nor with internal disorders, and no case of clinical overlap to discoid or systemic lupus erythematosus was observed. However, six patients with localized scleroderma and complaints of arthralgia all presented increased antibodies, and one patient showed overlap to rheumatoid arthritis. It is suggested that increased ANA and anti-DNA antibodies in localized scleroderma, associated with joint manifestations, represents a systemic component in this type of scleroderma, with activation of the immune system and similarities with generalized collagen diseases.

Antibodies, Antinuclear↗
PubMed2001

[Thrombopenia associated to localized scleroderma].

Two patients are reported with localized scleroderma who developed thrombocytopenia. One of these patients had a positive antinuclear antibody (ANA) and Anti-DNA antibodies tests. The occurrence of thrombocytopenia in localized scleroderma suggests an autoimmune mechanism, by the response to steroids and the presence of positive autoantibodies. This possible association emphasizes the need to look for hematologic disorders in patients with systemic, as well as localized scleroderma.

Adolescent↗
PubMed1995

No evidence for Borrelia burgdorferi-specific DNA in lesions of localized scleroderma.

A possible association of Borrelia burgdorferi with localized scleroderma is currently the focus of intense research and discussion. Skin biopsies from 30 patients with localized scleroderma (28 of the plaque type/morphea; two linear scleroderma) were analyzed for the presence of Borrelia burgdorferi using three different polymerase chain reaction systems for amplification of segments of borrelial genes. Formalin-fixed, paraffin-embedded biopsies of 14 patients and fresh-frozen, cryo-conserved biopsies of 16 patients with localized scleroderma were obtained. Lesions of all patients showed clear signs of scleroderma and disease progression at the time of biopsy. Fresh-frozen as well as formalin-fixed biopsies from patients with erythema migrans or acrodermatitis chronica atrophicans were used as positive controls. With all three polymerase chain reaction systems, borrelial DNA was detected in none of the 30 specimens of localized scleroderma. In contrast, with one polymerase chain reaction system, Borrelia burgdorferi-specific DNA was found in 24 of 27 frozen biopsies from patients with erythema migrans and in all 5 analyzed frozen biopsies of patients with acrodermatitis chronica atrophicans. In approximately half of the paraffin-embedded biopsies from patients with erythema migrans (nine of 23) and acrodermatitis chronica atrophicans (13 of 27), Borrelia burgdorferi-specific DNA was identified. These results question the association of localized scleroderma with known subtypes of Borrelia burgdorferi.

Acrodermatitis↗
PubMed1997

High-dose UVA1 radiation therapy for localized scleroderma.

BACKGROUND: Fibrotic skin lesions in patients with localized scleroderma can cause muscle atrophy, disfigurement, and flexion contractures. There is no effective therapy for this disease. Skin fibrosis is thought to be caused by decreased collagenase activity. Collagenase activity can be induced in dermal fibroblasts by UVA1 irradiation. OBJECTIVE: Our purpose was to assess whether UVA1 radiation therapy is effective for patients with localized scleroderma. METHODS: Patients with localized scleroderma (n = 17) were exposed 30 times to 130 J/cm2 UVA1 (high-dose UVA1 therapy; n = 10) or 20 J/cm2 UVA1 (low-dose UVA1 therapy; n = 7). Therapeutic effectiveness was assessed by evaluation of (1) clinical features, (2) thickness of sclerotic plaques, and (3) cutaneous elastometry. Sequential biopsy specimens from treated lesions were analyzed for collagenase I messenger RNA (mRNA) expression by semiquantitative reverse transcriptase-polymerase chain reaction. RESULTS: In all patients, high-dose UVA1 therapy softened sclerotic plaques, and complete clearance was observed in four of 10 patients. High-dose UVA1 therapy significantly reduced thickness and increased elasticity of plaques. These changes could not be detected in unirradiated control plaques and were still present in 9 of 10 patients 3 months after cessation of therapy. For all factors assessed, high-dose UVA1 was superior to low-dose UVA1 therapy (p = 0.001). High-dose UVA1 therapy increased collagenase I mRNA expression about 20-fold in treated plaques. CONCLUSION: High-dose UVA1 therapy is effective in the treatment of localized scleroderma. Effectiveness is UVA1 dose dependent and is associated with induction of collagenase I expression.

Adult↗
PubMed1996

Systemic and localized scleroderma in children.

All forms of scleroderma are rare in childhood. The most common form in childhood is localized scleroderma, which may take the form of morphea or linear scleroderma. Localized scleroderma is often benign but may cause significant deformity if it occurs on the face or extends across joint surfaces. Progressive systemic sclerosis is much less frequent in childhood but may have a rapidly progressive and ultimately fatal course. CREST (calcinosis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, and telangieactasia) syndrome also may occur in childhood with manifestations similar to those seen in adults. In addition, some children in whom mixed connective tissue disease is initially diagnosed ultimately develop progressive systemic sclerosis. Research into both the causes and the optimal therapy for childhood scleroderma is hampered by the small number of patients treated at individual centers. This limitation has made it difficult to perform well-controlled studies.

Child↗