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PubMedDate not supplied

[Localized scleroderma in childhood].

Scleroderma is a rare disease in children: the clinical presentation in childhood is even more varied than in adult life. It is characterized by 'hard skin' with cutaneous features including hypo- and hyperpigmentation, thickening or thinning and loss of elasticity. It ranges from circumscribed and self-limiting pigmentary disorders to disabling and disfiguring involvement of an extremity and a rapidly fatal outcome. Scleroderma must be differentiated from many scleroderma-like conditions. Therapeutic problems are also discussed.

Adrenal Cortex Hormones↗
PubMed1989

Oesophageal motility and lower oesophageal sphincter competence in progressive systemic sclerosis and localized scleroderma.

Oesophageal motility and lower oesophageal sphincter (LOS) competence were investigated in 13 patients with progressive systemic sclerosis (PSS) and in 16 patients with localized scleroderma (LS) by means of oesophageal manometry and 24-h pH monitoring of the distal oesophagus. Results were compared with those of a control group consisting of asymptomatic volunteers. Marked abnormalities in oesophageal motility and in acid exposure in the distal oesophagus were observed in PSS patients only. The mean resting pressure of the LOS was 10.1 +/- 1.5 mmHg in PSS, 21.4 +/- 1.1 mmHg in LS, and 23.8 +/- 2.0 mmHg in asymptomatic controls. Overall sphincter length was 24.1 +/- 3.4 mm in PSS, 31.1 +/- 1.6 mm in LS, and 39.0 +/- 2.0 mm in the control group. Spincter abdominal length was 12.1 +/- 2 mm, 15.4 +/- 1 mm, and 25.0 +/- 1 mm, respectively. The amplitude and duration of oesophageal waves were markedly reduced at 5, 10, and 15 cm above the LOS in PSS patients, with only the upper part of their gullet being spared. An abnormal acid exposure in the distal oesophagus was observed in 84.6% of PSS patients, whereas only 18.2% (2 of 11) of pH-tested LS patients had an abnormal 24-h pH test. These data show that a marked oesophageal involvement is present only in the systemic form of scleroderma. Oesophageal tests may be useful for a circumstantial diagnosis whenever the diagnosis of PSS is uncertain; however, their use does not seem to be justified as routine in patients with LS.

Adult↗
PubMed1988

Flow cytometry of fibroblasts cultured from skin of patients with localized scleroderma.

Fibroblast cultures were initiated from affected and unaffected skin sites of 6 patients with localized scleroderma. Two of the affected cell lines exhibited more than threefold increases in procollagen production and mRNA levels. All the cell lines were analyzed by flow cytometry to detect a possible heterogeneity of scleroderma fibroblast cultures which could explain the production of excessive amounts of collagen. No evidence of a subpopulation responsible for elevated collagen production was detected using cytoplasmic dot hybridization of cells fractionated by flow cytometry. When compared with the nonaffected controls, all the cell lines from affected skin areas of scleroderma patients were found to exhibit a lower level of cellular autofluorescence, suggesting an alteration in metabolic activity. The results show that the heterogeneity of scleroderma fibroblasts that was found in vivo is lost when the cells are cultured.

Adolescent↗
PubMed1988

Identification of fibroblasts responsible for increased collagen production in localized scleroderma by in situ hybridization.

Skin biopsies from seven patients with localized scleroderma (morphea) and from two healthy individuals were studied by in situ hybridization to localize the cells responsible for increased procollagen production. In scleroderma lesions, high levels of pro alpha 1 (I) and pro alpha 1 (III) collagen mRNAs were detected in some but not all fibroblasts, suggesting the presence of a subpopulation responsible for the increased collagen production. The levels of pro alpha 1 (I) and pro alpha 1 (III) collagen mRNAs in these fibroblasts were clearly elevated compared to control skin specimens hybridized at the same time under identical conditions. Most of the scleroderma samples represented intermediate stages where the fibroblasts containing elevated levels of type I and type III procollagen mRNAs were located in the papillary and upper reticular layer of the dermis. One of the scleroderma samples from an early inflammatory stage of the disease was found to contain activated fibroblasts in all dermal layers and also in aggregates adjacent to inflammatory cell infiltrates. In situ analyses were also performed on cell cultures from affected and unaffected skin of one scleroderma patient. These experiments revealed a homogeneous population of activated fibroblasts in cultures producing high levels of collagen. The results suggest that development of fibrosis in scleroderma could evolve through activation of a certain fibroblast subpopulation. During cell culturing, however, cell selection or uncharacterized regulatory mechanisms appear to modulate the behavior of these cells with respect to collagen production.

Autoradiography↗
PubMed1978

Concurrent localized scleroderma and discoid lupus erythematosus. Cutaneous 'mixed' or 'overlap' syndrome.

Four patients with concurrent, chronic, progessive, localized scleroderma and discoid lupus erythematosus were studied; the condition originated as linear scleroderma in three of them. Three of the four patients were young females at the onset of the first skin disease. Dermatopathologic study confirmed the scleroderma and lupus erythematosus (LE). Direct immunofluorescence showed a positive band test in three cases. Unusual serological results included a positive LE clot test in three cases, a positive extractable nuclear antigen test in one case, and a negative antinuclear antibody test on repeated occasions in all four cases. Rare cutaneous disease similar to systemic, "mixed," or "overlap" connective tissue disease exists and offers an opportunity to study unusual immunologic and pathological events in both scleroderma and LE.

Adolescent↗
PubMed1989

Serum aminoterminal propeptide of type III procollagen in progressive systemic sclerosis and localized scleroderma.

Sera from 31 patients with progressive systemic sclerosis (PSS), 5 patients with widespread localized scleroderma (LS), and 3 patients with lichen sclerosus et atrophicus were analyzed for aminoterminal propeptide of type III procollagen (PIIINP) using a radioimmunoassay based on human propeptide. Thirty-eight per cent of the patients with PSS had levels above normal range, including all of the 3 patients with diffuse scleroderma. The same applies to 4 of 5 patients with widespread localized LS, while PIIINP in all 3 patients with lichen sclerosus et atrophicus were within normal levels. In patients with acrosclerosis, elevated PIIINP seems to be correlated to rapid progression and extension of lesions. A significant increase in PIIINP was found in a patient following discontinuation of prednisone and cyclophosphamide, while the present investigation did not allow judgement of effects of treatment with either penicillamine or cyclosporin A.

Humans↗
PubMed1975

Localized scleroderma.

Familial scleroderma is rare; only seven documented instances of the disease have been reported, to our knowledge. This report adds two more families to the literature. Three children in one family and two in the other had clinically and histiologically established localized scleroderma.

Adolescent↗
PubMed1992

No evidence for a spirochaetal origin of localized scleroderma.

We looked for evidence of a Borrelia infection in 15 patients with morphoea. We were not able to detect antibodies to Borrelia burgdorferi in any of these 15 patients. None of the 14 skin biopsies examined by immunohistochemistry showed evidence of spirochaetes. Skin biopsies were cultured in 10 patients. All were negative. These results do not support a spirochaetal origin of localized scleroderma.

Adolescent↗
PubMed1990

[Histological study on localized scleroderma].

To define stage-specific and type-specific histological findings in morphea, 21 (14 plaque, 2 linear, and 5 en coup de sabre type) patients were examined. A) Fibrotic changes; 1) Every case had the fibrotic change of various degrees. 2) Nodular sclerotic fibrosis was found in 7 cases with morphea, namely in 5 of 11 cases with a plaque type and 2 of 4 en coup de sabre type morphea. And the above mentioned 7 cases were within two years since the onset, and no nodular fibrosis was found in the morphea with the longer duration than two years in history. Nodular fibrosis was located in the middle or lower dermis adjacent to the typical sclerotic dermis. It was strongly suggested that this nodular fibrosis is as an initial change of localized scleroderma. 3) Nodular fibrosis expanded to the neighboring area and made a typical histological feature of morphea which is completely different from that of diffuse scleroderma. B) Inflammatory findings; 1) Inflammatory changes were divided into, a perivascular, a diffuse or non-perivascular, and a mixed type. 2) The pure perivascular type was found in 10, the pure diffuse type only in one, the mixed type in 6 cases, and the other 4 cases had no inflammation. 3) Marked or moderate inflammation was found in cases with short history of the disease except for one case. 4) Inflammatory cells in the morphea were mainly composed of lymphocytes and histiocytes, but occasionally of plasma cells in 11 of 17 cases. C) Pigmentary changes; Incontinentia pigmenti was found in 18 of 21 cases.

Adolescent↗
PubMedDate not supplied

[Localized scleroderma (morphea) and septic arthritis. Clinical manifestations of Lyme borreliosis seen in El Ferrol].

Two cases of Lyme's disease seen at El Ferrol (Spain) were described. One of them developed a recurrent knee arthritis and the other had a localized sclerodermia (morphea) syndrome. Diagnosis was established by means of clinical picture and serologic tests (enzyme-linked analysis and/or indirect immunofluorescence tests). Joint involvement has often been described in patients diagnosed of having Lyme's disease in Spain, however, the relationship between morphea and borreliosis is still a matter of controversy. We believe that patients with localized sclerodermia and high significant titers of specific antibodies against B. burgdorferi should be treated with antimicrobial agents.

Adult↗