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Localized scleroderma--en coup de sabre.
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[Ultrastructural study of the skeletal muscle in localized scleroderma].
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Morphoea (localized cutaneous scleroderma) treated with low-dosage penicillamine (4 cases, including coup de sabre).
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Treatment of localized linear scleroderma with phenytoin.
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[Detection of pulmonary hypertension in patients with local circumscribed scleroderma].
In three patients with local circumscribed sclerodermia (duration of the disorder 6 months to 3 years), a latent or manifest pulmonary hypertension of the precapillary type in the presence of otherwise unremarkable lung function, radiological and ergometric data, was observed. The pulmonary involvement in this disease would appear to have a relatively early onset.
Expression of osteonectin, decorin, and transforming growth factor-beta 1 genes in fibroblasts cultured from patients with systemic sclerosis and morphea.
A characteristic feature of fibroblasts cultured from affected skin areas of patients with systemic sclerosis (SSc) and localized scleroderma (morphea) is excessive activation of collagen biosynthesis. To elucidate the nature of fibroblast activation in scleroderma we have studied the expression of 3 noncollagenous connective tissue components, osteonectin, small dermatan sulfate proteoglycan (proteoglycan II, decorin), and transforming growth factor-beta 1 (TGF-beta 1), by measuring their mRNA levels in fibroblast cultures from 6 patients with SSc and 3 with morphea. A clear correlation was observed between the increase in type I collagen and osteonectin mRNA in these cell lines. The apparent overproduction of osteonectin by scleroderma fibroblasts is in accordance with the suggested activation of osteonectin expression during tissue remodeling. The levels of decorin mRNA showed marked variation in the cell lines, but were in no correlation with collagen or osteonectin mRNA. The levels of TGF-beta 1 mRNA were found to be slightly elevated in fibroblasts grown from affected scleroderma skin. This may suggest that this potent activator of collagen production has a role during the initial activation of dermal fibroblasts both in SSc and morphea.
Localized scleroderma-like lesions after bone marrow transplantation in man. A chronic graft versus host reaction.
Localized scleroderma-like skin lesions which developed in two children, from 8 to 10 months after successful bone marrow transplantation for aplastic anaemia, showed histopathological features resembling those of scleroderma. This finding, like the animal models described in the literature, provides additional support for the auto-immune nature of scleroderma.
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.
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.
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.
Diameter of the collagen fibrils in the sclerodermatous skin of porphyria cutanea tarda.
The diameter of collagen fibrils was studied in a patient with porphyria cutanea tarda in a specimen obtained from the sclerodermatous skin of the back. A bimodal distribution of the diameter of the fibrils was observed with maxima at 33.5 nm and 68.7 nm. The mean diameter was 67 nm with a standard deviation of 10.6 nm. These results are similar to those obtained previously in localized scleroderma (morphoea).
Solitary morphea profunda in a 5-year-old girl: case report and review of the literature.
A 5-year-old girl had a solitary sclerotic plaque on the back of recent onset. The histopathologic features were consistent with morphea profunda. Thickening and homogenization of collagen bundles were demonstrated in the dermis and subcutaneous tissues, admixed with a prominent lymphocytic and plasma cell inflammatory infiltrate. Solitary morphea profunda is a variant of localized scleroderma that has not been reported previously in childhood. Cases described in the literature as nodular or keloid morphea may represent a similar entity.
Vitiligo-like depigmentation and morpheas after specific intralymphatic immunotherapy for malignant melanoma.
We report the case of a patient with stage 2 malignant melanoma (MM), who received a specific immunotherapy consisting of intralymphatic injections of irradiated MM cells. She developed subsequently vitiligo-like leukoderma and several plaques of localized scleroderma. Simultaneously an increase in the patient's cytotoxic activity against MM cells was detected in vitro. The responsibility of immune phenomena and specific immunotherapy for the appearance of depigmentation and morpheas is discussed.
Ichthyosiform and morpheaform sarcoidosis.
Specific (usually papules and nodules showing a granulomatous histology) and non-specific (e.g. erythema nodosum) cutaneous lesions presenting manifestations of sarcoidosis have been well described. Two patients with unique presentations are here described; one with ichthyosiform cutaneous lesions and one with not previously described cutaneous lesions which mimicked linear morphea (localized scleroderma). The association of articular manifestations without pulmonary involvement was also unusual.
Eosinophilic fasciitis.
The fascia had received little attention until Shulman's delineation of EF. Evidence is now accumulating that in addition to EF and scleroderma, significant fascial inflammation may be seen in polymyositis, dermatomyositis, eosinophilic polymyositis, systemic lupus erythematosus, and mixed connective tissue disease. It is still unclear whether EF represents a variant of scleroderma; however, it is becoming increasingly recognized that scleroderma shares many features in common with EF including eosinophilia, hypergammaglobulinemia, positive ANA and rheumatoid factor, and an association with hematologic disease. The rarity of Raynaud's phenomenon and significant visceral changes help distinguish EF from systemic scleroderma. In this regard, however, EF more closely resembles the localized scleroderma syndromes, especially morphea profunda and pansclerotic morphea. Biopsy in EF, systemic scleroderma, and localized scleroderma will show comparable changes, the essential difference being the levels at which they occur.
[Facial hemiatrophy, homolateral cervical linear scleroderma and thyroid disease].
A case of facial hemiatrophy and homolateral cervical band of scleroderma, complicated by hypothyroidism is reported. This case raises two problems: one is the problem of distinction between Romberg's disease and facial hemiatrophy due to a genuine localized scleroderma; the other concerns the relationship between localized scleroderma and dysthyroidism. The generalized scleroderma-dysthyroidism association has now been recognized, but the coexistence of thyroid disease and localized scleroderma has not yet been reported. Several pathogenetic hypotheses on this association are discussed.
Cyclosporin in localized and systemic scleroderma--a clinical study.
Four patients with systemic scleroderma and 1 patient with localized scleroderma were treated with ciclosporin (CS), given in daily doses between 2.2 and 5.6 mg/kg body weight for 3-26 months. Under this medication clinical improvement was observed in 4 patients with partial regression of cutaneous sclerosis and inflammation, healing of fingertip ulcerations or leg ulcers and improvement of articular mobility. However, in 1 patient with rapidly advancing systemic scleroderma a short-term therapy with CS in low doses (2-3 mg/kg body weight) resulted in arterial hypertension and renal dysfunction. Therefore careful selection of patients and close-meshed controls are indicated when CS is considered as anti-inflammatory treatment in scleroderma.