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PubMed1977

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.

Adolescent↗
PubMed1984

Blood flow of morphoea plaques as measured by laser-Doppler flowmetry.

Blood flow measurements by laser-Doppler flowmetry were performed on 15 patients with localized scleroderma (morphoea). Thirty morphoea plaques were studied by means of measurements of the sclerotic centre, the perilesional area, and regional control of the normal-appearing skin of the individual patients. Blood flow was increased (P less than 0.01) in the sclerotic area of all patient material and in the 10 patients having one or a few plaques (P less than 0.01). Five patients with generalized morphoea showed the same tendency. Blood flow was also increased in the perilesional area (P less than 0.01) of the total material, and in the patients with one or a few plaques (P less than 0.05), particularly in the case of a 'lilac ring'. 133Xenon washout measurements in 3 patients showed parallel results. The blood flow of plaques with clinically 'advanced' scleroderma was higher (P less than 0.01) as compared to plaques with 'slight' scleroderma. Measurements in pigmented spots after previous morphoea showed normal blood flow.

Adolescent↗
PubMed1983

Collagen synthesis in generalized morphea.

Synthesis of collagen and non-collagenous proteins was measured in fibroblast cultures derived from different layers of the dermis from a patient with an early stage of localized scleroderma. Increased synthesis of collagen was found in fibroblasts grown from the subcutaneous fat of this patient, whereas cells obtained from the papillary dermis revealed normal metabolism. These data agree with the results obtained in previous experiments with cells derived from patients with progressive systemic sclerosis in primary culture, thus indicating that the two diseases have a common pathomechanism. Several subcultures of the activated fibroblast populations were also studied. Normal collagen synthesis in these cultures was observed after the fifth passage, probably indicating selection of cell populations or loss of the previous phenotype.

Adipose Tissue↗
PubMed1990

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.

Adult↗
PubMed1984

Enophthalmos: a clinical review.

Twenty-six cases of enophthalmos were reviewed. The causes in order of frequency were: orbital asymmetry (8); trauma (5); orbital metastasis (4); microphthalmos (2); orbital varix (2); maxillary mucocele (2); localized scleroderma (1); absence of sphenoid wing (neurofibromatosis) (1); post irradiation atrophy (1). Only six of the patients (23%) were referred with the diagnosis of enophthalmos, suggesting the sign maybe subtle and is frequently missed or misdiagnosed. The nature of the causes underscore the need for careful and thorough diagnosis. In particular, the therapeutic implications of diagnosing metastatic disease, maxillary mucocele, and orbital varices is noted. A review of etiology and mechanisms of enophthalmos point to the diversity, importance and conditions causing this sign.

Adolescent↗
PubMed1988

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.

Biopsy↗
PubMed1979

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).

Aged↗
PubMedDate not supplied

Histologic observations of pleomorphic, variably acid-fast bacteria in scleroderma, morphea, and lichen sclerosus et atrophicus.

Variably acid-fast coccoid forms, suggestive of cell wall deficient forms of mycobacteria, were observed in the dermis in microscopic sections of skin from six patients with generalized scleroderma, 10 patients with localized scleroderma (morphea), and four patients with lichen sclerosus et atrophicus (LSA). These coccoid forms were found within the collagen bundles, around the adnexae (hair shafts, pilosebaceous units, eccrine glands), and less commonly around the blood vessels and nerves. These coccoid forms may be related to cocci and also to granular coccoid elements of corynebacteria-like coccobacilli, which, on occasion, can be cultured from the skin of these three diseases. The findings in this study support the three-decade old hypothesis concerning the constant association of pleomorphic acid-fast bacteria with scleroderma. The study also suggests that closely related diseases, such as morphea and LSA, are also associated with the presence of similar appearing microbes.

Adolescent↗
PubMed1991

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.

Child, Preschool↗
PubMed1992

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.

Aged↗
PubMedDate not supplied

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.

Adult↗
PubMed1989

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.

Eosinophilia↗
PubMed1992

[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.

Facial Dermatoses↗
PubMed1990

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.

Acute Kidney Injury↗
PubMed1977

[Idiopathic and sclerodermic facial hemiatrophy with generalized myopathy. Clinical, electromyographic and histologic examinations of six patients].

Six patients with facial hemiatrophy (H.f) were thoroughly examined by clinical and laboratory investigations. Two were found to have idiopathic and four facial hemiatrophy due to different types of localized scleroderma. In all cases a generalized myopathy was present, demonstrated by electromyographical, histological, and biochemical means. In none of these cases a hereditary or neurological cause for the facial hemiatrophy was found. However, in two cases autoantibodies against nuclei and muscle were repeatedly obtained. No prolongation of sensory or pain chronaxy occurred in either the patients with sclerodermal or idiopathic facial hemiatrophy. These observations suggest that facial hemiatrophy can originate (a) in a localized scleroderma in the involved part of the face, (b) in an ipsilateral "sclérodermie en coup de sabre", or (c) in the coexistence of both. There is a close pathological and physiological correlation between idiopathic and sclerodermal facial hemiatrophy. In both forms of facial hemiatrophy the disease involves skeletal muscle tissue systematically. This myopathy is similar to that of progressive scleroderma, far exceeding the limited muscular involvement of localized scleroderma. Sclerodermal facial hemiatrophy can be associated with autoimmune phenomena.

Adipose Tissue↗
PubMed1987

Antinuclear and anti-single-stranded DNA antibodies in morphea and generalized morphea.

The clinical features of localized scleroderma have allowed investigators to distinguish three morphologic variants: morphea, generalized morphea, and linear scleroderma. The latter has been reported to have a higher frequency of antinuclear antibodies and has been associated with antibodies to single-stranded DNA (ssDNA). In this study we determined the frequency of antinuclear antibodies and anti-ssDNA antibodies in 22 patients with morphea or generalized morphea. None had Raynaud's phenomenon or evidence of a systemic connective-tissue disease. Antinuclear antibodies were present in 18% of patients when serum samples were tested on mouse kidney substrate but were found in 50% of HEp-2 cells. The serum samples contained anti-ssDNA antibodies in 59% of the patients, with the highest levels of ssDNA binding observed in patients with generalized morphea. The frequency of antibodies to ssDNA was higher in patients with clinical evidence of active compared with inactive disease. Discordance in immune reactivity indicates that at least three distinctive serum autoantibodies exist in morphea and generalized morphea: anti-ssDNA antibodies and antinuclear antibodies with either homogeneous or speckled immunofluorescence patterns. These findings are similar to those recently described in linear scleroderma and suggest that comparable serum autoantibody abnormalities are present in all the variants of 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↗