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Stiff skin syndrome (SSS) is a disease similar to scleroderma with an unknown etiology. Stone-hard areas of skin are observed from birth or in early childhood. In this article we describe a 15-year-old girl with skin hardening and limitation of movement. We diagnosed the case as SSS, of which we have not encountered a similar report in the Turkish literature.
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.
After the skin, the gastrointestinal tract is the most frequently affected organ in systemic sclerosis. Gastrointestinal symptoms already may be present early in the course of the disease and do not necessarily correlate with objective findings. Esophageal dysmotility is not specific for systemic sclerosis but occurs in other connective tissue diseases as well. Peripheral macrovascular disease was shown to be increased in patients with limited cutaneous sclerosis; signs of autonomic dysfunction were found in patients with the CREST (calcinosis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasia) variant. Pulmonary involvement was shown to be moderately or severely decreased in 40% of a large cohort of scleroderma patients. In one study, no support was found for the association between pulmonary involvement and gastroesophageal reflux. Peripheral nerve involvement is often subclinical and might be associated with anti-U1-RNP and anti-topoisomerase I antibodies. Internal organs are seldomly affected in localized scleroderma. When occurring in childhood and involving an extremity, localized scleroderma can cause growth failure, resulting in long-term functional disability.
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Autoantibodies to HMG-17, a non-histone nucleosomal protein, were found in systemic lupus erythematosus (SLE), mixed connective tissue disease (MCTD) and ANA positive pauciarticular juvenile rheumatoid arthritis (JRA), but not in rheumatoid arthritis (RA). Using highly purified HMG-17 derived from porcine thymus, we tested sera from 50 patients with scleroderma for antibodies to HMG-17 by enzyme-linked immunosorbent assay (ELISA). There were 16 patients with diffuse cutaneous systemic sclerosis (dSSc) and 34 with limited cutaneous systemic sclerosis (1SSc) with age at disease onset 40.25 +/- 11.68 and 39.94 +/- 15.68 years, respectively, and disease duration 6.03 +/- 4.98 and 13.34 +/- 11.80 years, respectively (P < 0.0001). Anticentromere antibodies (ACA) were found in 65% of 1SSc patients but not in dSSc (P < 0.0001) while the prevalence of antinuclear antibodies (ANA) with other than ACA patterns did not differ in the two groups. Anti-HMG-17 antibodies occurred in 20 patients (40%), five with dSSc (31%) and 15 with 1SSc (41%). Twelve of the 20 HMG-17 positive patients were also positive for ACA (60%) but this association was not significant. No association was found between anti-HMG-17 and other antibody patterns. In conclusion, anti-HMG-17 antibodies occur in one third of scleroderma patients, do not discriminate scleroderma variants and are not associated with other autoantibodies.
The prognosis and treatment of scleroderma depend on the clinicopathological form of the disease: superficial, localised scleroderma is only a minor cosmetic problem; circumscribed, deep forms of scleroderma may cause troublesome symptoms which justify steroid therapy. Circumscribed and diffuse scleroderma are similar to systemic scleroderma for which prognostic criteria have been identified. There is a poor prognosis in men, in extensive skin involvement and in intestinal, cardiac or renal involvement. Limited skin disease and anti-centromere antibodies are associated with a good prognosis. The absence of an animal model and the uncertainty as to the underlying cause, explain the therapeutic problems. The authors discuss the respective value of a number of "specific" drugs, including D-penicillamine which seems to be the most hopeful line of treatment. Continuous follow-up of this chronic condition remains essential for the diagnosis and symptomatic management of visceral complications.
An autopsy case-control study of renal vascular histology and morphometry in systemic sclerosis (scleroderma) was performed. Thirty-five of 70 systemic sclerosis cases had renal tissue available for study: 26 had diffuse cutaneous involvement (9 with "renal crisis" and 17 without) and 9 had limited cutaneous disease (CREST syndrome [calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasias]). Age-matched (within 10 years) and sex-matched controls with renal specimens were obtained. New sections were cut from tissue blocks, and morphometry was completed using a Zeiss Image Analyzer. Using analysis of variance, the intimal area (Ai) was significantly increased (intimal thickening) in small and medium-sized arteries of patients with diffuse scleroderma and in small arteries of CREST patients, compared with those in controls, while a decreased medial area (Am) was seen consistently in all groups. The proportion of the vessel wall occupied by intima (Ai:[Ai + Am]) was significantly greater in all vessel size groups in patients with diffuse scleroderma compared with that in controls. The percentage of luminal occlusion was greatest in patients with diffuse disease with renal crisis. These same patients had severe edematous and mucinous intimal thickening in small and medium vessels, often in association with fibrinoid necrosis. We conclude that renal vascular structural changes are an integral part of systemic sclerosis. However, the significant differences between diffuse scleroderma patients and CREST syndrome patients, for both intimal thickening and percentage of luminal occlusion, suggest that the arterial disease in these 2 patient subsets is distinctive.
Sensory neuropathy of trigeminal nerve is one of the neurological complications of systemic diseases, particularly scleroderma. Three cases are reported in which onset occurred at different stages of the disease, the main symptom being painful dysesthesias. All three branches of the trigeminal nerve may be affected but a preference is apparent for V2 and V3. Signs are usually limited to hypoesthesia and the chronic course is influenced little by treatment.
In Wegener's granulomatosis peripheral nerves are more often involved than central nervous structures; functional psychoses are secondary e.g. to renal complications. Neurologic-psychiatric symptoms are varied and result from local spread from the upper respiratory tract with separate granulomata and vasculitis. Striped muscle is involved in about 10% of cases. Specific vegetative disturbances of function do not appear to occur. This is also true for Granuloma gangrenescens. Its multicentric inflammatory, ulcerating and necrotic foci involve midline structures and often start at the anterior neuroporus. Becet's disease, however, shows clear characteristic localisation, symmetry of various dermatologic phenomena in the extremities and the almost obligatory involvement of mouth, stomach and the genitals and their surroundings. This one may assume to be due to a mesencephalic parasympathicotonic reaction as the basis for the occurrence of perioral and acro-syndroms after Fischer-Brügge and Sunder-plassmann. Some similarity with the localization of other collagenous diseases is evident. The anterior basal ganglia represent a favored focus where e.g. initial, imcomplete and readily recurrent processes, possibly of an exsudative nature, may start off neurovascular changes of limited areas without necessarily producing clinically detectable signs of the usual neurologic-psychiatric type. In Behcet's disease, rather as in scleroderma, the nervous system appears to be decisively involved. About one third of the cases show changes in the nervous system ("Neuro-Behcet" in the usual sense) with little peripheral signs and rare myositis. But Behcet's disease may also-like erythematodes or progressive sclerodermia-simulate a disseminated encephalomyelitis. The pathogenetic factors of Reiter's syndrome appear to be closely related. The relatively constant main symptoms are also localized and permit the conclusion that they depend on vasomotorically linked innervation of the first bulbar segment. The preference for distal localization of bilateral skin involvement hints - as in Behcet's disease at hypothalamic distribution; main localization in midface and the oral mucosa, in eyes and the ends of the midline and, often, also involvement of certain midline and, often, also involvement of certain favored joints permit the assumption of mutural relations between the cranial and sacral fields of incidence of the trophotrop-endophylactic system which is linked via the parasympathic with periphery. According to present knowledge the bundles of Schütz and, further, Krüke may be the pathways. Where pelvic processes predominate viscero-cutaneous or cuto-visceral reflexes may - as in dermatomyositis - be involved. E.g visceroreflectory changes explain coincidences of signs in similar or adjacent segments of the urogenital system, the rectum and certain joints. Psychose on a physical basis are rare. As in other collagen diseases the spinal cord is rarely involved, but "multiple sclerosis" may be simulated...
Supplemental hand scintigrams with abnormal features were obtained from 29% of patients (134 of 463) who were referred for routine minified bone imaging with 99mTc-Sn-polyphosphate. A wide spectrum of normal activity distribution ranging from well-defined to "wash-out" images is described in 329 cases (71%). In the abnormal images of the joints and individual bones, the changes--although not always characteristic of some particular disease--may often suggest a diagnosis and/or its pathophysiologic status. The joints with heavy uptake correlate well with the presence of active clinical findings, e.g., in the arthritides. The bone feature associated with metabolic disease, especially when full-blown, may be fairly characteristic. A potential application is in the assessment of digital circulation, particularly in obliterative vascular diseases such as scleroderma, Buerger's disease, chronic neuropathies, and possibly other collagen or vascular diseases that involve the hands. Interesting images, probably of somewhat limited usefulness, are observed in some congenital anomalies, fractures, camptodactyly, contracture deformities, unilateral lymphedema after mastectomy, etc.
Organs which contain collagen can have connective tissue new growth in systemic scleroderma. The occurrence of scleroderma of the larynx and organs of speech is rare. The disease develops in the mucous membrane in three stages: oedema; infiltration and induration; and atrophy. The oedema stage is generally the shortest. Three patients with systemic scleroderma with involvement of larynx and organs of speech have been seen. In one is described (graphically, sonographically and with stroboscopy) the progress of the disease from an initial severe hoarseness to later resolution of the oedema. In another patient involvement of the soft palate produced rhinolalia. In spite of the limitation of tongue movement in 2 patients there was no disorder of articulation.
The efficacy of plasma exchanges (PE) during the course of scleroderma has only been investigated for short periods. The aim of this study was to follow patients over a long enough period to observe the course of the clinical and paraclinical symptoms in the short, medium, and long term. Forty patients, 24 women and 16 men, were treated by PE and observed for 1-3, 3-12 and over 12 months. Immunological, biological and clinical course and any undesirable side effects were evaluated using a detailed questionnaire. Concomitant therapies were reported and most frequently consisted of corticosteroids, colchicine, factor XIII or vasodilators (nifedipine, captopril). The therapeutic effectiveness of PE was assessed on the basis of improvements in cutaneous, digestive, joint, muscular, lung, cardiovascular and renal lesions. Our findings confirmed the effectiveness of short-term PE on scleroderma (52% of the patients improved during the first 3 months). However, this improvement was transient (5% improvement between 3 and 12 months and only 2.5% over 12 months) and limited to the cutaneous and muscular lesions. Thus, PE cannot be recommended for the treatment of progressive systemic sclerosis.
A 54-year-old Korean male with scleroderma-like manifestation of primary systemic amyloidosis presented with firm cutaneous induration of face and distal extremities, subcutaneous induration of the trunk and proximal extremities, limited range of motion in all joints, hoarseness, and dysphagia. Monthly high-dose intravenous immunoglobulin (hdIVIg) was given (three treatments, each time administering 0.4 g/kg per day for five days), and both signs and symptoms began to improve. However, the quantitative analyses of serum protein did not improve. Therapeutic plasma exchange (TPE) was performed monthly to clear the elevated serum immunoglobulin, and after several treatments, their levels normalized and symptoms were maintained in the improved state for more than two years. To summarize, hdIVIg and TPE combination therapy may be used as a safe first-line treatment for patients with primary systemic amyloidosis presenting with symptomatic monoclonal gammopathy.
There are closed relations between progressive systemic sclerosis (PSS) and exposure to quartz dust in the GDR. The recognition of PSS as an occupational disease is regulated by law. By analysis of the dust exposed working places a limit for silica concentrations were fixed. It is unlikely, that silica concentrations below 10% in the respirable dust induce a PSS. There are no significant differences in lung function of PSS-patients with and without quartz exposure.
In 28 patients with progressive systemic sclerosis clinical symptoms are correlated with roentgen findings. The systemic disease of connective tissue shows typical roentgenological signs, such as soft tissue calcifications, generalized or localized osteoporosis, arthritis, dysfunction of oesophagus, small and large bowel, pulmonary fibrosis and cardiomegaly. A limited prognostic statement according to a clinical and radiological classification seems possible.
We report an unusual scleroderma case. A 45-year-old woman had recurrent morphea lesions over 10 times in 6 years. She had had preceding inactive systemic scleroderma. New morphea lesions developed cyclically on various portions of her body and improved within 2 years. Interestingly, new lesions have developed on once involved skin as well as uninvolved skin. No exacerbation of systemic scleroderma was induced by outbreaks of new morphea lesions. We could not find a similar case in the literature and named it "Palindromic morphea" because of its unique clinical course. D-penicillamine treatment had a limited effect. Minimal oral prednisolone (5 mg/day) completely suppressed the multiple recurrence of the morphea lesions and enhanced improvement of the sclerosis.
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