SKIN COLLAGEN CONTENT AND THICKNESS IN SYSTEMIC SCLEROSIS

The thickness and collagen content of forearm skin were measured in 13 patients with systemic sclerosis, in 8 of whom the forearm skin was found to be clinically affected by the disease. No increase in skin thickness or its collagen content was found. The thickness and collagen content of the clinically affected forearm skin were usually decreased and collagen density was normal. It is concluded that the clinical impression of thickness and toughness is due to binding down of the skin to deeper structures. The similarity of atrophic morphoea and systemic sclerosis is discussed.     

THE SKIN CHANGES IN SCLERODERMA (SYSTEMIC SCLEROSIS)

Scleroderma (systemic sclerosis) may be subdivided into an acrosclerotic form (Types 1 and 2) and a diffuse form (Type 3) on the extent of the skin involvement. The acrosclerotic form has generally a relatively good prognosis and the diffuse form a poor prognosis. Although there is a general tendency for the skin sclerosis to progress, the change may be slow and may halt. Other skin manifestations include pigmentation, telangiectases, calcinosis, hyperkeratoses and various forms of ulceration. Recent studies on electron microscopy and biochemical findings in scleroderma skin are described. They indicate the presence of young collagen.     

GLYCOSAMINOGLYCAN IN THE SKIN AND URINE OF A HUNTER SYNDROME PATIENT WITH A SPECIFIC SKIN LESION

The glycosaminoglycan (GAG) contents of the cutaneous papules, the non-eruptive skin, and the urine of a patient with Hunter syndrome were examined. Both skin samples contained hyaluronic acid (HA) and dermatan sulfate (DS) as major components and heparan sulfate (HS) and chondroitin sulfate (CS) as minor components. The HA content of the papules was greatly increased, while that of the non-eruptive skin was normal. The amounts of DS and HS were increased in all three samples; the papules, non-eruptive skin, and urine. HS from the patient's skin and urine had an electrophoretic mobility different from that of authentic HS. It seemed interesting from a pathogenetic viewpoint that the components of GAG in the cutaneous papules differed from those in the non-eruptive skin and urine.     

SUBCUTANEOUS CHROMAOBLASTOMYCOSIS OCCURRING IN A PATIENT WITH SYSTEMIC LUPUS ERYTHEMATOSUS

A case of subcutaneous chromoblastomycosis occurring in a patient with systemic lupus erythematosus is presented. Because of the probable association with immunosuppression and the varity of the condition we feel this case should be reported.     

IMMUNOLOGIC PARAMETERS IN SYSTEMIC SCLEROSIS

Background. Immunologic abnormalities seem to play an important role in systemic sclerosis (SSc). Methods. We studied the following immune parameters to get more insight into SSc: autoantibodies (antinuclear antibodies (ana ), anti-Scl-70, anticentromere antibodies (aca ) subsets of lymphocyte subpopulations and markers of their activation, as well as serum levels of il -2, the soluble il -2 receptor (sil -2r ), il -6 and its correlation to N-terminal procollagen-Ill propeptide (p iii p ), and finally, the il -6 production by SSc and normal dermal fibroblasts. Results. In patients with active SSc, we found a reduced number of cd 2+ T-lymphocytes and an increase in the expression of T-lymphocyte activation markers such as cd 25+ and cd 71+, hla -dr la, as well as elevated serum levels of sil -2lr and il -6. SSc fibroblasts did not produce more il -6 than normal fibroblasts in monolayer cultures. Conclusions. Our data show that a wide range of immunologic parameters are altered in SSc. In general, T-helper (th ) lymphocytes are activated possibly because of reduced T-suppressor (ts ) and natural killer (nk )-cell levels, TH may polyclonally stimulate B cells, which in turn produce higher amounts of autoantibodies. Our findings support the concept that TH cell-derived cytokines/growth factors stimulate matrix protein synthesis by fibroblasts, resulting in generalized fibrosis.     

ABNORMAL AND UNSTABLE PATTERNS OF THE DNA OF STROMA CELLS IN A PATIENT WITH TUBEROUS SCLEROSIS

Explant cultures were prepared using normal-looking skin and the so-called adenoma sebaceum (AS) from the face of a 42-year-old male with tuberous sclerosis, Pringle's disease (PD), and the DNA content of the subcultured non-epithelial cells (NECs), stroma cells, were investigated per nucleus and per cell by a flow cytofluorometer.

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PROGRESSIVE SYSTEMIC SCLEROSIS ASSOCIATED WITH CUTANEOUS AMYLOIDOSIS

A 43-year-old woman presented with a 3-year history of Raynaud's phenomenon and a six-month history of numbness in both arms. Sclerosis was noted on the entire body surface. The skin of the face was smooth and the lips were constricted (Fig. 1). The fingers and hands were atrophic and sclerotic, and full extension of the fingers and metacarpal joints was impossible (Fig. 2). There was pigmentation on the dorsal aspect of the hands. From the nape to the upper back, pruritic wavy, rippled or reticular pigmented macules in addition to sclerosis were noted (Fig. 3). Other parts of her skin did not show such a wavy pigmentation. Physical examination revealed no specific findings in the lung, heart, and abdomen. Neurologic examination was unremarkable. Motor function including muscle tonus was normal.
Laboratory studies disclosed that the complete blood count and tests of hepatic and renal function were within normal limits. Antinuclear antibody was I:80 and showed a speckled pattern, antitopoisomerase 1 antibody, anticen-tromere antibody, anti-Sm antibody and anti-RNP antibody were all negative, and the CH50 was 31.5 units/ml; C3 was 59.4 mg/dL; and C4, 17 mg/dL. Radiologically the chest and esophagus were normal.
Pulmonary function and electro-cardiogram were also normal. Histologic examination of a skin biopsy obtained from the upper back revealed that the collagen bundles throughout the dermis were thickened, homogenous, and closely packed. In the upper dermis, a small number of inflammatory cells around blood vessels was observed. Eosinophilic homogeneous masses were seen in the papillary dermis and upper dermis (Fig. 4). These homogeneous masses were positive to Dylan' (Fig. 5), Congo red, and thioflavin T staining. Therefore, the diagnosis of cutaneous macular amyloidosis was made.     

EXTRACORPOREAL PHOTOCHEMOTHERAPY IN PROGRESSIVE SYSTEMIC SCLEROSIS

Background. Extracorporeal photochemotherapy, an immune-modulating form of therapy, has been shown to be effective in the treatment of autoimmune diseases. We evaluated the effects of extracorporeal photochemotherapy in the treatment of patients with progressive systemic sclerosis (pss). Methods. Nine patients with active progressive systemic sclerosis were treated with extracorporeal photochemotherapy on 2 successive days monthly. The duration of therapy ranged from 6 to 21 months. Results. A significant improvement was noted in the skin, musculoskeletal system, functional index, and symptoms including Raynaud's phenomenon, dyspnea, fatigue, dysphagia, and arthralgias, as well as improvement of cutaneous ulcers. Stabilization of the pulmonary function studies was also noted in the majority of patients over the course of therapy. No serious side effects were noted throughout the course of therapy in the 9 patients. Conclusions. The results suggest that photopheresis may be beneficial in selected early cases of progressive systemic sclerosis.     

SYSTEMIC SCLEROSIS ASSOCIATED WITH CARCINOMA OF THE LUNG

SUMMARY.— A case of systemic sclerosis associated with squamous cell carcinoma of the lung is presented. The relationship between malignant disease and systemic sclerosis is briefly discussed. Haemoptysis occurilng in a patient with systemic sclerosis may not be caused by the disease but be due to neoplastic change in the lung, and therefore merits full investigation.     

CARDIAC INVOLVEMENT IN PATIENTS OF SYSTEMIC SCLEROSIS

Background:

Systemic sclerosis is a multi-systemic autoimmune disorder. Cardiac involvement by the disease, although not included in the diagnostic criteria, may be seen either clinically, histologically or may be revealed by various investigative modalities.

Purpose:

To see the profile of cardiac involvement in patients of systemic sclerosis.

Materials and Methods:

Forty-seven patients of systemic sclerosis were included in the study. After taking a complete history and doing a detailed physical examination, the patients were submitted to electrocardiogram ECG (all leads), echocardiography and x-ray chest. Furst''s organ indices scoring system for cardiac involvement was followed.

Findings:

Forty-seven patients of systemic sclerosis were included in the study. Five females gave a history of palpitations. A loud pulmonic heart sound was heard in 1. Arrhythmias were observed in 5 patients. Significantly, echocardiography revealed valvular involvement in 5 patients. Left ventricular hypertrophy was seen in 2 patients.

Conclusions:

In our patients, cardiac involvement was rare. In contrast to other studies, valvular involvement was a prominent feature.

Limitations:

Complete evaluation for arrhythmias with 24-h Holter monitor was not used     

XERODERMA PIGMENTOSUM VARIANT: DNA PLOIDY ANALYSIS OF VARIOUS SKIN TUMORS AND NORMAL-APPEARING SKIN IN A PATIENT

Background. The patient with xeroderma pigmentosum provides an appropriate human model for the evaluation of tumor development and progression. Methods. We describe a patient with variant-type xeroderma pigmentosum who developed actinic keratoses, a squamous cell carcinoma, and its metastasis into a lymph node. DNA ploidy was measured and analyzed in cells of these tumors and the patient's sun-exposed as well as sun-shielded skin. Results. The sun-shielded skin showed a normal diploid dna distribution pattern, while the sun-exposed skin had an increased number of hyperdiploid cells. The actinic keratosis showed further increased hyperdiploid cells. The squamous cell carcinoma showed a large number of hyperdiploid cells and formed an aneuploid cell fraction. Histographically, the aneuploid cell fraction became more apparent and was revealed to be a major fraction in the metastatic squamous cell carcinoma. Conclusions. The changes in the DNA ploidy pattern well reflect the clinical and histologic characteristics of the respective skin conditions and likely provide the cellular basis for the sequential or stepwise carcinogenic process in the patient's xeroderma pigmentosum skin. Further studies are necessary to determine whether the present results explain the similar carcinogenic process in sun-damaged skin of the nonpredisposed general population.     

EFFICACY OF DEXAMETHASONE PULSE THERAPY IN PROGRESSIVE SYSTEMIC SCLEROSIS

Background. Systemic sclerosis is a disease of unknown etiology for which no specific treatment is effective. Pulse therapy with corticosteroids has been tried for various autoimmune disorders with minimal side effects. We undertook this study to determine the efficacy of dexamethasone pulse therapy in progressive systemic sclerosis (PSS). Methods. Five women with PSS between the ages 30 and 60 years, received 100 mg dexamethasone in 500 mL of 5% dextrose by slow intravenous infusion over 3 hours for 3 consecutive days, once a month. Results. All patients had symptomatic and clinical improvement. The vital capacity improved in three and posttreatment histopathologic regression was seen in two patients. Conclusions. Dexamethasone pulse therapy may provide an additional option for treating systemic sclerosis.     

INSULIN PRODUCTION AND GLUCOSE TOLERANCE IN PATIENTS WITH GRANULOMA ANNULARE

Summary.— Ten patients with granuloma annulare were studied for evidence of carbohydrate intolerance. By traditional criteria, 2 had an abnormal glucose tolerance test. Of the remaining 8,3 had an abnormal cortisone-glucose tolerance test. The findings indicate a higher incidence (50%) of glucose intolerance in these patients than is found in the average population. In the cortisone-glucose tolerance test, plasma insulin response to glucose loading was significantly lower in the population of 10 patients with granuloma annulare than in a normal control group. The remaining 4 granuloma annulare patients with normal glucose values in both the standard and cortisone-induced glucose tolerance tests were aiso evaluated by measuring plasma insulin response to glucose loading. These 4 patients, when evaluated as a group, demonstrated also a significantly lower insulin response to glucose than the control population. These results suggest a decreased pancreatic beta cell sensitivity to glucose stimulation in patients with granuloma annulare.     

SIGNIFICANCE OF IN VIVO-BOUND IMMUNOGLOBULINS AND COMPLEMENT IN THE SKIN OF SYSTEMIC LUPUS ERYTHEMATOSUS PATIENTS

A direct immunofluorescent test was used to demonstrate the in vivo deposits of immunoglobulin (Ig) and complement in the skin of 30 patients with systemic lupus erythematosus (SLE). These deposits were demonstrated in all skin lesions and in 70% of apparently normal skin of patients with lupus nephritis. On the contrary, they were seldom found in seemingly normal skin of patients without nephritis, though they could be seen in skin lesions. There was significant difference between these two groups and as well as an intimate relation of the deposits to clinical severity. The fixed Ig and/or complement could be found in a band arrangement in the basement membrane zone (BMZ) area, nuclear areas of epidermal and/or dermal cells or both BMZ area and nuclear areas.     

LICHEN PLANUS MIMICKING AND COEXISTING WITH PSORIASIS IN A BLACK PATIENT

We report a case of lichen planus coexisting with psoriasis in a black patient. As the lesions of lichen planus appeared similar in appearance to the psoriatic plaques its diagnosis was missed by several dermatologists. This may be due to the dark skin of this patient making the characteristic clinical differences between the two conditions less obvious. The coexistance of these two relatively common conditions is probably under reported in the literature. However, differentiation is important as treatment and prognosis of the two disorders differ.