BACKGROUND: RS3PE (Remitting Seronegative Symmetrical Synovitis with Pitting Edema) syndrome is characterized by bilateral and symmetrical tenosynovitis of the distal extremities. It occurs with acute onset in older patients aged over 50 years. This heterogeneous entity can be isolated or can reveal various rheumatic diseases and neoplastic conditions. We report a case of RS3PE syndrome associated with unusually severe cutaneous necrotic and haemorrhagic lesions, and revealing malignant monoclonal IgM proliferation. CASE REPORT: A 62-year-old man was admitted for acute and symmetrical synovitis of both hands and forearms associated with fever and increased acute phase reactants. Severe necrotic and haemorrhagic edema developed simultaneously. Laboratory tests ruled out infectious disease and collagen vascular disorder. Clinical symptoms responded promptly to corticosteroids to reveal severe erosive arthropathy of both wrists. The monoclonal IgM proliferation discovered during the acute phase evolved into a malignant medullary plasmocytosis. After adequate treatment of this proliferation, no haematological, cutaneous or articular relapse occurred during the two-year follow-up period. DISCUSSION: This type of cutaneous symptoms has never been reported during the course of RS3PE syndrome. It may be supposed that the severity of the initial clinical picture was linked in this patient to the paraneoplastic nature of this. 相似文献
ABSTRACT: In a prospective study of 150 patients with Behcet's disease, significant associations were shown between CRP positivity and the presence of erythema nodosum (p < 0.02) and acute thrombophlebitis (p < 0.05) and between ESR and erythema nodosum (p < 0.01), acute thrombophlebitis (p < 0.001), and acute arthritis (p < 0.01). Mucocutaneous, ocular, or central nervous system activities did not show significant associations with these indices of inflammation. In 50 patients in whom quantitative CRP determinations were performed, clinical disease activity was accompanied by slight to moderate increases in CRP and ESR. 相似文献
A 64-year-old woman complained of severe infrapubic pain and pollakisuria with nausea, vomiting and diarrhea, but with normal urinalysis since 1987. The clinical diagnosis of interstitial cystitis (IC) was made, and cystectomy was performed in 1996. The bladder taken was markedly shrunken with a capacity of 50 ml, and showed bleeding on the mucosal surface. Histological findings of the bladder showed ulcer formation in the mucous membrane, and marked infiltration of mononuclear cells, edema and fibrosis in the submucosal tissue. She had noticed exudative erythema, swelling and sclerosis on the bilateral fingers and dorsal aspects of the hands since 1993, and Raynaud's phenomenon and morning stiffness of the fingers from November, 1998. Laboratory data showed positive anti-nuclear antibody (titer: 1: 2,560) and anti-U1 RNP antibody (titer: 69.5 by ELISA). A diagnosis of mixed connective tissue disease following IC was made. 相似文献
BACKGROUND: Phytophotodermatitis is a phototoxic reaction, occurring in skin exposed to sunlight after contact with plants containing furanocoumarins. Typical reactions are mild, showing erythema with post-inflammatory hyperpigmentation. A 6-year-old boy presented with marked, symmetric, painful erythema and edema of both hands that rapidly developed into dramatic bullae covering the entire dorsum of the hands. The history revealed that the hands had been bathed in lime juice for a prolonged period in the preparation of limeade. OBJECTIVE: This report documents an unusual bullous presentation of phytophotodermatitis resulting from contact with furanocoumarins in local limes. This study was conducted to identify and measure the inciting substances from the rind and pulp of the limes. METHODS: Psoralen, xanthotoxin, bergapten, and isopimpinellin content were measured by gas chromatography and high-pressure liquid chromatography RESULTS: The rind contained 6- to 182-fold concentrations of all furanocoumarins measured when compared with pulp. Bergapten was the most abundant substance in the rind. CONCLUSION: Hydration of the skin during the preparation of limeade combined with increased levels of bergapten in local limes to produce a dramatic bullous reaction. We encourage clinicians to consider the possibility of phytophotodermatitis in severe bullous skin reactions. 相似文献
Necrolytic acral erythema is a newly described entity characterized by sharply demarcated scaly plaques on the dorsum of the hands and feet. More than 30 patients have been reported since 1996, all of whom had anti-hepatitis C virus antibody. A 32-year-old Taiwanese woman had been diagnosed with and treated for systemic lupus erythematosus with lupus nephritis about 10 years earlier. Soon thereafter, she noted several well-demarcated keratotic plaques with erythematous borders on her feet, with sparing of the soles. Histopathology showed diffuse parakeratosis with a neutrophil infiltrate, hypogranulosis, pale upper keratinocytes, scattered and grouped dyskeratotic cells, psoriasiform hyperplasia and a mild lymphocytic infiltrate in the upper dermis. The diagnosis was made after three biopsies. The lesions regularly worsened just before and during menstruation, but patch and intradermal tests for progesterone and estrogen were negative. There was no evidence of either hepatitis B or hepatitis C infection. The lesions did not respond to treatment with zinc. The rash regressed spontaneously when corticosteroids were stopped and recurred when they were restarted, finally resolving completely after she was treated with high-dose pulse steroids for her lupus. 相似文献
Growth differentiation factor‐15 (GDF‐15), a member of the transforming growth factor‐β superfamily of cytokines, plays an important role in cell growth, signal transduction, and apoptosis regulation. The aim of this study was to evaluate serum GDF‐15 levels and their relationships with disease‐related variables in patients with Behçet's disease (BD). Forty‐six patients diagnosed with BD and 30 demographically matched healthy control subjects participated in the study. GDF‐15 levels were measured in blood samples from patients and controls. The Behçet's Disease Current Activity Form (BDCAF) was used to evaluate the disease activity of BD. There were no significant differences between the two groups in C‐reactive protein (CRP) level, mean erythrocyte sedimentation rate (ESR), age, body mass index, and mean GDF‐15 levels (P > 0.05). Serum GDF‐15 levels were positively correlated with findings for peripheral arthritis and CRP, and with BDCAF erythema nodosum, BDCAF arthralgia, and BDCAF arthritis scores. Patients with BD were divided into two groups according to the presence of peripheral arthritis; nine subjects (20%) were positive for peripheral arthritis. Serum ESR, CRP, white blood cell counts, and GDF‐15 levels were significantly higher in the group that was positive for peripheral arthritis (P < 0.05). GDF‐15 may play a role in the progression and pathway of Behçet's joint involvement and erythema nodosum that is independent of classic inflammatory response measures. 相似文献
A 32‐year‐old man presented with conspicuous acneiform pitting scars on the right nasolabial fold of 3 years’ duration ( Fig. 1 ). He stated that the scars were preceded by erythematous plaques. Physical examination also revealed perilesional erythematous infiltration and telangiectasia. Tracing back the history, there was no malar erythema, oral ulcer, or arthralgia. A skin biopsy showed irregular acanthosis, follicular plugging, vacuolar degeneration of the basal cell layer with marked melanin incontinence, and heavy periadnexal mononuclear cell infiltration ( Fig. 2a,b ). Direct immunofluorescence studies displayed continuous granular deposition of immunoglobulin G (IgG) and C3 along the dermo‐epidermal junction. The hemogram, antinuclear antibody (ANA) test, complement, and urinalysis were within normal limits. Based on the histopathologic findings and a positive lupus band test, a diagnosis of discoid lupus erythematosus (DLE) was made. Figure 1 Open in figure viewer PowerPoint An atrophic plaque composed of conspicuous acneiform pitting scars with surrounding erythema and telangiectasia on the right nasolabial fold 相似文献
A 67-year-old Japanese man had been treated occasionally for asthma by oral administration of prednisolone at a dose between 5 and 15 mg/day for 18 years. In December 1993, the patient had a severe asthma attack, during which he developed a pneumothorax, complicated by pneumonia. He was hospitalized immediately, and antibiotics and hydrocortisone were administered intravenously. The treatment was, however, ineffective and his condition deteriorated. In February 1994, he was treated with pulse therapy of 1 g/day of sodium hydrocortisone for 3 days. During the pulse therapy, erythema and red papules were noticed on his right dorsum manus. The erythema progressed with purpura, induration, and local fever. The papules became necrotic, giving rise to small ulcers with effusions (Fig. 1). The patient also developed a subcutaneous nodule on the proximal region of his forearm. Laboratory investigations revealed anemia (hemoglobin (Hb), 9.6 g/dL), leucocytosis (leukocyte count, 11,800/mm3), slight liver dysfunction (GOT 32KU, GPT 71KU, LDH 759UU), and hypoaluminemia (albumin (Alb), 2.7 mg/dL). A biopsy of the indurated erythema of the dorsum manus was performed. The histopathology of the biopsied tissue showed diffuse infiltration with inflammatory cells, edema, and necrosis in the mid-dermis and fat. The infiltrate consisted of lymphocytes, neutrophils, histiocytes, and occasional giant cells. Many mycelial form fungal elements were observed (Fig. 2a), and aggregates of hyphae were also found in the lower dermis (Fig. 2b). Similar findings were noted for the biopsied specimen of the forearm subcutaneous nodule. Biopsied material was inoculated on Sabouraud's dextrose agar, and floccose and smoky-gray colonies grew rapidly at room temperature for 7 days (Fig. 3a). Microscopic findings showed that the hyphae were broad, branching, and brown in color. Round or oval conidia were produced singly on short conidiophores from the sides of the hyphae, or terminally on long conidiophores (Fig. 3b). Scanning electron microscopy showed an oval conidium produced from the terminus of the conidiophore with annellation. Although we observed the cultured mycelium for several months, cleistothecia were not produced. From these findings, we identified the organism as Scedosporium apiospermum, the asexual stage of Pseudallescheria boydii. The organism was not isolated from sputum or blood cultures. The patient was given fluconazole intravenously at 400 mg/day. Although the treatment was stopped after 2 weeks, because of increasing serum transaminase, the erythema and subcutaneous nodule almost completely disappeared after 5 weeks. 相似文献
In this study, we examined peripheral insulin resistance in patients with Behçet's disease (BD) characterized by chronic inflammation and endothelial dysfunction. Fourteen patients with BD and 15 healthy controls were recruited to the study. Insulin resistance was investigated by the hyperinsulinaemic–euglycaemic glucose clamp technique. BD patients displayed an enhanced rate of insulin resistance compared to healthy controls (P = 0.014). The insulin sensitivity (M), measured as the glucose utilization rate under steady‐state conditions of euglycaemia, was significantly decreased (P = 0.001) in BD patients compared to the controls (4.09 ± 0.16 vs. 5.60 ± 0.27 mg/kg/min). The C‐reactive protein (CRP) level, but not the erythrocyte sedimentation rate (ESR), was significantly related to the presence of insulin resistance (CRP: rs = 0.589, P = 0.27; ESR: rs = 0444, P = 0112), whereas no relationship was found between the M‐value and ESR or CRP. We conclude that patients with BD exhibit peripheral insulin resistance; this could be explained as the diverse consequences of inflammation and endothelial dysfunction in BD. 相似文献
A 68-year-old white woman was referred to us by her rheumatologist for possible participation in a clinical study of photopheresis for scleroderma. In February 1993, she noticed edema of her distal phalanges, Raynaud's phenomenon in both hands, flu-like symptoms, fatigue, intermittent diarrhea, abdominal pain, tearing in both eyes, dyspnea on exertion, dysphagia, and odynophagia. Bilateral silicone-gel breast implants had been placed 12 years before; 2 months before her present evaluation, they were removed and found to be ruptured. Physical examination revealed edema, limited to the fingers and hands bilaterally, and slight induration of the skin on the dorsum of both hands and distal forearms. The remainder of the physical examination was normal. According to our study protocol, a skin biopsy specimen from the dorsum of the right hand was taken, but all other laboratory investigations were refused. Histopathologic examination revealed multiple clear spaces of varying sizes in the dermis and multinucleated macrophages containing small refractile particles, characteristic of silicone granuloma (Figs. 1 and 2); however, the specimen showed no evidence of scleroderma. X-ray energy dispersive analysis by scanning electron-microscopy confirmed the presence of elemental silicon in the small refractile particles. The patient did not receive any treatment after her diagnosis and shortly thereafter, she was lost to follow-up. 相似文献
An 86-year-old African-American man presented with tonic-clonic seizures. Intravenous phenytoin was urgently administered into the dorsum of the right hand. The patient developed a raised purple area of discoloration around the intravenous insertion site within 2 h and edema and vesiculobullous lesions of the distal forearm, hands, and fingers within 8 h. Microscopic sections from a biopsy at 12 h revealed epidermal necrosis, superficial ulceration, and a mild superficial and deep perivascular lymphoid infiltrate, associated with numerous thrombi of small vessels throughout the dermis. The findings were judged to be consistent with soft-tissue injury associated with intravenous administration of phenytoin, also termed purple glove syndrome. Purple glove syndrome, named for its distinctive purple discoloration and swelling of the hands in the distribution of a glove, is an uncommon complication of intravenous phenytoin administration through small dorsal veins of the hands. It is comprised by pain, discoloration, and edema in the vicinity of intravenous infusion of phenytoin through dorsal veins of the hand. The histopathologic features of fully developed lesions have been reported; however, early-stage findings have not been previously described, and the histogenesis of this lesion is controversial. The presence of thrombi in this early-stage lesion suggests that thrombosis plays a role in the initial pathogenesis of this condition. 相似文献
We present a case of Reiter syndrome (RS) associated with Terry nail in a 32-year-old male patient who also had subungual keratosis and onycholysis. The case had all the characteristic findings of complete RS including urethritis, conjunctivitis, arthritis and skin lesions, and based on these findings a clinical diagnosis was established. The patient complained of milky-white coloured leakage that was more frequent in the mornings, and dysuria. Urinary analysis revealed leucocytes and erythrocytes. Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) values were high. The case was considered as RS due to gonococcic urethritis. However, there was no growth in the culture. Self-report of the patient revealed that he had had continued symptoms and 9 months after the development of RS he developed Terry nail. To the best of our knowledge this is the first case of RS associated with Terry nail. 相似文献
Background Skin lesions are among the most common complications of contact with sulfur mustard. Objective This study was aimed to measure skin water content and transepidermal water loss (TEWL) in patients with a history of sulfur mustard contact. Methods Three hundred ten male participants were included in this study: 87 (28.1%) sulfur mustard–exposed patients with current skin lesions (group 1), 71 (22.9%) sulfur mustard–exposed patients without skin lesions (group 2), 78 (25.2%) patients with dermatitis (group 3) and 74 (23.8%) normal controls (group 4) The water content and TEWL of skin was measured at four different locations of the body: forehead, suprasternal, palm and dorsum of hand. Nonparametric statistical tests (Kruskal–Wallis) were used to compare the four groups, and P < 0.05 was considered statistically significant. Results The mean age of participants were 44.0 ± 6.7, 41.9 ± 5.9, 43.8 ± 9.3 and 44.8 ± 8.9 years in groups 1 to 4, respectively ( P = 0.146). Xerosis, post-lesional hyperpigmentation and lichenification were significantly more common in either sulfur mustard–exposed participants or non-exposed participants with dermatitis ( P < 0.05). Skin hydration was higher in subjects with sulfur mustard contact than in non-injured participants ( P < 0.05) in the dorsum and palm of hands and forehead. TEWL was significantly higher in participants only in suprasternal area and dorsum of hand. Conclusion Contact with sulfur mustard agent can alter biophysical properties of the skin-especially the function of stratum corneum as a barrier to water loss-several years after exposure.
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