Acquired cutis laxa confined to the face.
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Biomedical subjects
Publications and source records attributed to A Parodi.
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Ninety-one patients with progressive systemic sclerosis have been examined both clinically and serologically in order to have a better prognostic insight. Three main serological profiles have been isolated. The patients with anticentromere antibodies (ACA) represented one third of the cases, developed skin sclerosis rather later and rarely exhibited ankyloses and ulcerations. The esophagus was commonly involved while the lung, heart and kidneys were not. ACA-positive patients were not identified with the CREST syndrome, as the latter disclosed other profiles with the same frequency. Patients with anti-Scl-70 antibody represented one fourth of the cases and had the fastest progression, developing sclerosis in less than 5 years after the onset of Raynaud's phenomenon. Ankyloses and lung fibrosis, as well as joint, heart and kidney involvement, were found in most of them. Patients with anti-SSA/Ro antibodies were uncommon, but corresponded to a severe subset, having a fast progression and a constant involvement of the lung. Probably due to the rougher definition of their serology, patients with antinuclear, antispeckle-patterned and anti-Ku antibodies or without any detectable antibody could be defined less easily and corresponded to an intermediate position between ACA- and anti-Scl-70-positive patients. Though it is probably premature to trust it completely, a serological classification may provide the prognostic clues clinical classifications cannot.
A patient with an unusual form of follicular impetigo, who later developed classical systemic lupus erythematosus (SLE) is described. When she had pyoderma, direct immunofluorescence and serology already suggested a connective tissue disease. Pyoderma could have been the presenting manifestation of SLE.
Two patients with erosive lichen planus and latent HBV infection who had circulating antibodies directed to nuclei of epithelial cells are described. The nature of such antigen has been investigated by indirect immunofluorescence, double immunodiffusion, counterimmunoelectrophoresis, enzyme-linked immunosorbent assay, enzyme treatments, and immunoblotting. The antigen cannot be identified as RNP, histone, soluble nuclear protein, nDNA, or ssDNA. It may be a DNA protein complex and preliminary immunoblotting data support the thesis that it may be a multimolecular complex.
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We describe 3 cases of papular and nodular mucinosis (PNM), a clinically distinctive cutaneous mucinosis associated with lupus erythematosus (LE), which has received little attention in the dermatologic literature. Histopathology shows deposits of mucin in the dermis without microscopic features of LE, while immunofluorescent studies disclose linear or granular deposits of IgG, IgM and C3 at the dermoepidermal junction. In about 80% of the 14 cases described in the literature, PMN has been associated with systemic LE with prevalent joint and kidney involvement. The possible prognostic significance of this singular dermatosis is discussed.
Overlap syndromes (OS) are complex clinical entities in which symptoms and serological profiles of diverse connective tissue diseases, mainly systemic lupus erythematosus (SLE), progressive systemic sclerosis (PSS), dermato/polymyositis and Sjögren syndrome, converge in a single patient. On the basis of the personal experience, the Authors try to classify OS from the serological point of view. Six different serological profiles have been recognized. Anti-U1RNP antibodies define the classical mixed connective tissue disease (MCTD) according to Sharp. These patients are probably most common and present with Raynaud's phenomenon and puffy hands which never reach sclerodactily. In Italian patients, the symptoms of lupus erythematosus are not as frequent as in USA patients, probably due to the prevalence of Negroes in the latter. The absence of renal involvement is, instead, common as in other series. The general trend of these patients is towards progressive systemic sclerosis and lung fibrosis. Anti-Ku antibodies define another group of patients with the same clinical symptoms as MCTD and good prognosis. Anti-Ki-SL antibodies, by contrast, characterize a less common group of patients in which the renal and the pulmonary involvement is frequent. Their prognosis is poor. Anti-Sm and anti Ro/SSA antibodies, which are more commonly found in SLE patients, may be detected also in some of the patients with OS who have a poor prognosis. Patients with anti-SSA, for example, have, as in PSS, an early onset and a rapid development of lung fibrosis. Finally, anti-La/SSB antibodies define patients who associate Sjögren syndrome to SLE or PSS.(ABSTRACT TRUNCATED AT 250 WORDS)
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Circumstantial evidence of phototoxicity of nifedipine is poor, being based only on uncontrolled clinical evidence. The authors have assessed nifedipine phototoxicity in vitro by photohemolysis and Candida albicans tests and found it measurable at 10 and 100 micrograms/ml. These dosages exceed the 0.1 microgram/ml blood levels that are usually attained in vivo.
The occurrence and persistence of DNA damage, as detected by the alkaline elution technique, have been studied in some tissues of both fetal and adult Sprague-Dawley rats (18th day of gestation) after administration of a single equimolar dose (0.5 mmol/kg) of ethyl methanesulfonate (EMS), N-ethyl-N-nitrosourea (ENU), N-nitrosodiethylamine (NDEA), and N-methyl-N-nitrosourea (MNU). EMS, ENU, and MNU, injected intravenously, produced a statistically significant increase of DNA elution rate, which is considered indicative of DNA fragmentation, in both maternal and fetal liver, kidney, and brain. NDEA, introduced by gastric gavage, induced DNA breaks in both liver and kidney of dams, but only in the liver of fetuses. The frequency of DNA lesions was found to vary with the four alkylating agents and in the three organs tested, to exhibit a different time course, and usually to be higher in maternal than in fetal tissues. Results provided by the concomitant determination of DNA binding levels demonstrated a satisfactory correlation with the amounts of DNA fragmentation. In contrast, the values of both these parameters did not show any positive correlation with the different susceptibility of the three organs to tumor induction. In conclusion, these findings suggest that when a compound is not available in radiolabeled form, measurement of DNA fragmentation may represent a useful alternative to the determination of DNA binding level in order to obtain information on the distribution of its reactive species in maternal and fetal tissues.
We describe a patient with discoid lupus erythematosus who developed annular lesions of the thigh and chilblainlike lesions of the fingers matching those described in the original reports of Rowell's syndrome. The patient also had circulating anti-Ro(SS-A) antibodies whose similarity to the anti-Sj-T antibodies found in the original Rowell's syndrome cases has been recently claimed. A review of the literature suggests that most of the cases of Rowell's syndrome described thus far in fact may be cases of coincidental association of lupus erythematosus and erythema multiforme.
Three patients are described with clinical features of connective tissue diseases, namely, dermatomyositis, progressive systemic scleroderma, and systemic lupus erythematosus. In two patients the symptoms of disease overlapped. The rare anti-Ku antibody was found in all of them. Anti-Ku antibody characterizes those patients, with polymyositis-scleroderma overlap syndromes who have a good prognosis. One of our patients, who also had severe anti-Sm-positive systemic lupus erythematosus, seems to be an exception.
A patient with photosensitivity is described who presented with an overlap syndrome combining features of systemic lupus erythematosus with a mild polymyositis and scleroderma and with symptoms of the sicca syndrome. In addition, he was anti-SSA/Ro positive and had a constellation of antibodies directed to ENA including the anti-SL-Ki antibody. The condition was fatal and he died from heart failure and respiratory insufficiency.