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Biomedical subjects

A Rebora

Publications and source records attributed to A Rebora.

At least 37 records · Page 2Linked to original sources

Scleroderma subsets are best detected by the simultaneous analysis of the autoantibody profile using commercial ELISA.

OBJECTIVE: Antinuclear antibodies are often present in patients with systemic sclerosis but do not provide useful prognostic information if studied individually. Although an autoantibody profile should be studied, this has never been done in a large series of patients. METHODS: Anticentromere, anti-topoisomerase-I, anti-extractable-nuclear-antigen, antihistone and anticardiolipin antibodies were studied by means of enzyme immunosorbent assay in 90 systemic sclerosis patients. RESULTS: We confirmed that anticentromere antibodies characterize limited forms of the disease with less frequent visceral involvement while anti-topoisomerase-I antibodies characterize diffuse forms with severe gastrointestinal, heart and lung involvement. Antihistone antibodies alone or associated with anticentromere antibodies characterize a disease subset with more frequent visceral involvement and a probably poor outcome. Patients with anticardiolipin antibodies, instead, did not display severer heart or vascular involvement. CONCLUSIONS: Systemic sclerosis patients are often found to have antinuclear antibodies. In addition to the well-known disease subsets identified by anti-topoisomerase-I and anticentromere antibodies, another one can be established, characterized by antihistone antibodies often associated with anticentromere antibodies. Patients displaying this profile have high prevalence of lung, kidney and heart involvement.

Adult↗

Perception of baldness and hair density.

BACKGROUND: Androgenetic alopecia needs to be scored precisely. OBJECTIVE: A possible measure is the ratio between the hair density in the parietal area and that in the occipital area which, being not affected by baldness, supposedly has a 'normal' density. METHODS: On the vertex and just below the occipital protuberance of 109 men, two 1-cm(2) areas were identified. In both areas, hairs were clipped short and photographed by a videomicroscope. Hairs were then counted within a 30-mm(2)-wide central square section. RESULTS: In the occipital area, the average count was 127/cm(2), without differences among the Hamilton/Norwood classes. In the parietal area, the average density significantly diminished from 138 to 47/cm(2). A main difference was found between classes 1-3 vertex and classes 4-6. CONCLUSIONS: The parietal/occipital ratio decreased significantly only when baldness was clinically manifest. The parietal/occipital ratio cannot be a better measure of baldness severity than the rough Hamilton/Norwood scale. The perception of early baldness does not depend on the diminished hair density, but also on the progressive thinning of the hair shafts.

Adolescent↗

Fosinopril as a possible pemphigus-inducing drug.

Fosinopril has recently been added to the angiotensin-converting enzyme inhibitors inducing pemphigus. The observation of a patient in whom pemphigus vulgaris (PV) worsened after taking fosinopril prompted us to study an experimental way to assess its responsibility. Slices of normal human skin (NHS) were simultaneously incubated for 2, 6, 12 and 24 h at 4 degrees C with progressively diluted fosinopril and captopril solutions and used as indirect immunofluorescence (IIF) substrates for 2 sera containing anti-desmoglein-3 (anti-Dsg3) antibodies at a dilution of 1/160. With captopril, IIF was negative, irrespective of dilution and time of incubation. Only at 1/40,000 dilution was IIF positive. With fosinopril, IIF was negative for the 2- and 6-hour-long incubations but turned positive after 12 h and so remained with all other solutions and incubation times. IIF negativity with captopril suggests that anti-Dsg3 antibodies contained in the PV sera were unable to find molecules in NHS to bind to. Captopril would therefore induce acantholysis by blocking the adhesion molecules. With fosinopril, instead, a partial block of the adhesion molecules was seen only with the very concentrated solution, unlikely to occur in vivo. Fosinopril, therefore, is probably unable to block the adhesion molecules in vivo. Our method might be used to verify the acantholytic properties of a drug.

Acantholysis↗

Cutaneous mucinosis of infancy: is it a real entity or the paediatric form of lichen myxoedematosus (papular mucinosis)?

We describe a girl presenting with a childhood dermal mucinosis in which we had the unique opportunity to find all the transitional histological features of lichen myxoedematosus (papular mucinosis), from its early focal mucin deposition in the reticular dermis to its late findings of interstitial mucin deposition, dermal fibrosis and fibroblast proliferation. Her father reported having had similar lesions when he was a child, which completely disappeared during adolescence. This case, and a re-evaluation of the literature, suggests that cases of cutaneous mucinosis of infancy that are not hamartomatous conditions such as mucinous naevi are in fact the infantile presentation of lichen myxoedematosus (papular mucinosis) and, in addition to other cases in the literature, suggests a genetic and familial factor in lichen myxoedematosus (papular mucinosis).

Child↗

Updated classification of papular mucinosis, lichen myxedematosus, and scleromyxedema.

Lichen myxedematosus (LM) is an idiopathic cutaneous mucinosis; its classification dates back to 1953, when Montgomery and Underwood distinguished 4 types of LM: a generalized lichenoid eruption, later called scleromyxedema, a discrete papular form, a localized or generalized lichenoid plaque form, and an urticarial plaque form. In the literature, the terms LM, papular mucinosis, and scleromyxedema have been often used indiscriminately as synonyms, but most reported cases of LM or papular mucinosis without indication of the subtype appear in fact to be cases of scleromyxedema. On the basis of personal experience, the anatomoclinical manifestations of published cases of LM, papular mucinosis, and scleromyxedema are reviewed to distinguish clearly between a generalized form with systemic, even lethal, manifestations and a localized form, which does not run a disabling course. LM includes two clinicopathologic subsets: a generalized papular and sclerodermoid form (also called scleromyxedema) and a localized papular form. Diagnosis of scleromyxedema should fulfill the following criteria: (1) generalized papular and sclerodermoid eruption; (2) mucin deposition, fibroblast proliferation, and fibrosis; (3) monoclonal gammopathy; and (4) the absence of thyroid disease. The criteria for localized LM are as follows: (1) papular or nodular/plaque eruption; (2) mucin deposition with variable fibroblast proliferation; and (3) the absence of both monoclonal gammopathy and thyroid disease. The localized form is subdivided into 5 subtypes: (1) a discrete papular form involving any site; (2) acral persistent papular mucinosis involving only the extensor surface of the hands and wrists; (3) self-healing papular mucinosis, of a juvenile and an adult type; (4) papular mucinosis of infancy, a pediatric variant of the discrete form or of acral persistent papular mucinosis; and (5) nodular form. A third group of atypical or intermediate forms, not meeting the criteria for either scleromyxedema or the localized form, includes cases of (1) scleromyxedema without monoclonal gammopathy, (2) localized forms with monoclonal gammopathy and/or systemic symptoms, (3) localized forms with mixed features of the 5 subtypes, and (4) not well-specified cases.

Diagnosis, Differential↗

Cutaneous mucinoses: microscopic criteria for diagnosis.

The clinical aspects and the histologic features of cutaneous mucinoses have been reviewed and their classification updated. Cutaneous mucinoses are divided into distinctive (primary) cutaneous mucinoses in which the mucin deposit is the main histologic feature resulting in clinically distinctive lesions, and disorders associated with histologic mucin deposition as an additional finding (secondary mucinoses). The former are further divided into degenerative-inflammatory mucinoses, which may be either dermal or follicular, and into neoplastic-hamartomatous mucinoses. Histopathologic diagnosis is particularly difficult for dermal mucinoses and requires clinicopathologic correlation. Three histologic clues, namely the pattern of mucin distribution (diffuse or focal), the level of mucin deposit in the dermis and some additional findings may help diagnosis. Follicular mucinoses have the easiest pattern to recognize histologically, but the distinction between Pinkus' follicular mucinosis and follicular mucinosis with mycosis fungoides is very difficult.Lastly, neoplastic-hamartomatous cutaneous mucinoses include mucinous nevus, a benign hamartoma, and myxoma, which is a benign tumor to be differentiated from reactive cutaneous focal mucinosis.

Cytodiagnosis↗

Topical metronidazole does not abate UVB-induced erythema.

BACKGROUND: The capacity of topical metronidazole to reduce erythema severity scores in rosacea has been attributed to its anti-inflammatory activity. METHODS: Nine healthy subjects were irradiated on three 1-cm(2) areas on their volar forearm with 2 MEDs of UVB. Twenty-four hours later, one of the irradiated areas was treated daily with metronidazole 1% vanishing cream, one with the vehicle only and the third was left untreated (control). The intensity of erythema was measured, at 24, 48, 72 and 96 h after irradiation, by a Chromameter CR200 (Minolta, Japan) and expressed in the L*a*b* system. The a* parameter was taken as the measure of redness. RESULTS: Overall, redness diminished over time in a linear way, but treatments did not influence it significantly. CONCLUSION: In our vehicle, metronidazole 1% cream failed to abate the UVB-induced erythema. An activity other than the anti-inflammatory one should be suggested to explain the topical metronidazole efficacy in rosacea.

Administration, Cutaneous↗

Diagnosing Pemphigus foliaceus: a retrospective analysis of clinical, histological and immunological criteria.

BACKGROUND: Clinical, histological and immunological criteria distinguish pemphigus foliaceus (PF) from pemphigus vulgaris (PV), but whether and how often they are concordant in the same patient is unknown. METHODS: Seven clinical records were selected from two hospital settings for having a diagnosis of PF and the initial serum and histopathological specimens still available. Controls were 8 PV records selected in the same way. Histopathological slides were re-evaluated. Stored sera were studied by indirect immunofluorescence (IIF), Western blot and ELISA. RESULTS: Acantholysis was superficial in all PF patients and deep in all PV patients. Mucosal lesions were not exclusive of PV. IIF was positive in 43% of PF patients. Western blot revealed desmoglein 1 in 86% of PF patients and in 25% of PV. ELISA revealed anti-desmoglein-1 antibodies in up to 71% of PF and in 62% of PV patients, in 1 failing to detect anti-desmoglein-3 antibodies. CONCLUSIONS: Histopathology remains the most reliable criterion for diagnosing PF. Western blot and ELISA, especially in combination, may be only of confirmatory value.

Acantholysis↗

Baldness and coronary artery disease: the dermatologic point of view of a controversial issue.

OBJECTIVE: Several articles, most of them written by nondermatologists, have stressed that bald men have a higher risk for coronary artery disease than men who are not bald. This study was performed to evaluate the validity of such conclusions from a dermatologic point of view. DESIGN: A review of the 24 articles in literature from 1954 to 1999 as provided by MEDLINE and a previous review. RESULTS: Five articles contained simple comments; 1 was a review of the previous literature; and 3 dealt only with the lipid profile. The remaining 15 articles dealt with coronary artery disease and baldness, and 9 of these concluded that there is a relationship between the 2 conditions, especially in younger subjects with severe early-onset androgenetic alopecia. CONCLUSIONS: Baldness did not coincide with androgenetic alopecia in some of the articles examined, which makes it difficult to settle the issue. Subjects who develop baldness before their 30s may have a higher risk for coronary artery disease than other men, and they may be individuals with early-onset androgenetic alopecia who also present with particularly elevated dihydrotestosterone-testosterone ratios. The baldness theory should be included as a secondary hypothesis in large epidemiological studies of coronary artery disease. Such studies should include dermatologic expertise for accurate, cost-effective evaluation of baldness.

Adult↗

Bullous pemphigoid in Liguria: a 2-year survey.

BACKGROUND: The epidemiology of bullous pemphigoid (BP) is not clear because of the heterogeneity of the disease, and its possible association with internal malignancies has been under debate for many years. We report the findings of a 2-year study on incident BP cases in the Liguria region of Italy. SUBJECTS AND METHODS: Thirty-two patients with BP were collected over the 2-year period. Diagnosis was made based on clinical findings and confirmed by histology, direct immunofluorescence (DIF) and indirect immunofluorescence (IIF) with salt-split skin and monkey oesophagus, and immunoblotting (IB). All patients were thoroughly investigated for possible malignancies and all were followed up for 6 months to monitor the response to treatment. RESULTS: DIF showed linear deposits at the dermoepidermal junction in all but one patient. IIF gave positive findings for 15 sera tested with monkey oesophagus and 20 tested with salt-split skin. IB gave positive findings in 19 cases. There was a malignancy in six cases, but no clinical or immunological features that could be considered to predict this occurrence. CONCLUSIONS: The findings of this study are in accordance with most of the data found in the literature, including the fact that IgG serum levels did not predict the course of the disease. Contrary to previous indications, IgE levels were not indicative of disease course either. Mucosal lesions, erythema multiform-like lesions, negative IIF findings and antibodies to AgPB2 were not a prediction for the development of malignancy.

Aged↗

Measuring the activity of the disease in patients with cutaneous lupus erythematosus.

The Systemic Lupus Activity Measure (SLAM) is a system proposed by rheumatologists to measure disease activity in their patients with systemic lupus erythematosus (LE). It involves scoring a group of clinical symptoms and laboratory findings, the maximum possible score being 84. In systemic LE, the mid-point is between 9 and 12. We applied SLAM to 176 patients with cutaneous LE. Ninety-seven had localized discoid LE (L-DLE), 59 had disseminated discoid LE (D-DLE) and 20 had subacute cutaneous LE (SCLE). Eighty-five patients had low activity disease (0-4 points), 72 mildly active disease (5-9 points), 15 moderately active disease (10-14 points) and only four had very active disease (>/= 15 points). The most frequent lesions in patients who scored more than 10 points were photosensitivity, cicatricial alopecia, Raynaud's phenomenon and oral ulcers. Fifty patients were followed up for more than 5 years (mean follow-up 9 years). Nine of these had an increased SLAM score. Seven had L-DLE, one D-DLE and one SCLE. Seven of the 50 patients had photosensitivity, five cicatricial alopecia, five non-cicatricial alopecia, two Raynaud's phenomenon and two oral ulcers. Three patients who started with L-DLE evolved to D-DLE. The SLAM system is useful in the monitoring of disease activity in patients with cutaneous LE. Over time, even L-DLE patients may develop active disease. Photosensitivity, alopecia, oral ulcers and Raynaud's phenomenon seem to herald a worse prognosis.

Acute Disease↗