Primary cutaneous follicular center cell lymphoma: immunophenotypical and immunogenotypical aspects.
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Biomedical subjects
Publications and source records attributed to R Caputo.
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Osteoporosis becomes a common pathologic feature in patients given a long-term corticosteroid treatment: actually, bone formation proves decreased, and bone resorption increased. Said reduced bone formation is ascribable to an inhibited osteoblastic function: on the contrary, increased bone resorption may be ascribed to a suppressed intestinal calcium absorption with a consequent secondary increase in the parathyroid hormone secretion. The present trial had the purpose of assessing the BMC (Bone Mineral Content) in 10 patients with bullous diseases, such as pemphigus and pemphigoid, in the course of treatment with corticosteroid agents and nasal spray salmon calcitonin (sCT). The patients, 3 males and 7 females, mean age 62.9 years (min 33, max 93) were given intranasal sCT at the dosage regimen of 200 IU/day/6 months. No patients had previously received corticosteroid treatment. Six patients were given betamethasone, and 4 methylprednisolone, compared thereafter with hydrocortisone; the minimum dose was 70 mg and the maximum dose 400 mg. To evaluate the BMC, the patients were subjected to a single photon absorption densitometry (SPA). Distal radius determinations were reasonably related with the axial skeleton BMC. Determinations were carried out at the start of the trial, at the 3rd month as well as the end of treatment. At the start of the trial, the mean BMC was 0.765 (+/- SE 0.056) calculated at the radial distal end; at the 6th month of treatment the mean BMC resulted to be 0.847 (+/- SE 0.059). Actually, as reported by various investigators, BMC may attain an over 10% loss in the course of a corticosteroid treatment, an evidence that is not encountered in the case of an intranasal sCT administration.(ABSTRACT TRUNCATED AT 250 WORDS)
Hyperkeratosis of the follicular channel is the most common finding in acne skin. The hyperkeratosis may represent an altercated keratinization process or the consequence of the abnormal sebum production and excretion. Several review demonstrated the anti-comedo activity of azelaic acid in acneic skin. This action may be due either to an anti-hormonal effect or to a change in keratin production. The aim of this work is to investigate the anti-comedo activity of 20% azelaic acid cream topically applied in a group of teen-agers affected by acne. A horny layer biopsy with cyanoacrylate glue was performed before and after four months of treatment with azelaic acid cream in ten acne patients. About 1 cm square horny layer biopsy was metallized and observed at the scanning electron microscopy to count the number of comedos. With this method we detected a reduction of about 26% of the comedos after four months of azelaic acid treatment. The result we obtained is in accordance with previous works about the anti-comedo activity of azelaic acid.
A total of 89 patients (mean age 42 years) with clinical signs of photo-ageing were evaluated in an open study to determine the clinical efficacy and tolerance of topical tretinoin therapy. Each evening for 6 months about 1 cm of cream containing tretinoin was applied to the face. Patient compliance and tolerance were maximized by gradually increasing the tretinoin concentration in the cream over the treatment period: 0.01% tretinoin for 1 month, then 0.025% for 1 month and finally 0.05% for 4 months. By the end of the 6 months' treatment period, 95.8% of patients showed improvement in the clinical symptoms of photo-ageing (coarse and fine wrinkling, skin thinning, mottled hyperpigmentation and laxity), except for xerosis. During treatment, 17 (19.1%) patients withdrew from the study, in five (5.6%) this was due to treatment-related side-effects. Patient compliance was rated as excellent in 47.0% of cases, good in 48.5% and fair in 4.5%; tolerability was excellent in 51.4% of cases, good in 44.4% and fair in 4.2%. Improvements were confirmed by scanning electron microscopy, computerized profilometry and computerized image analysis of skin replicas of the same areas taken before and after treatment, with a reduction in width and depth of wrinkles, improved skin texture and increased follicle density.
Due to its particular physiology, baby skin is highly sensitive towards cleansing agents especially during the child's first period of life. Dermatologists therefore suggest cleansing rules and recommend the use of suitable products to avoid skin defects by cleansing agents.
Fourteen cases of primary cutaneous B-cell lymphomas were investigated at the immunohistochemical and molecular level to further characterize this newly defined entity. Neoplastic cells from all cases, phenotyped with a panel of monoclonal antibodies, were positive for HLA-DR, for the B-cell markers CD19, CD22, but not CD23 (except one case), and negative for the T-cell marker CD2. Monoclonal immunoglobulin light chains were demonstrated in six cases. The reactivity with the Ki-67 monoclonal antibody indicated that the neoplastic cells are proliferating. In five biopsies the presence of dendritic cells infiltrating the neoplastic areas was revealed using the monoclonal antibody Kim4b. By Southern blot analysis, clonal rearrangement of the immunoglobulin heavy chain gene (involving one or both alleles) was shown in 12 of 14 cases and of the light chain genes in 13 cases. The bcl-2 oncogene, normally involved in nodal follicular lymphomas, was in germ-line configuration. The c-myc and the beta and gamma chain genes of the T-cell receptor were also in the germ-line configuration. None of the cases presented Epstein-Barr virus sequences. These data indicate that primary cutaneous lymphomas of B-cell origin share morphological and phenotypic similarities with the nodal B-cell lymphomas of follicular histotype, are proliferating, and express in 45% of cases clear monoclonal immunoglobulin light chain; the molecular analysis confirms the B-cell derivation and the monoclonal nature of this neoplasia; it also shows that neither bcl-2 nor c-myc oncogenes are involved and that no inappropriate rearrangements of the T-cell receptor genes are found in this lymphoma.
An 11-year-old girl affected by keratosis punctata palmaris et plantaris, histologically showing focal acantholytic dyskeratosis, is described. This case demonstrates that keratosis punctata palmaris et plantaris may represent a new clinical expression of persistent multiple focal acantholytic dyskeratosis.
Preliminary clinical studies (2) proved the effectiveness of topical azelaic acid (AZA) in the treatment of acne vulgaris. During the period of 1982-86 we carried out two studies with 20% AZA cream in patients with acne to determine its clinical indications and therapeutic schedules. The first, open study was of 100 unselected patients of either sex, while a second group of 30 patients formed a part of a larger (309 patients) multicentre single-blind comparison of topical AZA vs. 5% benzoylperoxide (BPO) gel for the treatment of papulopustular acne. The rates of improvement obtained indicate that topical AZA can be considered an effective therapy chiefly for papulo-pustular acne, with a very good local tolerance.
In a series of investigation using 20% azelaic acid as a therapy for acne, it was found that the treatment, compared with most common therapies (benzoylperoxide, oral tetracycline) significantly reduced inflamed lesions in papulo-pustular acne. The rates of improvement obtained indicate that topical azelaic acid treatment can be considered an effective therapy for papulo-pustular acne and it compares well with other agents. Azelaic acid cream shows a progressive and significant beneficial effect and its action is more pronounced in long-term treatment.
A unique case of Lipodystrophia centrifugalis sacralis infantilis in a caucasian is reported. This case fulfils all the clinical requirements of the centrifugalis lipodystrophy described in Oriental children by Imamura et al. (1) and usually localized on the abdomen. The 15-year follow-up of this case clearly demonstrates the tendency toward spontaneous remission of the disease after puberty.
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Response to treatment with daily intramuscularly administered crude calf thymic extract (Suppressin) in 11 patients with Langerhan's histiocytosis (L.H.) is reported. In ten patients, T-lymphocytic subsets were studied before starting immunotherapy: OKT3 positive and OKT4 positive cells were reduced in four patients; OKT8 positive cells were reduced in two patients; three patients were normal. After immunotherapy, one patient entered complete remission, four patients had stationary disease, and six had marked clinical progression. Subsequently eight patients underwent conventional chemotherapy, and only three entered complete remission. This study has demonstrated the heterogeneity of immunological patterns in L.H. and justifies the necessity for investigations on the immunoregulatory mechanism of L.H.
To clarify the nature of reticulohistiocytoma of the dorsum, 19 cases, including three of the seven original cases described by Crosti, were evaluated clinically, histologically, and immunologically. In seven cases gene rearrangement analysis was also performed. Results indicate that reticulohistiocytoma of the dorsum must be considered a primary cutaneous B cell lymphoma of follicular center cell origin. This localized skin disease has a very slowly progressive course, with many patients showing no systemic involvement even after prolonged follow-up.
After an accident in a chemical plant in Seveso, Italy, on July 10, 1976, 2,3,7,8-tetrachlorodibenzo-p-dioxin (TCDD) spread over a populated area. The event was exceptional because children were also affected and because the contamination took place not only through direct exposure but also through inhalation and the ingestion of contaminated foods, especially fruits and vegetables. This paper illustrates the early dermatologic lesions, the late acneic (chloracne) lesions, and their evolution during a 10-year period. Peculiar cutaneous findings, histologic data, and a comparison with previously reported similar accidents are also included.
Reticulohistiocytosis, a rare disorder occurring almost exclusively in adults, was seen in "pure" diffuse cutaneous form in an 8-year-old boy who had tuberous sclerosis since birth. The clinical features consisted of many papulonodular lesions, located mainly on the trunk and to a lesser extent on the head and limbs. Histologic findings were distinctive. Ultrastructural examination revealed the presence of pleomorphic cytoplasmic inclusions in almost all of the giant histiocytic cells of the infiltrate.
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Dermochondrocorneal dystrophy (François' syndrome) is an extremely rare disorder characterized by firm, nodular lesions involving the hands and the face; osteochondrodystrophy of the peripheral extremital bones, resulting in limitation of movement; and corneal dystrophy marked by white or brownish opacities. A nonfamilial case of dermochondrocorneal dystrophy was studied in a 45-year-old woman who had severe involvement of the gingival and palatal mucous membranes.