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Biomedical subjects

G Pravatà

Publications and source records attributed to G Pravatà.

17 recordsLinked to original sources

Increased SS bonds in chronic solar elastosis: a study with N-(7-dimethylamino-4-methyl-3-coumarinyl) maleimide (DACM) stain.

We have studied the distribution of SH groups and SS linkages in solar elastosis, in comparison with that in aged and juvenile sun-unexposed skin, using N-(7-dimethylamino-4-methyl-3-coumarinyl) maleimide (DACM) stain. In heavy solar elastosis we found increased fluorescence for SS bonds in the upper and middle reticular dermis in the elastotic masses; these were separated from the epidermis by a network of actinically-damaged, SS-positive elastic fibers, showing irregularly and variously interwoven oxytalan and elaunic fibers. These latter, unlike the elastotic masses, revealed also SH groups. In moderate and weak elastosis, where the thick irregular masses were absent, the fluorescence for SH and SS linkages was present in equal proportions in the altered elastic fibers; collagen bundles revealed only fluorescence for SS bonds. Aged and juvenile sun-unexposed skin showed the same proportions of SH and SS linkages in the changed elastic fibers. Collagen bundles of aged and juvenile skin showed fluorescence for SS bonds; SH groups were present on a few collagen fibers only in aged skin. Our results are consistent with opinion supporting an elastic origin of elastotic degeneration, which could be the consequence of a defect, due to chronic solar exposure, in one of the steps--degradation or synthesis--of metabolic turnover of elastic fibers, resulting in abnormal and excessive polypeptide chains extremely rich in disulfide bonds.

Adolescent

Multiple nevoid spiradenomas.

This report delineates a new clinical presentation of multiple benign spiradenoma (BS), a diffuse linear nevoid pattern. A 16-year-old girl, in otherwise good health, had many papulonodular lesions, varying in color from blue-gray to bright red, disposed linearly on the right half of her body, involving the periorbital region, cheek, lateral side of the neck, trunk, linea alba, right thigh, and leg. Only a few nodules were slightly painful. Lesions were reported to be present at birth; no family members had similar lesions, either in solitary or multiple form. Excisional biopsies of three lesions from the neck, abdomen, and leg, respectively, were performed; histologically, all three samples showed a picture of BS. In some areas, the tumor was directly connected to a pilosebaceous unit. Furthermore, a focal area with pilar differentiation at the periphery of the lobules was observed, which might suggest that BS can differentiate toward various cutaneous adnexal structures. Our patient probably is the first one reported with diffuse linear nevoid lesions of BS, present at birth and involving the right half of the body.

Adenoma, Sweat Gland

Granulomatous slack skin: report of a case associated with Hodgkin's disease and a review of the literature.

We report a case of granulomatous slack skin (GSS) associated with Hodgkin's disease, and review the literature on this entity. GSS, a variant of cutaneous T-cell lymphoma, clinically presents with erythematous patches in the flexures, which gradually transform into bulky, pendulous areas of skin. Histology shows an elastolytic granulomatous infiltrate, with atypical lymphoid cells, and occasional epidermotropism. As far as we are aware, 10 cases of GSS, including our patient, have been reported in detail. The male:female ratio of these cases is 9:1, and the age range 15-51 years. Five cases were associated with Hodgkin's disease, one with small lymphocytic lymphoma, and one developed cutaneous T-cell lymphoma. The axillae, abdomen and groins were the most frequently affected areas. No definitive management for GSS has been established. Surgery has been performed in localized forms, and systemic treatments have included corticosteroids, dapsone, chlorambucil, nitrogen mustard, and radiotherapy. Our patient was treated with chemotherapy for his Hodgkin's disease, and this resulted in complete remission of the lymphoma. Subsequent maintenance therapy with interferon-alpha produced good control of the cutaneous lesions.

Adolescent

Transfusion-associated graft-versus-host disease--report of two further cases with an immunohistochemical analysis.

Transfusion-associated graft-vs.-host disease (tGVHD) is a severe disease usually affecting immunocompromised hosts with haematological neoplasia. Two patients with acute leukaemia are reported, who developed fatal tGVHD after blood transfusions. Intercellular adhesion molecule 1 (ICAM-1), vascular cell adhesion molecule 1 (VCAM-1) and endothelial leucocyte adhesion molecule 1 (ELAM-1) expression and the CD4/CD8 ratio were assessed in lesional skin. ICAM-1 was strongly expressed on epidermal keratinocytes and endothelial cells (EC) and correlated with HLA-DR staining. VCAM-1 was strongly expressed on EC in the superficial dermal vessels. ELAM-1 stained weakly on EC in some of the superficial vessels. CD8+ lymphocytes showed prominent epidermotropism; the CD4/CD8 ratio was 0.8 in case 1 and 1.2 in case 2. Infiltrating cells were positive for CD3, CD11a, and CD18. Langerhans' cells were almost completely absent. The dermatologist must be aware of the importance of such a rare, unexpected and almost always fatal complication of blood transfusion, in order to make an early diagnosis. Irradiation of blood products is the only effective way to prevent tGVHD in all subjects at risk.

CD4-CD8 Ratio

Lack of antiandrogenic effects of topical bifonazole on sebaceous glands and hairs in the hamster flank organ.

The activity of topically applied bifonazole was evaluated in vivo in the three androgen-dependent structures of the hamster flank organ, i.e. the pigmented spot, sebaceous glands and hairs. Topical bifonazole in our experience did not demonstrate any morphological effect on sebaceous gland and hair even when applied in the dosage of 3 mg/cm2/day. On the basis of our morphometric results we can conclude that topically applied bifonazole does not interfere with cutaneous androgen metabolic transformations in the pilosebaceous unit of the flank organ.

Administration, Topical

Localized crusted scabies in the acquired immunodeficiency syndrome.

Crusted scabies (CrS) is an uncommon occurrence among patients with AIDS. Indeed to date only five cases have been described, all with widespread lesions. A case of localized CrS appearing as a yellowish and crusted plaque on the second right toe is reported in a woman with AIDS. Scraping off the verrucous surface, as well as punch-biopsy revealed many mites within the horny layer. The infestation is related to the cutaneous immune response and thus CrS should be considered an opportunistic infestation in AIDS. The importance of the early diagnosis of CrS in order to prevent disseminated lesions and involvement of other health-care workers is underlined.

AIDS-Related Opportunistic Infections

[Solar keratosis: a histochemical study on the distribution of SH groups and SS bonds].

The distribution of free SH groups and SS covalent linkages in hypertrophic, atrophic, acantholytic, bowenoid, pigmented solar keratoses (SK) and squamous cell carcinoma was evaluated. The sulphydryl groups were present in cytoplasms with a granular pattern and nucleoli mainly in atrophic, hypertrophic and bowenoid SK; the distribution of SS linkages appeared as a brilliant ovoid fluorescence localized in living layers, due to individually keratinized cells in SK. Similar results were found in squamous cell carcinoma. Our results agree with the opinion which considers SK as in situ carcinomas.

Carcinoma, Squamous Cell

Angiocheratoma corporis diffusum with normal enzyme activities.

A female case of angiokeratoma corporis diffusum without systemic involvement, with alpha-galactosidase A activity in the normal range, alpha-L-fucosidase in the lower levels of the normal range, and a few amount of urinary sialic acid is reported. Some problem about differential diagnosis with inherited disorders as Fabry's disease, fucosidosis, sialidosis is discussed. Although cases of angiokeratoma corporis diffusum without any underlying enzyme defect have been reported, we believe that angiokeratoma corporis diffusum is always related to known or unknown enzymatic defect, which activities could result in the normal range probably in relation to enzymatic polymorphism.

Adolescent

[Scleromyxedema without paraproteinemia].

The identification of an abnormal paraproteinaemia has been considered as main criterium for the diagnosis of scleromyxedema. We report a case of scleromyxedema in a 51-year old man, without any immunochemical evidence of paraprotein. Our case is a clinical support to the independence of this disease from gammopathy, as underlined by other experimental and clinical data. Thus demonstration of paraprotein should not be considered as a fundamental criterion for the diagnosis of scleromyxedema.

Blood Protein Electrophoresis

[Solar keratosis. Morphometric observations on atypia and polarity of keratinocytes of the basal stratum].

Nuclear morphometric parameters and polarity of basal keratinocytes have been evaluated by computerized image analysis in solar keratoses, comparing the results with normal sun-exposed skin and squamous cell carcinoma 1st Broders degree. The cellular index demonstrated higher levels in all keratoses. The morphometric nuclear parameters revealed major values in atrophic, bowenoid, acantholytic, hypertrophic and pigmented solar keratoses, respectively. The loss of polarity demonstrated higher levels in bowenoid, atrophic, hypertrophic, pigmented and acantholytic histotypes, respectively. Solar keratoses could be classified as in situ carcinomas rather than precancerous lesions.

Carcinoma, Squamous Cell

[Usefulness and limitations of the evaluation of sex-hormone-binding globulin in women with a female pattern of androgen-induced baldness].

Sex-hormone binding globulin (SHBG) and androgen serum levels have been evaluated in a homogeneous group of women with female pattern of androgenetic alopecia (AA), stage II, without any clinical or anamnestic evidence of acne, hirsutism, irregular menses. Results did not show any significant difference between patients and controls. Since SHBG levels are androgen-dependent, the discordant results of previous published series regarding women with AA could be related to the presence of a variable number of patients with clinical and/or anamnestic evidence of a "cryptic" hyperandrogenism in some series, and to their absence in others. Statistically significant low average SHBG values could have been sustained by a mild, heterogeneous and not significant androgen excess. In our opinion the genetically-determined response of the target organ to androgens seems to play the major pathogenetic role in AA, at least when the woman does not reveal any clinical or anamnestic evidence of cryptic or clear hyperandrogenism.

Adult

Ovulatory patterns in women with juvenile and late-onset/persistent acne vulgaris.

The ovulatory patterns in women with acne vulgaris were evaluated in order to understand their relationship with androgenic levels. Ovulation disturbances were found in 58.3% of patients with prevalence of anovulation in the juvenile acne and of luteal insufficiency in the late-onset/persistent acne. Significant negative correlation was found between T free and P in the late-onset/persistent group (r: -0.629; p = 0.016): This may be interpreted as a rather steady endocrine status in which the raised androgenic levels, probably due to peripheral conversion, are concomitant to absent or insufficient ovulations. In the younger patients both the androgen excess and the ovulation disturbances could be due to an abnormal or delayed maturation of the hypothalamus-pituitary ovarian axis. The evaluation of the ovarian function in women with acne vulgaris may be useful to detect ovulatory disturbances in view of a possible resolution of both the problems by specific endocrine management.

Acne Vulgaris

[Fabry's disease: kidney insufficiency in heterozygous patient].

We report a rare heterozygous status for Fabry's gene with severe kidney involvement and normal alpha-galactosidase A activity, together with the intrafamilial variations in the clinical expression of the disease. The random X inactivation hypothesis seems to explain such a variable expression of the alpha-galactosidase gene in our cases.

Adult