Marked hyperandrogenemia and acne associated with polycystic ovaries in Greek women with polycystic ovary syndrome.

PCOS represents the commonest endocrinopathy among women of reproductive age. We conducted this study to evaluate the association between polycystic ovaries and clinical and biochemical features of the syndrome. TVS was performed in 74 women with the clinical diagnosis of PCOS. The findings were compared to biochemical, hormonal and clinical features of the syndrome. Statistical analysis revealed a significantly higher prevalence of acne, LH/FSH ratios and testosterone levels in women with PCO compared to those with normal ovarian morphology. In the subgroup analysis, total ovarian volume correlated significantly with hirsutism scores. Our study revealed a great prevalence of polycystic ovaries in Greek women with PCOS, and emphasizes the significance of transvaginal ultrasound in establishment of the diagnosis of the syndrome. The presence of PCO may not be clinically important when present alone without clinical manifestations but reflects the underlying hyperandrogenemia in PCOS women, representing a useful tool in the management of these patients.

Human embryonal tissues of all three germ layers can express the CD30 antigen. An immunohistochemical study of 30 fetuses coming after therapeutic abortions from week 8th to week 16th of gestation.

Originally, expression of the CD30 antigen was shown to be typical of the tumor cells of Hodgkin disease and of anaplastic large cell lymphomas. In reactive lymphoid tissue, CD30 is expressed only in a small population of activated lymphoid blasts. Since then, several reports have been published describing CD30 expression in non lymphoid tissues and neoplasms, such as embryonal carcinomas, seminomas, cultivated macrophages, histiocytic neoplastic cells, deciduals cells, and mesothelioma cells. In order to gain insight into the functions of CD30, given that it can mediate signals for cell proliferation and apoptosis, we studied the distribution of the antigen in different fetal archival paraffin-embedded tissues from week 8th to 16th of gestation. We investigated the immunohistochemical expression of CD30 in 30 paraffin-embedded tissue samples representing all three germ layers, using the monoclonal antibody Ber-H2 CD30 is expressed early in human fetal development (8th-10th week) in a wide variety of tissues, with the exception of the skin and thymus in which it is expressed later on. This is consistent with the observation that these organs are not fully differentiated before 10th and 13th week, respectively. No expression was observed in the cardiovascular and respiratory systems. The finding of CD30 expression in the terminal period of organogenesis, period, which is highly hormone related, implies that the antigen has an important role in cell development, maturation, and pathway to terminal differentiation in almost all fetal tissues and structures.

Oral administration of cyclosporin A in patients with severe alopecia areata.

Alopecia areata is a chronic, nonscarring hair loss condition with an unpredictable course that may cause emotional stress in affected patients. Regarding its pathogenesis, the most accepted theory is that alopecia areata is a T-cell-mediated autoimmune condition that is most likely to occur in genetically predisposed individuals. Cyclosporin A is an immunosuppressive agent that has provided new approaches in the treatment of autoimmune diseases. Hypertrichosis, one of the common side effects of orally administered cyclosporin A, encouraged a number of investigators to use the drug in the treatment of alopecia areata, but the reports on this subject have been controversial. We present a small series of patients with severe alopecia areata treated systemically with cyclosporin A at a dose of 3-5 mg/kg for 6 months as well as their 3-month follow-up after cessation of the drug.

Successful treatment of genital and facial psoriasis with tacrolimus ointment 0.1%.

Tacrolimus is an immunomodulatory agent that inhibits the activation and maturation of T-cells and blocks transcriptional activation of several cytokine genes. It also interferes with the function of Langerhans cells, basophil cells and mast cells. Recent studies have demonstrated the efficacy of topical tacrolimus in inflammatory skin disorders. Our objective was to assess the efficacy of topical treatment with tacrolimus ointment 0.1% in patients with psoriasis on the anogenital region and the face. Included in the study were 10 patients with a long-standing history of genital and facial psoriasis, partially controlled with periodic use of topical corticosteroids. Tacrolimus ointment 0.1% was applied twice daily for 10 days. The patients were followed-up every 3 weeks for a total period of 12 weeks. The severity of psoriasis was evaluated in all patients at baseline (day 0) and at the end of weeks 3, 6, 9 and 12. Clinical severity of erythema, scaling, infiltration and lesional extent were graded using a 0-3 scale indicating none, mild, moderate and severe expression, at baseline and at follow-ups. An overall severity score of 0 (clear), 1-4 (mild), 5-8 (moderate) or 9-12 (severe) was then assigned to each patient by adding the scores for the above parameters. On each visit, every patient was evaluated clinically. The decision to reapply the drug was determined by the clinical response of each patient at each visit. At the end of the study, patients also assessed efficacy, safety and tolerance after topical application of tacrolimus ointment using a 0-5 scale for each parameter: A marked improvement was noticed in all patients at the end of the first week without drug-related adverse effects. There were 15 recurrences during the 12-week period in all patients. In conclusion, tacrolimus ointment 0.1% seems to represent a safe new option for the treatment of genital and facial psoriasis. Further studies are probably needed to specify the therapeutic dosage and maintenance therapy

Ber-H2 (CD30) immunohistochemical staining of human fetal tissues.

OBJECTIVE: CD30 antigen has long been considered to be restricted to the tumour cells of Hodgkin's disease and of anaplastic large cell lymphoma as well as to T and B activated lymphocytes. It is now apparent that the range of normal and neoplastic cells, which may express CD30 antigen, is much wider than was at first thought. In order to gain insight into the physiological function of CD30 antigen, we studied the distribution of its expression in the tissues of fetuses from week 8th to week 16th. MATERIALS AND METHODS: We investigated the immunohistochemical expression of CD30 antigen in paraffin-embedded tissue samples representing all systems from 30 fetuses after therapeutic abortion at 8th to 10th and 12th to 16th week of gestation, respectively, using the monoclonal antibody Ber-H2. RESULTS: Our results demonstrated that CD30 is expressed early in human fetal development (8th to 10th week of gestation) in several fetal tissues derived from all three germ layers (gastrointestinal tract, special glands of the postpharyngeal foregut, urinary, musculoskeletal, reproductive, nervous, endocrine systems), with the exception of the skin and hematolymphoid system (thymus), in which the antigen is expressed later on (10th week onwards). Expression of CD30 was restricted to the hematolymphoid system in the 12-16 weeks of gestation. No expression of the marker was observed in the respiratory and cardiovascular systems during the entire period examined. CONCLUSIONS: CD30 antigen is of importance in cell development, and proliferation. It is also pathway-related to terminal differentiation in many fetal tissues and organs.

Long-term remission of recalcitrant tumour-stage mycosis fungoides following chemotherapy with pegylated liposomal doxorubicin.

Advanced stage mycosis fungoides (MF) generally has a poor prognosis, and currently there is no standard treatment available. Here we report the case of a young woman with recalcitrant tumour-stage MF (T3, stage IIb) whose disease was unresponsive to several therapeutic modalities, but who has showed sustained clinical response to pegylated liposomal doxorubucin. No severe infectious complications have been observed. The use of this drug in tumour-stage MF should be investigated further.

Mastocytosis with skin manifestations: current status.

AIM: To review our present knowledge about mastocyte origin, mastocytosis classification and management. METHODS: Literature review. RESULTS: Mastocytoses are chronic and recurrent disorders with symptoms which might either be limited only to the skin or to internal organs as well. The mastocytes, coming from bone marrow progenitor cells, migrate to tissues where they participate in inflammation and in cellular immunity as well as in the metabolism of connective and osseous tissues. Their proliferation causes the appearance of mastocytoses. The classification of the clinical manifestations of the mastocytoses into cutaneous, reactive (under the influence of the degranulator factors) and systemic disease, facilitates dialog among clinicians. Determination of prognosis and appropriate therapeutic regimens depend on individual features. CONCLUSIONS: Mastocytosis diagnosis is verified by histological study of skin lesion biopsy material. Management is symptomatic and unfortunately does not eradicate the disease.

[Cutaneous hyalinosis: 3 cases]. / Hyalinose cutanéomuqueuse: trois cas.

INTRODUCTION: The authors report three cases of "Hyalinosis cutis et mucosae"; the first and the second case concern two siblings children (brother and sister) while the third is reported on a 42-year-old woman. PRESENTATION OF CASES: The case history and the personal anamnesis, the morphology of the skin lesions, the symptoms indicating laryngeal mucous affection as well as the histopathological picture, are compatible with the "Urbach-Wiethe disease". Furthermore, in the third case, the electron microscopic findings confirm the diagnosis. DISCUSSION: The particularities of the present cases are discussed in connection with the corresponding bibliographical data.

[Acquired localized epidermolysis bullosa. A case with scalp involvement and immunoelectron microscopic study]. / Epidermolyse bulleuse acquise localisée. Un cas avec atteinte du cuir chevelu étudié en immunomicroscopie électronique.

The case of a 59-year old man who had a clinical Brunsting-Perry pemphigoid localized to the scalp is described. Direct immunoelectron microscopy demonstrated IgG and C3 immune deposits in the anchoring fibril zone, as in epidermolysis bullosa acquisita. No circulating antibodies were detected by Western immunoblotting on epidermal and dermal extracts. These findings show that Brunsting-Perry pemphigoid is not an immunological entity and that it widens the clinical spectrum of epidermolysis bullosa acquisita.