[Efficacy of sequential corticotherapy in a case of painful acral erythema caused by high-dose 5-fluorouracil].
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Biomedical subjects
Publications and source records attributed to G Lorette.
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BACKGROUND AND DESIGN: The incidence and distribution of autoimmune subepidermal bullous diseases were estimated from prospective data (including immunoelectron microscopy) obtained from 100 cases during a mean period of 35 months in three university dermatologic centers in Amiens, Limoges, and Tours, France, that correspond to a cumulative reference population of 3.55 x 10(6). RESULTS: Using data from these regions, we found a mean annual incidence of autoimmune subepidermal bullous diseases to be 10.4 per million people and, therefore, estimated the overall number of new cases of these disorders in France to be about 590 cases per year. According to clinical and immunoelectron microscopic criteria, a precise diagnosis was established in 94 cases, distributed as follows: bullous pemphigoid, 69 cases; cicatricial pemphigoid, 12 cases; linear IgA dermatosis, five cases; herpes gestationis, four cases; epidermolysis bullosa acquisita, two cases; and vesiculobullous systemic lupus erythematosus, two cases. CONCLUSION: Our prospective study is the first assessing the incidence and distribution of autoimmune subepidermal bullous disorders that systematically included immunoelectron microscopic data. Our estimated incidence of bullous pemphigoid (seven new cases per million people per year) is large enough to establish bullous pemphigoid as the major autoimmune subepidermal bullous disease for the purpose of therapeutic trials. On the contrary, all other disorders, particularly epidermolysis bullosa acquisita (estimated annual incidence, 0.17 to 0.26 per million people), were very rare and reflect the paucity of patients available for short-term clinical studies in France.
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INTRODUCTION: Cutaneous polyarteritis nodosa is a subset of polyarteritis nodosa (PAN) characterized by a good prognosis because of the absence of severe visceral involvement. Dapsone, colchicin, non steroidal anti-inflammatory drugs are the first line treatments of cutaneous PAN. Corticosteroids are an alternative therapy. CASE-REPORT: A case of cutaneous PAN followed for 6 years is reported. Dapsone, colchicin, corticosteroid and cyclophosphamide have failed to control cutaneous lesions. Intravenous immunoglobulins 400 mg/kg/d five day monthly for 5 months was successful, but a cutaneous relapse occurred 3 months later which was another time controlled with intravenous immunoglobulins. DISCUSSION: Some cases of necrotizing vasculitis have been treated with intravenous immunoglobulins. This is the first reported case of cutaneous PAN treated with intravenous immunoglobulins. Because of the lack of controlled studies, the indications of intravenous immunoglobulins in vasculitis are restricted to those resistant to corticosteroids and immunosuppressive therapy.
We report the case of a 7-year-old boy with blue rubber bleb nevus syndrome and frequent digestive tract hemorrhage. Gastrointestinal hemangiomas were treated with laser therapy. Intermittent steroid therapy was administered from the age of 6 years. The results are compared to the relevant medical literature.
We have studied changes in the elasticity and viscosity of the skin in patients with lymphoedema, using a technique involving vertical extensibility by suction. We measured parameters which included immediate extensibility (Ue, which reflects the elastic properties of the skin), and delayed extensibility (Uv which reflects intracutaneous movements of a viscous type). In grade III lymphoedema, Ue is decreased and Uv is increased. These changes are explained by volume variations and histological alterations, and tend to normalize after treatment. Our study shows that measurement of extensibility is useful in evaluation of volume variations, effects of therapy, and disease evolution, as Ue variations correlate with volume variations and with Uv changes. This technique also provides information which is useful in assessing patients' functional difficulties in relation to skin infiltration, and might be of value in lymphoedema follow-up.
BACKGROUND: The aim of our study was to image psoriasis plaques by ultrasound to assess the changes in psoriasis and to measure and quantify them objectively. MATERIALS AND METHODS: Thirty-one psoriasis plaques were studied in 19 patients. Measurements of skin thickness were obtained with a high resolution B-mode echographic system. RESULTS: Some changes were seen in psoriatic skin. A new structural element was observed: a wide subepidermal nonechogenic band. The other changes were a decrease in dermal echoes that were less intense and less dense, and an increase in the epidermal and dermal skin thicknesses. The skin thickness was increased in all psoriasis plaques as compared to apparently normal skin (P < 0.001). The average increase was 67% for whole skin and 200% for epidermis. CONCLUSIONS: Ultrasound imaging of psoriatic skin allowed the identification of different skin changes induced by psoriasis, and particularly, the differentiation between epidermal and dermal alterations. We presume that epidermal thickness reflects epidermal proliferation and desquamation, and the increase in the dermal and whole skin thickness reflects infiltration. We feel that ultrasound imaging of psoriatic skin is a quantitative method that is as easy and noninvasive as the psoriasis area and severity index (PASI). It could be used for following up patients with psoriasis and could achieve widespread use, especially in research protocols.
Clinical aspects of sequestrated meningocele can be varied, causing difficulties in histopathological diagnosis. The meningeal tissue is scanty and appears as nonspecific connective tissue, therefore it may be overlooked. One classical and two unusual clinical presentations of sequestrated meningocele are described: one of the latter presenting as two atrophic midline bald patches, and the other as a tiny occipital midline hair tuft. In the unusual cases, the pathologic diagnosis was made possible only with the combination of clinical, histologic, and immunohistochemical studies. Sequestrated meningocele should be included in the differential diagnosis whenever hair abnormalities are observed in neonates and young children. We believe that there is a causal relationship between anomalies of the central nervous system and the cutaneous adnexal malformations.
INTRODUCTION: Melanoma is rare in children. We report a new case. CASE REPORT: A black tumor on the medial aspect of the right thigh was removed at the age of 5 years. Histologic diagnosis was melanoma. One year later, a metastasic inguinal lymph node appeared in the right inguinal fold. Exeresis with curetage was performed followed with chemotherapy. Outcome has been favourable to date. DISCUSSION: The diagnosis of melanoma is difficult in children and is usually mistaken. In this case, the histologic diagnosis appears to have been exact and chemotherapy was performed. The rationale and modalities have been discussed.
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Blue Rubber Bleb Naevus Syndrome is a rare pathology: 70 cases have been noted since the first description, by Gascoyen, in 1860. It is an acquired disease which usually appears before the age of ten. It is marked by angiomatic cutaneo-digestive tumours combined with chronic anaemia and iron deficiency. Its many and various complications, particularly cataclysmic haemorrhages, car jeopardize the vital prognosis throughout. This little known syndrome is rarely diagnosed at the time of its first manifestations. We recall, from two cases and a review of their literature, the clinical characteristics of the syndrome, its complications and its essentially symptomatic treatment. We therefore suggest an approach to its diagnosis.
Langerhans' histiocytosis is a proliferation of atypical Langerhans cells which may affect several different organs. Two women had lesions of the vulva which led to the diagnosis. Vulvular involvement is the most frequent in gynaecological localization of Langerhans' histiocytosis. Pathological and immunohistochemical tests confirm the diagnosis.
We made a prospective study of 60 pregnant women from February 1992 to July 1992. Women with pathologic pregnancies were excluded. Fifty-one women had pigmentary changes; the most frequent was linea nigra in 45. Melasma was observed in 3 women only, and the other local melanosis were rare. Vascular changes appeared in 50 women and vascular spiders in 32. Only one vascular spider was seen on a leg; all the others were on the upper part of the body. Eighteen women had a palmar erythema. New striae distensae appeared in 37 women, the more often on the abdomen. Among the other non classified skin changes, acne appeared in 14 women, oedemas of the legs in 22, oedemas of the eyelids in 3 and molluscum fibrosum gravidarum in 4. We emphasize that skin changes are a frequent and polymorphous feature in pregnancy.
BACKGROUND AND DESIGN: For a period of 1 year, all pregnant women presenting with itching were investigated by clinical, histologic, immunopathologic, and laboratory studies. Fifty-one of 3192 pregnant women were studied. RESULTS: We identified (1) two typical cases of herpes gestationis, with an approximate incidence of one in 1700 pregnancies; (2) 22 cases of pruritus gravidarum, including five cases with a polymorphous skin eruption, with an incidence of one in 145 pregnancies; (3) 25 cases of polymorphic eruption of pregnancy, including diseases without maternal or fetal side effects and without criteria defining herpes gestationis or pruritus gravidarum, with an incidence of one in 130 pregnancies; and (4) two cases of intercurrent disease (one scabies and one exfoliative dermatitis). CONCLUSION: Our study is a prospective homogeneous account of pruritic dermatosis of pregnancy. Our results show that the incidence of herpes gestationis is higher than is usually reported in the literature and that pruritus gravidarum must be considered in the presence of itching occurring during pregnancy, with or without skin eruption.
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