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Biomedical subjects

L Misery

Publications and source records attributed to L Misery.

At least 37 records · Page 2Linked to original sources

[Aquadynia: a role for VIP?].

BACKGROUND: Aquadynia (water-related cutaneous pain) is a very rare disorder, recently described. CASE REPORT: A 40 year-old woman suffered from aquagenic pruritus, complicated by paresthesia and pain. There was no clinical argument in favor of a psychiatric disorder, Fabry's disease or any other disease. Clinical and histological cutaneous examinations were normal. Immunohistochemical study of neurotransmitters (substance P, calcitonin gene-related peptide or CGRP, vasoactive intestinal peptide or VIP, somatostatine) did not show any modification in nerve density. However, VIP-immunoreactive epidermal cells were observed. Electromyography and study of somesthesic-evoked potentials were normal. No treatment had provided any efficacy. Clonidine and capsaicin had been prescribed with partial success. DISCUSSION: Three other cases of aquadynia have been reported. Differential diagnoses of aquadynia are aquagenic pruritus and urticaria, hysteria or simulation, Fabry's disease, erythermalgia, peripheral neuropathy or polycythemia vera. The presence of VIP-immunoreactive cells suggests that VIP could be produced by these cells after contact with water. The effects of propanolol and clonidine on aquadynia are in favor of an adrenal component.

Adult↗

[Treatment of familial erythermalgia with the association of lidocaine and mexiletine].

INTRODUCTION: Erythermalgia is a rare acrosyndrome characterized by reddening of the skin, local increase heat and pain. The disease is frequently resistant to treatment. Recently, Kuhnert et al. presented very favorable results using a combination of lidocaine and mexiletine. We used this treatment in 4 patients suffering from familial erythermalgia. OBSERVATIONS: In a family exhibiting severe familial erythermalgia involving 5 members over 3 generations, we treated 4 patients aged 41, 39, 19 and 15 years. In these patients, the erythermalgia known since early childhood, progressed in the form of multiple flares (6 to 7/day) during the day and at night, lasting several hours and often accompanied by headaches. The impact of the disease on their quality of life was major. Only cold-water baths provided temporary relief, obliging them to live with their "feet in cold water". After they had been informed of the modalities of treatment and in the absence of any contraindication, notably cardiologic, 200 mg (100 mg in the youngest patient) of lidocaine were infused in 4 hours in a single intravenous injection on the first day. Mixelitine was introduced on the second day at the dose of 600 mg in 3 oral intakes (200 mg in the youngest patient). The painful paroxistic symptomatology rapidly improved and the flares had disappeared on the 3dr day, thus permitting the progressive reduction in analgesics and major improvement in quality of life. This beneficial effect persisted with oral mexiletine alone, 2 years after the infusion of lidocaine in the first patient treated (and one year after in the other patients). COMMENTS: Primary familial erythermalgia is highly resistant to treatment. The combined action of lidocain and mexiletine, usually well tolerated (class IB antiarrythmic), blocks the sodium channels. The mechanism of action of their analgesic effect is peripheral or central or even mixed. This benefit warrants confirmation in other forms of erythermalgia.

Administration, Oral↗

[Unilateral focal dermal hypoplasia].

INTRODUCTION: Goltz's syndrome or focal dermal hypoplasia is an X-linked dominant disease. Patients suffer from cutaneous, bone, dental and ocular disorders. The mutated gene has not been identified. CASE-REPORT: A 16 month-old girl presented, since her birth, numerous malformations and cutaneous lesions. She had only three toes and three metatarsians on the right foot. The right hand possessed only four fingers but five metacarpiens. No bone abnormality was observed on the left side. Cutaneous lesions were also limited to the right side: linear para-median epidermal hamartoma on the face, several areas of alopecia, longitudinal atrophic and hyperpigmented striations on the lower limbs. Both median incisors were dysplasic. Nails of right hand and foot were absent or striated. Radiographies did not show infra-clinical bone lesions. At birth, trans-fontanellar sonography and sonographies of thorax and abdomen were normal, as well as ophthalmological examination. Psycho-motor development was normal. The diagnosis of Goltz syndrome was proposed. There was no other case in the family. DISCUSSION: This case is very unusual because lesions were restricted to the right side. To our knowledge, only one other case has been reported. Mutation de novo is probable and may be post-zygotic. In the future, there is no reason to fear the occurrence of another case in this family.

Abnormalities, Multiple↗

[Is alopecia areata a psychosomatic disease?].

INTRODUCTION: Destruction of hair follicles by lymphocytes induces alopecia aerata. Hence, immunological mechanisms are involved in this disease, as for numerous dermatoses. Nonetheless, alopecia aerata appears to be a psychosomatic disease. Is there any contradiction? CURRENT KNOWLEDGE AND KEY POINTS: Alopecia aerata often occurs after stress, particularly during mourning. Psychiatric disorders are more frequent in patients with alopecia than in healthy subjects. But these disorders might be secondary to the visible hair disease. Psychopathological mechanisms need to be clarified but alexithymia seems to be the key for understanding how stress could induce hair loss. In the skin (and the scalp) all functions are narrowly controlled by nerve fibers. Among these functions are hair growth and immunity. Immune cells and hair follicle cells possess receptors for neurotransmitters, which are synthesized by neuronal endings. When activated, these receptors are able to modulate cell properties. The same phenomena are described with stress-induced hormones. In alopecia aerata, like in numerous other diseases, psychosomatics and immunology are not opposed because immune cells are controlled by the nervous system through neurotransmitters. FUTURE PROSPECTS AND PROJECTS: Research needs to be thorough in both the fields of psychology and neurobiology. Psychotherapies or psychotropes appear to be useful in the treatment of alopecia aerata.

Alopecia Areata↗

Modulation of substance P and somatostatin receptors in cutaneous lymphocytic inflammatory and tumoral infiltrates.

BACKGROUND: The expression of receptors for neuropeptides in the skin is modified in skin diseases. OBJECTIVE: We studied the cutaneous expression of substance P (SP) and somatostatin (SOM) receptors (SPR and SSTR, respectively) in skin affected by cutaneous inflammatory or tumoral T-cell infiltrates because these two neuropeptides are the ones most involved in inflammation. METHODS: We revealed expression of these receptors using a binding in situ technique that gave highly specific results. Skin biopsies were incubated with biotinylated neuropeptides (SP or SOM). RESULTS: In normal skin, SSTR were observed on blood vessels, smooth muscle fibres and sweat glands. SSTR expression was modified only when expressed by keratinocytes in Ofuji papuloerythroderma and by plasmocytes in plasmocytoma. SPR distribution was not modified in subjects with atopic dermatitis or lupus. The expression of SPR in the epidermis was diminished in Ofuji papuloerythroderma and parapsoriasis and absent in mycosis fungoides. CONCLUSIONS: These results suggest that malignant lymphocytic infiltrates can inhibit SPR expression on keratinocytes.

Humans↗

Functional HIV CXCR4 coreceptor on human epithelial Langerhans cells and infection by HIV strain X4.

HIV can cross the intact epithelium of genital mucosae via Langerhans cells. Fresh Langerhans cells are known to express CD4 and CCR5. The presence of CXCR4 on the surface of cultured but not freshly isolated Langerhans cells has been described. In the present study, we demonstrate that CXCR4 was expressed by fresh Langerhans cells isolated and purified from epidermis. However, the percentage of Langerhans cells expressing CXCR4 or CCR5 increased during maturation of the cells in culture, especially in the presence of exogenous granulocyte-macrophage colony-stimulating factor. To determine whether CXCR4 was functional, freshly isolated Langerhans cells were infected with HIV LAI, a T-cell-tropic strain, and p24 protein production was measured in culture supernatants. p24 production was observed when infected Langerhans cells were cocultured with SupT1 cells. However, the presence of HIV provirus DNA was evidenced within the infected Langerhans cells by nested PCR. Ultrastructural studies confirmed the formation of syncytia when Langerhans cells were cocultured with SupT1 cells. Preincubation of Langerhans cells with azidothymidine or SDF-1-alpha, a natural ligand for CXCR4, prevented infection. These data demonstrated that CXCR4 is present on the surface of Langerhans cells freshly isolated from human skin epidermis and that this expression is functional.

CD4 Antigens↗

[Photodermatosis and photoprotection in children].

Photodermatosis is an heterogeneous group of affections characterized by an abnormal sensitivity of skin to sunlight responsible of a local or generalized eruption. Rare in children, the most frequent of them such as sun-burns, phytophotodermatosis and phototoxicity are fortunately benign, brief and spontaneously resolving. Some of them disappear spontaneously after few years like polymorphic light eruptions which are rather observed in children older than ten years. In case of recurrence, rare diagnosis have to be investigated with specific tests (phototests, blood exams, genetic investigations) as some metabolic or genetic disorders may be revealed by a photosensitivity, especially before the age of three years. The questioning of the parents and of the child is fundamental. Many diagnosis may be discussed depending on age and circumstances. Treatment consists in photoprotection recommendations, solar eviction and regular application of protective screens, whose spectrum of protection are different and have to be known in order to prescribe an adapted photoprotection.

Age Factors↗

The neuro-immuno-cutaneous system and ultraviolet radiation.

Numerous cells are closely associated with cutaneous nerve fibers, which through the action of neuropeptides are able to modulate cellular function. Anatomical and physiological links between immune cells and nerves and other cells in skin are so close that we propose the concept of a neuro-immuno-cutaneous system (NICS). Under the impact of ultraviolet radiation (UVR) virtually all properties of the NICS are modified. UVR induces melanin synthesis and immunosuppression, events in which neuropeptides, especially melanocyte stimulating hormone (MSH) and the calcitonin gene-related peptide CGRP, play a role.

Calcitonin Gene-Related Peptide↗

Presence of circulating abnormal CD34+ progenitors in adult Langerhans cell histiocytosis.

Langerhans cell histiocytosis (LCH) is related to the proliferation of cells, which are similar to Langerhans cells (LC) but possess many abnormal characteristics. Lesions are widespread and this fact suggests that LCH cells or their precursors are present in the blood of patients. In five adult patients, we have isolated and cultured CD34+ blood progenitors of dendritic cells. We studied their phenotype by flow cytometry and their functional properties in mixed culture with heterologous lymphocytes and with autologous lymphocytes in the presence of tri-nitro-phenyl antigen (TNP). The amount of CD34+ precursors was dramatically higher than controls but a high mortality occurred during the in vitro differentiation. The phenotype of surviving cells was similar to LC phenotype (CD1a+, CD83+, Lag+) but some of them expressed CD2. These cells were able to induce T cell proliferation in mixed culture. They could not initiate primary response to TNP, except in a patient treated with thalidomide. In our hands, these CD34+ cells may be precursors of LCH cells.

Adult↗

Expression of substance P receptors in normal and psoriatic skin.

Recently, substance P receptors (SPR) have been detected in neonatal foreskin. Our purpose was to determine the expression of SPR in other localizations than neonatal foreskin. As SP has been implicated in the pathogenesis of inflammatory cutaneous lesions, we wondered whether SPR localization was modified in psoriatic lesions. In normal skin, SP binding sites were detected using biotinylated SP and abrogated by a specific NK1 antagonist (spantide) on blood vessels, sweat glands and hair follicles. In the normal epidermis, SPR were usually observed on granular layers but may also be observed on other cell layers. The SP binding processed on cultured keratinocytes demonstrated that SPR were expressed in the epidermis, except basal cell layers, confirming that keratinocytes constitutively express SPR. In skin lesions of psoriatic patients, SP binding sites were expressed on the uppermost keratinocytes which are not granular cells, and seem to be overexpressed. Our results raise the question of the role of SPR on psoriatic keratinocytes.

Adult↗