[Febrile cellulitis surrounding a scar revealing a large immunoblastic B-cell lymphoma]. / Dermohypodermite fébrile péricicatricielle révélatrice d'un lymphome B immunoblastique à grandes cellules.

BACKGROUND: Secondary skin sites of lymphoma appear in the advanced stages of the disease. We report the first case of a pericicatricial skin infiltration, mimicking febrile dermohypodermitis, revealing diffuse immunoblastic large B-cell non-Hodgkin's lymphoma. PATIENTS AND METHODS: Four months after decompressive cervical laminectomy, a 56-year-old man presented an inflammatory pericicatricial patch evoking cellulitis in a setting of hyperthermia and lymphadenopathy. Blood cultures and bacteriological analysis of skin biopsy samples were negative. The images showed infiltration of the soft subcutaneous areas and polyadenopathy. Two weeks later, several subcutaneous nodules appeared on the trunk. Histological analysis and immunolabelling pointed to immunoblastic large B-cell non-Hodgkin's lymphoma. A clone of B lymphocytes CD45+, CD20+, CD79a+, Bcl2+, CD5+, MUM1+, CD3-, CD10-, CD23- and Bcl6- was seen. The remainder of the extension examination was negative. CHOP-rituximab polychemotherapy resulted in complete regression of all lesions, notably the inflammatory cervical plaque. DISCUSSION: Secondary skin manifestations of lymphoma are generally non-specific (pruritus, ichthyosis, purpura, etc.) rather than specific in terms of lymphoid infiltration. As in our patient, certain cutaneous sites of lymphoma may have a misleading clinical presentation, histological analysis alone was able to provide a conclusive diagnosis. In our patient, the highly specific infiltration seen around the entire scar could either suggest a Koebner phenomenon or point to a role of the cutaneous aggression within the development of an inflammatory process contributing to pericicatricial infiltration by lymphoid cells. Locoregional invasion from the osseous part of the cervical spine and not macroscopically diagnosed during neurosurgery could also be responsible.

[Syringotropic and pilotropic cutaneous T-cell lymphoma without follicular mucinosis]. / Mycosis fongoïde syringotrope et pilotrope sans mucinose folliculaire.

BACKGROUND: Mycosis fongoides rarely exhibits predilection for infiltration of hair follicles and eccrine glands. We report the case of a patient with cutaneous T-cell lymphoma with syringotropism and pilotropism without follicular mucinosis. CASE REPORT: A 51-year-old man presented with erythematous infiltrated patches with alopecia and anhydrosis, cystic lesions, comedon-like lesions, follicular keratosis and an ulcer over the lower left leg. Clinical examination revealed no palpable adenopathy or hepatosplenomegaly. The patient complained about hypohydrosis. Histological examination of skin biopsies evidenced pilotropic cutaneous T-cell lymphoma without follicular mucinosis. Immunohistological examination and T-cell receptor B-chain gene rearrangement analysis showed a clonal population of T-cells. Moreover sweat glands and sweat ducts were infiltrated by atypical lymphocytes with syringolymphoid hyperplasia. DISCUSSION: Syringolymphoid hyperplasia and folliculotropism without follicular mucinosis are rarely seen in mycosis fongoides. Deep biopsies of adnexal structures are required for this critical diagnosis and clonal rearrangement of TCR genes is a reliable means of assessing clonality. Syringolymphoid hyperplasia and pilotropism without mucinosis are variants of cutaneous T-cell lymphomas.

[Acrodermatitis enteropathica-like syndrome secondary to branched-chain amino acid deficiency during treatment of maple syrup urine disease]. / Pseudo-acrodermatite entéropathique secondaire à un déficit en acides aminés ramifiés au cours du traitement d'une leucinose.

INTRODUCTION: Clinical pictures resembling acrodermatitis enteropathica have been described in acquired zinc deficiency and deficiencies of other nutrients such as biotin, essential fatty acids and amino acids as well as biotin metabolism disorders. We describe the case of an infant with maple syrup urine disease who developed an acrodermatitis-like syndrome due to iatrogenic valine and isoleucine deficiency. CASE-REPORT: A diagnosis of maple syrup urine disease was made in a 5-month-old infant girl with severe neurologic disorders with extremely high levels of the three branched-chain amino acids (leucine, valine and isoleucine) in plasma and urine. Seven days after the start of therapy with a diet excluding these branched-chain amino acids, plasma isoleucine and valine concentrations were low while plasma leucine remained elevated. At the same time, a periorificial and acral dermatitis appeared together with diarrhea. Serum zinc concentrations were normal. A diagnosis of acrodermatitis enteropathica-like syndrome secondary to isoleucine and valine deficiency was suspected. Valine and isoleucine supplementation resulted in rapid resolution of the eruption. DISCUSSION: Several cases of acrodermatitis enteropathica-like eruptions resulting from therapeutic protein restriction diets have been described in infants with different aminoacidopathies. The accompanying dermatosis was associated with a raised plasma leucine/isoleucine ratio and/or isoleucine deficiency, or valine deficiency. While the exact pathogenesis of the skin lesions has not been established, these observations show that branched-chain amino acids are essential for normal growth and differentiation of keratinocytes. The essential role of isoleucine is further substantiated by the fact that its presence is critical in keratinocyte culture media, with growth arrest occurring upon its depletion.

X-ray structure of a lectin-bound DNA duplex containing an unnatural phenanthrenyl pair.

DNA duplexes containing unnatural base-pair surrogates are attractive biomolecular nanomaterials with potentially beneficial photophysical or electronic properties. Herein we report the first X-ray structure of a duplex containing a phen-pair in the center of the double helix in a zipper like stacking arrangement.

Novel methods for biocatalyst screening.

There have been a number of recent advances in catalysis assays applicable for screening biocatalyst libraries in high-throughput format. These include instrumental assays such as high-performance liquid chromatography, mass spectrometry, capillary electrophoresis and IR-thermography, reagent-based assays producing spectroscopic signals (UV/VIS or fluorescence) in response to reaction progress, and assays based on fluorogenic or chromogenic substrates. These fluorogenic substrates enable the assaying of a variety of enzymes in enantioselective and stereoselective manner, including alcohol dehydrogenases, aldolases, lipases, amidases, epoxide hydrolases and phosphatases.

High-throughput screening for biocatalysts.

Recent progress in high-throughput enzyme assays includes new examples of fluorogenic and chromogenic substrates, fluorescence resonance energy transfer substrates, and applications of the pH and pM indicator methods. Recent developments of Horeau's pseudo-enantiomer derivatisation method to screen enantioselectivities in high-throughput have also been reported.

[Problems posed by the therapeutic use of photosensitizers in dermatology]. / Problèmes posés par l'utilisation thérapeutique des photosensibilisateurs en dermatologie.

Three phototherapeutic regimens with photosensitization are now used in dermatology: PUVA (psoralen + UVA), TUV (crude coaltar + UV), PRT (phototherapy with hematoporphyrin derivative). The efficiency of PUVA and TUV is well known in several dermatoses. PRT is now being tested experimentally. For TUV, the lack of a standardized regimen does not allow a clear-cut evaluation of the therapy. For PUVA, late side-effects, particularly carcinogenicity have to be considered. To improve efficiency and minimize the side-effects of PUVA some procedures, such as association with retinoïds, pharmaco-kinetic studies for individual adaptation of the therapeutic regimen and the use of new less mutagenic psoralens are helpful. The persistent phototoxicity following treatments with hematoporphyrin derivative constitutes the major side-effect observed, for this phototherapy.

Anomer-selective inhibition of glycosidases using aminocyclopentanols.

[reaction: see text] (1S,2S,3S,4R,5R)-4-amino-5-(hydroxymethyl)cyclopentane-1,2,3-triol 1 is prepared stereoselectively from D-lyxose and displays anomer-selective inhibition for beta-galactosidase (Ki = 3.0 x 10(-6) M) and beta-glucosidase (Ki = 1.5 x 10(-7) M), over alpha-galactosidase (Ki = 2.3 x 10(-5) M) and alpha-glucosidase (IC50 = 1.0 x 10(-4) M). There is no observable cross-reactivity with alpha-mannosidase, beta-mannosidase, or alpha-L-fucosidase.

Stereoselective inhibition of alpha-L-fucosidases by N-benzyl aminocyclopentitols.

[structure: see text] (1R,2R,3R,4R,5R)-4-Amino-5-methylcyclopentane-1,2,3 -tr iol 8, its 4S stereoisomer 9, and their acyclic analogues (R)- and (S)-2-aminobutanol 11 and 12 are selective but moderate inhibitors of alpha-L-fucosidases. N-Benzylation selectively enhances inhibition potency for aminocyclopentitol 8 (--> 1, K(i) = 6.8 x 10(-)(7) M) but decreases inhibition for its 4S-stereoisomer 9 (--> 2, K(i) = 1.1 x 10(-)(4) M) and for the aminobutanols 11 (--> 13, no inhibition) and 12 (--> 14, no inhibition).

Synthesis and evaluation of aminocyclopentitol inhibitors of beta-glucosidases.

[reaction: see text] (1R,2S,3S,4R,5R)-4-Amino-5-(hydroxymethyl)cyclopentane-1,2,3-triol 1, prepared from D-glucose, inhibits beta-glucosidases from Caldocellum saccharolyticum (Ki = 1.8 x 10(-7) M) and from almonds (Ki = 3.4 x 10(-6) M). Inhibition is not influenced by N-ethylation (--> 15) but is strongly reduced upon N-acetylation (--> 12). Inversion of stereochemistry at C(5) (--> 14) has little effect on inhibition of beta-glucosidases. These experiments suggest that 1 acts as an analogue of a protonated beta-glucoside.

[Persistent light reaction due to phenothiazines in atopic disease (author's transl)]. / Photo-allergie rémanente aux phénothiazines chez l'atopique.

Phototesting has been performed in 6 atopic patients who had been suffering of eczematous lesions on sun exposed areas. MED was always of normal value. Phototest was positive in 2 patients. Above all photopatch-test with chlorpromazine was positive in 5 out of 6 patients, allowing the diagnostic of persistent light reaction. The cause of photosensitivity may be the use of phenothiazin derivatives anti-histaminic drugs. The use of phenothiazin derivatives has to be avoided in the treatment of atopic condition for this reason and because of the potential risk of actinic reticuloid in the course of persistent light reaction.

[Cutaneous argyria: an electron microscopic study of four eases with microanalysis X study of one case (author's transl)]. / Etude en microscopie électronique et en microanalyse X de 4 cas.

This study was done in order to follow the fate of silver in the dermis of chronic argyria. Silver was easily recognizable in the tissue as irregular aggregates of elementary granules round or ovoid in shape, ranging from 30 to 40 mm in size. The microanalysis X showed that the metal was bound with sulfur. In the cases of recent intoxication, the main location of silver was intracellular: the granules were found in the lysosomes as elementary particle or as dense heterogen bodies. In the cases where intoxication had been stopped a long time ago, silver was found either on fibrillar component of the connective tissue or in the basal material of sweat glands. The results show that silver is at first phagocytized by macrophages but this cell is unable to perform complete degradation of the silver salt or metal. Then silver is found on connective fibers where it remains on sulfated glycoproteins.

[Cutaneous symptoms of carcinoid tumours of the small bowel (author's transl)]. / Les manifestations cutanées des carcinoïdes du grêle.

Cutaneous symptoms of carcinoid tumours are classified in 4 groups: --Acute type: flusch and vasomotor signs (the caracters of which are described). --Chronic type: telangiectasia, hyperpigmentation, sclerodermia-like, cutaneous maetastasis. --Rare type with non-diagnostic sign. --Types in association with other dermatological conditions either unrelated disease or with a likely related disease such as phacomatosis. Dermatological diagnosis is generally easy as cutaneous symptoms is concomitant with metastatic tumours and increased levels of serotonin and 5-H.I.A. The carcinoid syndrom may take place in the concept of the tumours of the A.P.U.D. system on clinical, embryological, histological and biochemical basis.

[Cutaneous symptoms of thrombocytemia (author's transl)]. / Manifestations cutanées des thrombocytémies

By the study of 4 personal cases and a review of the literature, the authors describe the symptoms which may complicate and even reveal an essential thrombocythemia. They are: painful symptoms which indicate a permanent or paroxystic vasculomotor syndrome by their localization and their course: -erythematous lesions of an inflammatory livedo type located on the legs and blue-red reticular spots on the sole of the foot: --occurrence of gangrene on finger ends or of necrotic ulcerations of the legs. Etiological conditions, exploration of primary hemostasis, physiopathology of the troubles are described. As for therapeutics the antiaggregants have a suspensive effect on the symptoms but only the control of the hematological status may allow recovery.

[Value of rheoplethysmographic methods in the etiological diagnosis of leg ulcers]. / Apport des méthodes rhéopléthysmographiques au diagnostic étiologique des ulcères de jambe.

Two recently introduced impedance plethysmographic methods have been used in the etiologic diagnosis of 100 leg ulcers: the occlusive rheoplethysmography (RPO) and the irrigraphy. RPO investigates the venous part of the lower limb circulation and irrigraphy the arterial blood flow. These two quantitative methods associated with Doppler examination of venous system allow to point out the type and the topography of the vascular alterations. The value of rheoplethysmographic methods in etiological leg ulcers investigation is reported.

[Influence of retinoids on the bioavailability of methoxy-8-psoralen]. / Influence des rétinoïdes sur la biodisponibilité du méthoxy-8-psoralène.

The retinoid-PUVA combination has been recognized as a satisfactory treatment of psoriasis. The absorption of 8-methoxypsoralen (8-MOP) is subject to wide interindividual variations under the influence of factors that are not yet known with certainty but are independent of age, sex and food taken at the same time as the psoralen. Whether concomitant retinoid administration influences the bioavailability of 8-MOP was considered an interesting question. The pharmacokinetics of 8-MOP were studied and compared in two populations of psoriatic patients: 119 patients treated with PUVA alone (psoralen-ultraviolet A) and 40 patients treated with the etretinate-PUVA combination (RePUVA). 8-MOP was assayed by the modified Ljunggren method 1 h, 1 h 30, 2 h, 2 h 30, 3 h and 4 h after ingestion of 8-MOP. The pharmacokinetic values recorded were: time and peak value of maximum plasma 8-MOP concentration (Tmax, Cmax) and area under the curve of time-related 8-MOP concentrations (AUC). The results obtained were as follows: Tmax PUVA 2 h 02 +/- 53 min RePUVA 1 h 56 +/- 50 min Cmax PUVA 159.12 +/- 88.85 ng/ml RePUVA 163.63 +/- 92.85 ng/ml AUC PUVA 343.33 +/- 211.06 ng*h/ml RePUVA 388.12 +/- 251.03 ng*h/ml Statistical analysis showed no significant difference in pharmacokinetic values between patients on PUVA alone and patients on RePUVA. Taking etretinate therefore does not alter the pharmacokinetics of 8-MOP and should not require any change in PUVA treatment.

[Fluindione-induced acute exanthematous pustulosis with renal involvement]. / Pustulose exanthématique aiguë généralisée à la fluindione avec atteinte rénale.

INTRODUCTION: Fluindione (Previscan) is an oral anti-vitamin K anticoagulant, widely prescribed in France. Contrary to phenindione, which is also an indanedione derivative, very few cases of immunoallergic reactions have been described. CASE REPORT: A 68 year-old man, treated with fluindione for cardiac arrhythmia, presented with a pustular eruption and erythema twenty days after initiation of treatment. The eruption was associated with hyperthermia, arthralgia, neutrophilia (11,000/mm2), hepatic cytolysis and renal involvement including acute renal failure, hematuria and proteinuria. In view of the absence of any earlier case in the literature, we did not impute fluindione and the drug was reintroduced and led to the rapid recurrence of all the same manifestations. DISCUSSION: These manifestations were consistent with an immunoallergic reaction to fluindione (probable intrinsic imputability I3) and acute interstitial nephritis (probable intrinsic imputability I3). We believe this is the first case of acute generalized exanthematous pustulosis induced by fluindione (intrinsic imputability Bo). A few rare cases of fluindione-induced hypersensitivity reactions and acute interstitial nephritis, however, have been described.

[Plasma levels of psoralens after celery ingestion]. / Etude des taux plasmatiques de psoralènes après ingestion de céleri.

Psoralens are photosensitizing substances present in many vegetables, some of which are routinely consumed. These vegetables are responsible for contact phytophotodermatitis, but it was agreed that they did not produce photodermatitis when taken orally. Ljunggren has recently questioned this concept by reporting a case of phototoxic accident which occurred after ingestion of 450 grams of celery roots (Apium graveolens). In a study in healthy volunteers we looked for psoralens in blood and analyzed the cutaneous photosensitivity by the minimal phototoxic doses (MPD) method, after ingestion of celery in large amounts (500 grams and more). Plasma concentrations of psoralens were inexistant in all subjects and at all sampling times, and no phototoxic reaction was detected by MPD. Celery roots, therefore, do not seem to be photosensitizing, even after ingestion in large amounts, but they might increase the risk of phototoxicity in PUVA-therapy. The same applies to fennel and parsnip.

[Immuno-allergological study on 30 atopic patients (author's transl)]. / Exploration immuno-allergologique de 30 sujets atopiques.

An immuno-allergological study was performed on 30 atopic patients and the data were compared to those of 30 healthy controls. The findings were: --increased level of IgE class immunoglobulins in the atopic patients; --increased frequency of the presence of specific reagin to numerous allergens in the sera of atopics (RAST). The total amount of IgE was related to the presence of numerous and/or strongly positive RAST; --immediate hypersensitivity reaction (IHR) to various allergens and specially house-dust. No well-definite correlation was found between IHR and RAST for the same allergens and between the in vitro determination of allergens and a known history of clinical provocation by these allergens. The frequency of positive IHR was related with high level of total IgE; --decreased frequency of delayed cutaneous hypersensitivity tests and specially of those with candidin extracts. Cutaneous hyporeactivity to natural antigens was in relation with high level of total IgE; --no significant statistical difference was found between the amount of T and B lymphocytes; --increased frequency of HLA-A2 group and decreased frequency of HLA-B7 group.

[Sporadic superficial pemphigus in the child: 2 cases]. / Pemphigus superficiel sporadique de l'enfant: 2 observations.

INTRODUCTION: Excepting the endemic foliaceus form, childhood pemphigus is uncommon. We report two cases of pemphigus foliaceus in children with typical clinical manifestations. CASE REPORTS: Case n(o) 1. A 5-year-old girl was seen for a vesiculobullous crusted dermatosis involving the trunk and the face which had developed over the last 5 months, predominantly in periorificial and fold localizations. Histology showed intragranulous acatholysis. Direct skin immunofluorescence was positive for anti-intercellular substance IgG and C3. Indirect immunofluorescence was positive for anti-intercellular substance antibodies at 1/500. The diagnosis of superficial pemphigus was retained and the child was given dapsone associated with systemic prednisone (1.5 then 2.5 mg/kg/d). Dapsone was stopped on day 15 due to poor hematological tolerance. Outcome was favorable allowing withdrawal of prednisone at 18 months. Case n(o) 2. A 6-year-old had developed since the age of 18 months a generalized and polycyclic pruriginous erythemato-squamous dermatosis with oozing discharge which started and predominated on the face (periorificial zones). Trace element (copper, selenium, zinc) and vitamin (A, E and B1) assays were within the normal range. Glucagon was normal. Histological examinations of several biopsies were non-contributive. Diagnosis of pemphigus foliaceus was finally obtained after repeated direct immunofluorescence tests which revealed anti-intercellular substance IgG. Indirect immunofluorescence was negative. The child was given prednisone (2 mg/kg/d). DISCUSSION: In children, pemphigus foliaceus has an exceptional frequency and diagnosis is often made quite late (mean 8 months). The diagnosis should always be entertained in children who develop chronic extensive erythemato-squamous and crusted dermatosis, even if formation is absent. Direct skin immunofluorescence confirms the diagnosis and should be repeated if negative in cases with highly suggestive clinical presentations. It would be reasonable to attempt "minor" treatments as the first line approach. Systemic corticosteroids are however the treatment of choice despite the risk of classical side effects. Childhood pemphigus foliaceus is not an attenuated clinical form of adult pemphigus. Mortality is not negligible and is close to that in adults.