Cutaneous leukocytoclastic vasculitis and renal cancer: two cases.

We report two cases of cutaneous leukocytoclastic vasculitis (CLV) leading to the discovery of an as yet asymptomatic, surgically curable clear cell carcinoma of the kidney. CLV causative factors or associated diseases are usually drugs, infection, or collagen vascular disease, but rarely malignancies. In such cases, these are more often malignant hematologic diseases than solid neoplasms. We believe that in apparently idiopathic CLV, a screening examination should be done to detect any underlying early-stage curable solid malignancy.

The fine structure of calcified Mandl's corpuscles in teleost fish scales.

Calcified Mandl's corpuscles present in the internal layer (or fibrillary plate) of the teleost fish scale were studied by transmission and scanning electron microscopy for a better understanding of this special type of mineralization process. The corpuscles show a great variability in their structure, form and surface features depending on the arrangement of the collagen fibrils in the internal layer of the different fish species studied, on the localization of the corpuscles in the scale and on the technical treatment to which the scale is subjected.

The fibrous structure of coelacanth scales: a twisted 'plywood'.

Isopedin is a network of collagen bundles present in the scales of most fishes. The scales of coelacanths show a remarkable three-dimensional arrangement of this network which is similar to a regularly twisted plywood. The successive fibrous layers cross at an angle which differs slightly from a right angle. It results that the whole system is twisted. The progressive rotation of the fibril direction is right-handed. Certain preferential orientations of fibrils have been observed, namely parallel to the growth rings. Such arrangments also exist in the embryonic cornea of birds and in the cuticle of certain insects, but do not present such an extensive and regular development.

Lymphoma developing in a patient with rheumatoid arthritis taking methotrexate.

We report one case of non-Hodgkin lymphoma in a patient, with a 30-year history of rheumatoid arthritis, taking low dose methotrexate weekly over a 10-month period. The mild immunosuppression that occurs with methotrexate therapy probably places patients with rheumatoid arthritis at added risk of developing lymphoproliferative diseases, but coincidence cannot be excluded.

Paraneoplastic Raynaud's phenomenon.

A testicular tumour could be diagnosed by the occurrence of a Raynaud's phenomenon complicated by severe digital arteritis. The arteritis rapidly regressed under prostacyclin therapy. Such vascular manifestations are frequent in testicular carcinoma, but they usually develop after chemotherapy. To our knowledge, this is the first case where they preceded the diagnosis and specific treatment of a tumour of the testis.

Subacute cutaneous lupus erythematosus associated with Hodgkin's disease.

Simultaneous occurrence of subacute cutaneous lupus erythematosus and malignancy has rarely been reported. We report the first case of subacute cutaneous lupus erythematosus associated with Hodgkin's disease. Although our case does not fulfill the criteria of paraneoplastic process, a relationship between the two disorders might be suggested by their simultaneous occurrence and in view of the cases of disseminated lupus erythematosus associated with lymphoma reported so far. The different hypotheses are discussed.

Contribution of collagen stable isotope biogeochemistry to the paleobiology of extinct endemic vertebrates from Tenerife (Canary Islands, Spain).

The paleodiet and paleoenvironmental context of two extinct species from Tenerife island, one giant rat Canariomys bravoi and one giant lizard Gallotia goliath, have been investigated using carbon and nitrogen isotopic compositions of fossil bone collagen. Preliminary to this study, a calibration of the isotopic variations of bone collagen from modern Rat Rattus rattus, Rabbit Oryctolagus cuniculus and Lizard Gallotia galotti relative to environmental conditions on Tenerife Islands has been attempted. No clear relationship could be found between collagen delta13C and delta15N values and aridity; the only relevant factors seem to be seashore proximity for rat, and the relative amount of C3 and CAM plants. It seems that anthropic activities have interfered with the expected relationships between collagen isotopic compositions and environmental conditions. Most fossil specimens yielded well preserved collagen. The isotopic composition of giant rat and giant lizard collagen suggest a purely C3 environment, possibly more humid than today on Tenerife. Large ranges of nitrogen isotopic compositions, especially within giant rats, may be due to local environmental conditions. Further work is needed in order to provide more valuable paleobiological information in order to better understand the role of environmental factors in the evolution and extinction of insular endemic species on Tenerife.

[Heparin-like anticoagulant in small cell bronchial carcinoma]. / Anticoagulant circulant "heparine like" au cours d'un carcinome bronchique à petites cellules.

Acquired circulating heparin-like anticoagulants may have different causes, the most frequent being plasmocyte proliferation. The authors report an exceptional case of association between an heparin-like inhibitor and a small-cell anaplastic lung carcinoma, and they review the pathogenesis, laboratory diagnostic methods and treatment of the anticoagulant.

[Antiseptic treatment of the umbilical cord in newborns: survey and recommendations]. / Antisepsie du cordon ombilical du nouveau-né: enquête et recommandations.

AIM: To determine whether umbilical cord care of the neonate is in accordance with the guidelines of antiseptic treatment at this age of life. MATERIAL AND METHODS: A survey was conducted during the 3rd trimester of 1996 in 57 maternity units and departments of neonatalogy in the region of Provence-Alpes-Côte d'Azur (south of France). A questionnaire was sent to the head of each unit asking the modalities of disinfection of the umbilical cord. RESULTS: Fifty units answered the questionnaire. Six different groups of antiseptic products were used, corresponding to 17 distinct commercial preparations. The simultaneous association of several products (two or three) was done in 70% of cases. Eosin was the most frequently used (60%), in association with 25 units. Alcohol was used in 28 centers (56%). It was associated 22 times. Chlorhexidine was used in 16 units (32%), twice alone, and with another topic 14 times. The commercial association chlorhexidine-benzalkonium chloride (Biseptine) was reported seven times (six times in association with another topical treatment). Ektogan (a powder of Zn and Mg peroxide and Zn oxide) was used in ten centers, always in association. Hexamidine was used in four units, once in association. Silver nitrate, Milian solution, iodinated alcohol, and povidone iodine were respectively used once. CONCLUSION: This survey shows that a great variety of umbilical cord care modalities is used in this region, and that the recommendations for antiseptic treatment in young babies, are not always respected. According to these, eosin, ethanol, Ektogan and iodine should not be used for this purpose. Although chlorhexidine has been proven to be the most suitable disinfectant, it comes only in third place, used in association in 95% of the cases. Several studies in neonates have shown that it is well tolerated and efficient even if it delays cord separation. This study should lead to interdisciplinary consensual guidelines for umbilical cord care.

[Cheilitis granulomatosa in a child]. / Chéilite granulomateuse chez une enfant.

UNLABELLED: Granulomatous cheilitis is a rare disorder characterized by intermittent swelling of one or both lips, which may become persistent. We report a case occurring in a child, which improved with minocycline treatment. CASE REPORT: An eight year-old girl was seen for the swelling of her upper lip, which persisted for more than one year. There was no history of applied irritants, local trauma or atopy. On examination, the upper lip was swollen, indurated, erythematous and fissured with a bilateral perleche. The tongue and gums were normal. There was no facial palsy. The girl was in good health and did not take any drugs. Blood investigations were normal. Patch tests were negative. Antibiotic treatment or local steroids provided only temporary improvement. A biopsy showed extravascular epithelioid and gigantocellular non-caseating granulomas with lymphoid infiltrates. There was no sign of sarcoidosis or Crohn's disease. Treatment with hydroxychloroquine for three months, then with oral metronidazole, did not improve the symptoms. Minocycline (100 mg/d) was effective. CONCLUSION: Granulomatous cheilitis should be considered in children as well and differentiated from infectious, allergic or factitious dermatoses. Granulomatous cheilitis can be associated with Crohn's disease or sarcoidosis, and could precede these disorders from several months or years, thus requiring a long-term follow-up.

[Digital necrosis in Raynaud's phenomenon revealing a testicular seminoma. Value of prostacyclin]. / Nécrose digitale au cours d'un phénomène de Raynaud révélateur d'un séminome testiculaire. Intérêt de la prostacycline.

A testicular tumour could be diagnosed by the occurrence of a Raynaud's phenomenon complicated by severe digital arteritis. The arteritis rapidly regressed under prostacyclin therapy. Such vascular manifestations are frequent in testicular carcinoma, but they usually develop after chemotherapy. To our knowledge, this is the first case where they preceded the diagnosis and specific treatment of a tumour of the testis.

[White papulosis of the neck. Clinical aspects of pseudoxanthoma elasticum]. / Papulose blanche du cou. Aspects cliniques de pseudoxanthome élastique.

INTRODUCTION: Acquired elastolysis of the papillary dermis simulating pseudoxanthoma elasticum and white papulosis of the neck are two skin diseases with clearly limited borders. The two entities can be distinguished clinically when papulae converge into yellowish-white patches and histologically by complete absence of the elastic network in the papillary dermis without thickening of the collagen network in the superficial and mid dermis. CASE REPORT: We observed a patient with the clinical and histological criteria for acquired elastolysis of the papillary dermis. Histological examination showed a thick collagen network as described in white papulosis of the neck. DISCUSSION: This case clearly demonstrates the histological continuum between these two entities. A review of the literature also provided evidence of borderline forms, leading us to propose grouping them together. Elastolysis of the papillary dermis would appear to be a relatively specific histological sign differentiating white papulosis of the neck, in a general sense, and acquired elastosis of the mid dermis. Two factors could explain the rare observations of acquired elastosis of the mid dermis in patients with acquired elastosis of the papillary derma: use of the Verhoeff Van Gieson stain without an internal control and imprecise localization of the biopsy.

["Bronze baby" syndrome]. / Le syndrome du "bébé de couleur bronze".

"Bronze baby" syndrome is a rare complication of phototherapy for neonatal jaundice occurring due to modified liver function, particularly cholestasis, of various origins. We report a case which occurred in a premature infant who developed a grey-brown coloration during phototherapy. The infant had haemolytic jaundice due to Rhesus incompatibility complicated by cholestasis of thick bile fluid. Abnormal accumulation of unexcreted photoproducts due to the cholestasis appeared to be the cause of the bronze coloration. The clinical course was favourable and the skin coloration returned to normal a few weeks after the end of the phototherapy. It is essential to identify the underlying liver disease in order to determine the prognosis of this syndrome.

[Chronic edema, monoclonal dysglobulinemia and profuse telangiectasia: a distinct entity?]. / Oedème chronique, dysglobulinémie monoclonale et télangiectasies profuses: une entité distincte?

INTRODUCTION: Monoclonal gammopathy and edema are features encountered during several diseases, especially systemic capillary leak syndrome. The diagnoses of POEMS syndrome, edematous systemic scleroderma and a fortuitous association may be also discussed. We report the cases of two patients which did not fulfill the criteria for such diagnoses. CASE REPORT: Although the 2 cases share some discrepancies, they have also similar and particular features: association of chronic edema, monoclonal gammopathy and profuse and acquired telangiectasias. DISCUSSION: The meaning of these cases remains to be clarified. It might be an entity close to the systemic capillary leak syndrome but characterized by the chronicity of edema and by a distinct cutaneous sign, the occurrence of numerous telangiectasias. These cases emphasize that the acquired and profuse telangiectasias belong to the wide range of cutaneous abnormalities which may be associated with monoclonal paraproteinemia.

[Cutaneo-mucous manifestations of dengue]. / Manifestations cutanéo-muqueuses de la dengue.

OBJECTIVES: To describe muco-cutaneous manifestations of dengue fever, assessing their incidence and histopathological aspects. PATIENTS AND METHODS: During a dengue 2 epidemic, occurring in Guadeloupe in 1994, all patients admitted with a confirmed diagnostic of dengue fever were assessed for dermatological changes by 2 clinicians; 5 patients underwent skin biopsy with immuno-fluorescence staining. RESULTS: Among 39 adult inpatients (Sex ratio 1.1, medium age 41 years) none presented a severe form of the disease, whereas 18/39 (46 p. 100) had some muco-cutaneous changes, associating rash (13 cases (33 p. 100)), mucous membranes involvement (7 cases (18 p. 100)), or minor haemorrhages (6 cases (15 p. 100)). The rash appeared macular, discrete, itching, troncular with peripheral extension, rather than maculo-papular (morbiliform) as usually described. Apart from cases which minor haemorrhagic changes, significatively associated with marked thrombocytopenia (medium 37 x 10(9)/1), dengue cases either with or without muco-cutaneous changes had similar clinical (duration, severity) or biological (neutro-lympho-thrombocytopenia, transaminases) features, and evolution. Histological changes appeared non specific (minor lymphocytic dermal vasculitis, non contributive immuno-fluorescence). DISCUSSION: Clinical and histological features of the rash are unspecific and inconstant: they do not allow an easy and accurate diagnosis. Complete clinical, epidemiological (very recent travel in endemic areas) or biological data should be collected, and early virological or later serological confirmation is needed. As well as travel facilities are growing, the dengue area is extending: dengue fever should therefore be considered in every traveller with fever and rash.

[Alternaria tenuissima plurifocal cutaneous infection]. / Infection cutanée pluri-focale à Alternaria tenuissima.

INTRODUCTION: Multiple skin localization of phaeohyphomycoses are rare and are encountered in immunodepressed subjects. We report a case due to Alternaria tenuissima in a patient given corticosteroids and IgA deficiency. CASE REPORT: A 73-year-old man presented with ulceronecrotic lesions on the two lower limbs which had developed over 15 days and poor general health. He had been taking corticosteroids for retroperitoneal fibrosis for 15 days. Skin biopsies demonstrated monoclonal dysglobulinaemia with IgG kappa and IgA deficiency. Itraconazol treatment was successful. DISCUSSION: Identification of Alternaria is in a skin sample, it not sufficient to determine its pathogenic nature. Strict criteria, especially histological criteria, are required. Most cases of cutaneous alternariosis are localized infections due to direct inoculation. They are seen in healthy patients or more often in immunodepressed subjects. Disseminated skin disease is rare and has been reported in 2 highly immunodepressed patients. Our patient was taking corticosteroids, but his deficiency state in IgA may have played a role in the development of the mycosis. Itraconazol appears to be effective treatment.

[Cutaneous Waldenström's macroglobulinemia]. / Localisations cutanées de la maladie de Waldenström.

BACKGROUND: We report the case of a patient in whom the first manifestation of Waldenström' s macroglobulinemia was specific skin lesions, treated with chlorambucil chemotherapy. CASE REPORT: A 76-years old woman was referred to us because of chronic red nodular lesions on her face. A biopsy specimen showed a dense lymphocytic dermal infiltrate and immunohistochemistry identified a monoclonal B lymphoid population with an IgM-kappa phenotype. The patient's disease was diagnosed as Waldenström's macroglobulinemia with cutaneous localization, on the basis of a high level of circulating macroglobulinemia and a lymphoplasmocytic infiltrate in the bone marrow expressing the same monoclonal IgM-kappa as in blood and skin. Treatment with radiotherapy (12 Grays) was unsuccessful. Chlorambucil (16 mg per day, 7 days per month) was then introduced with rapid disappearance of the skin lesions. Neutropenia led to withdrawal of this treatment after 4 courses. The skin lesions relapsed 18 months later and were cured with chlorambucil at a lower dose. DISCUSSION: Specific skin infiltrates have been rarely described during Waldenström's macroglobulinemia. Review of the literature showed eight cases of such lesions treated by chemotherapy with only two successes with oral cyclophosphamide and polychemotherapy (cyclophosphamide, vincristine and CCNU). Chlorambucil was used unsuccessfully three times. We hypothesize that primary resistance to alkylating-agent and the small number of cases of cutaneous Waldenström's macroglobulinemia may explain the poor response to systemic chemotherapy previously reported.

[Aseptic adenitis in pyoderma gangrenosum]. / Adénites aseptiques au cours d'un pyoderma gangrenosum.

INTRODUCTION: Extra-cutaneous manifestations of neutrophilic dermatosis are rare. Symptomatic cases are usually multiform and confusing. We report a case with an aseptic lymph node abscess associated with pyoderma gangrenosum. CASE REPORT: A 28-year-old woman with past history of pyoderma gangrenosum was seen for abdominal symptoms and fever related to an epigastric mass. Due to signs indicating abscess formation, surgery was performed and led to total regression of the symptomatology. Recurrence was evidenced 3 months later with skin lesions which were negative on bacteriological examinations. The diagnosis of lymph node manifestations of pyoderma gangrenosum was retained. General corticotherapy was very effective. Relapse occurred again 6 years later and was treated with thalidomide. DISCUSSION: Lymph node involvement has been described in Sneddon-Wilkinson's disease associated with pyoderma gangrenosum. In neutrophilic dermatosis, pulmonary manifestations appear to be the least exceptional of the extra-cutaneous lesions. The pathophysiology has not been elucidated although recent hypotheses suggest that G-CSF could be involved. Corticotherapy is remarkably effective in most cases but thalidomide could be an interesting alternative treatment.

[Pustular vasculitis disclosing Behçet disease]. / Vasculite pustuleuse révélatrice d'une maladie de Behçet.

INTRODUCTION: Pustular vasculitis is a classical manifestation of Behçet's disease. We report a case where the papulopustular eruption formed an arch on the skin and the histology examination showed predominant lymphocytic infiltration. CASE REPORT: A 29 year-old with a past history of recurrent buccal aphthosis had developed episodic papulopustular eruptions over the past 3 years. The diagnostic of Behçet's disease was made on the basis of an association with lymphocytic meningis, uveitis and erythema nodosum. General corticosteroid therapy was effective, but did not prevent skin relapse, controlled with colchicine. DISCUSSION: Pustular vasculitis usually presents with pustular eruptions on purpuric lesions and polynuclear infiltration of the dermis. Predominant lymphocytic infiltration is usually due to a pathergy phenomenon and would be specific for Behçet's disease. In our patient, colchicine was effective, although the mechanism remains to be explained.

[Malignant lymphoma in rheumatoid arthritis treated by low doses of methotrexate]. / Lymphome malin au cours d'une polyarthrite rhumatoïde traitée par de faibles doses de méthotrexate.

The authors report a case of non-Hodgkin's malignant lymphoma which developed in a patient under low-dose methotrexate for rheumatoid arthritis. Although rheumatoid arthritis without immunosuppressive therapy may be associated with an increased incidence of malignant lymphoma, the immunosuppressive effects of methotrexate may further promote the development of malignant lymphoma.