[Cutaneous leishmaniasis due to Leishmania major involving the bone marrow in an AIDS patient in Burkina Faso]. / Leishmaniose cutanée à Leishmania major avec atteinte de la moelle osseuse chez un malade infecté par le VIH au Burkina Faso.

BACKGROUND: Leishmaniasis covers three well-individualized clinical variants, each due to individual species found in different geographic areas. Herein we report the first case of cutaneous leishmaniasis due to Leishmania major involving bone marrow in an AIDS patient in Burkina Faso. CASE REPORT: A 38-year-old HIV-positive man presented with generalized, copper-coloured, painless, infiltrated, itching, papulonodular lesions present over the previous 10 months. Skin biopsy confirmed the diagnosis of diffuse cutaneous leishmaniasis. The bone-marrow smear showed numerous leishmania. The culture was positive and L. major was identified. The patient was being treated with antiretroviral medication and a pentavalent antimonial compound. The disease progression consisted of attacks and remissions separated by an average of three weeks. DISCUSSION: L. major is the Leishmania species identified in Burkina Faso. It is responsible for typical cutaneous leishmaniasis but particular clinical forms have been described in immunodeficient patients, especially with diffuse cutaneous involvement. The spread of L. major infection to bone marrow could represent a public health problem in our country, where the HIV epidemic is still not under control, and particular vigilance is thus called for.

Detection of Chlamydia trachomatis in symptomatic and asymptomatic populations with urogenital specimens by AMP CT (Gen-probe incorporated) compared to others commercially available amplification assays.

The present study was designed to evaluate the sensitivity and specificity of AMP CT (Gen-Probe Incorporated, San Diego, CA, USA) on urogenital specimens taken from symptomatic patients and on first void urine (FVU) specimens from asymptomatic patients. In symptomatic patients, 618 specimens from 140 men (140 urethral swabs and 140 FVU) and 202 women (202 endocervical swabs and 136 FVU) were tested by using cell culture, AMP CT and Amplicor Chlamydia trachomatis MWP (microwell plate) (Roche Diagnostics, Somerville, NJ, USA) on genital samples, and AMP CT and Amplicor on FVU. A clinical specimen was considered to be truly positive if either the cell culture was positive and/or both AMP CT and Amplicor were positive. In the asymptomatic population, a total of 300 FVU (136 women and 164 men) were tested by four amplification methods, AMP CT, LCx (Abbott, Abbott Park, IL, USA), Amplicor MWP, and Cobas Amplicor. A subject was considered to be infected when two or more amplification methods were positive. In the symptomatic population (prevalence 13%), concordant results were observed in 320/342 cases (93.5%). After analysis of discordant results, the sensitivity of AMP CT, Amplicor, and culture was 100%, 95.5%, 68.8%, respectively, and the specificity was 98.3%, 99.3%, 100% respectively. The number of false negative results by AMP CT in urine, probably due to labile inhibitors, was 3/276 (1%). In the asymptomatic population, the results were concordant in 298/300 (99.3%), seven positive and 291 negative. Two results were considered false positives, one by Cobas Amplicor, one by AMP CT. Compared to other amplification methods, AMP CT is at least as sensitive for the identification of chlamydial infection in symptomatic and asymptomatic men and women on genital or urine specimens.

Bowenoid papulosis. Demonstration of human papillomavirus (HPV) with anti-HPV immune serum.

Two cases of bowenoid papulosis (BP) occurred. The presence of human papillomavirus was demonstrated in one patient. In the other patient, condyloma acuminatum developed after initial spontaneous improvement in his lesions. The discovery of a possible oncogenic virus in BP and the epidemiologic behavior of carcinoma in situ and multicentric carcinomas suggest the need for a very careful follow-up evaluation of such lesions. Bowenoid papulosis may be a chronic dysplasia with a variable long-term prognosis linked to a cocarcinogenic factor.

[Factors associated with survival in Kaposi's sarcoma in patients infected with the human immunodeficiency virus. Clinical Epidemiological Group for AIDS in Aquitaine]. / Facteurs associés à la survenue de la maladie de Kaposi chez les patients infectés par le virus de l'immunodéficience humaine. Groupe d'Epidémiologie Clinique du SIDA en Aquitaine (GECSA).

BACKGROUND: To study behavioral risk factors of Kaposi's sarcoma (KS) among HIV infected homosexuals in Bordeaux, southwest France. METHODS: A case-control study was performed within the Aquitaine Cohort. Cases of KS surviving in 1995 and homosexuals were systematically enrolled. For each case, two controls were selected among homosexuals surviving in the cohort. Cases and controls were matched on year of diagnosis of HIV infection. Data collection was based on a self administered questionnaire focusing on use of recreational drugs, detailed sexual practices and sexually transmitted diseases in the year preceeding the diagnosis of HIV infection, in the year after the HIV diagnosis and in the year preceeding the diagnosis of KS (or an equivalent period of time for controls). RESULTS: Twelve cases were matched to 2 controls, 15 cases to one control and 13 cases remained unmatched. Matched analysis identified an association between KS and regular sexual partner (odds ratio = 0.07; 95% confidence interval: 0.01-0.52 and p < 0.001) and active and passive oro-anal intercourse before HIV diagnosis and before KS diagnosis (p = 0.01). In the unmatched analysis including all cases, we found an association between KS and the overall number of sexual partners (p < 0.03) for all periods of interest. CONCLUSIONS: This case-control study identified sexual practices in favor of a sexually transmitted agent of KS.

[Diverse aspects of congenital syphilis]. / Les divers aspects de la syphilis congénitale.

Together with the new rise of recent syphilis in the adult woman, congenital syphilis reappears sporadically in our countries, where prophylactic measures (prenatal serology) are sometimes defeated. It remains much more frequent in developing countries. One should know how to interpret a positive serology in a newborn, as it may only reflect passive transplacental transfer of maternal antibodies. One should on the other hand think of syphilis again when the clinical signs are more or less suggestive, the typical picture not being, by far, the most frequent. In spite of the efficacy of Penicillin G: 50,000 U/kg/day during 10 days, neonatal death rate for congenital syphilis is in the neighbourhood of 10%. Our major effort should therefore be aimed at prevention.

[Cerebral lesions in a case of Degos' disease]. / Lésions cérébrales dans un cas de maladie de Degos.

The central nervous system may be involved in Degos disease, but neuropathologic observations are still few. We report a typical case with cerebral involvement. The diagnosis was suggested by the characteristic skin lesions and vascular damage. In the central nervous system, occlusive changes of small arteries were responsible for areas of ischaemic necrosis. These foci of infarction were of varying size, age and distribution. Intestinal lesions were discrete and death was due to several cerebral infarcts.

[POEMS syndrome revealed by a scleroderma-like skin thickening]. / Syndrome POEMS révélé par un aspect de sclérodermie.

BACKGROUND: POEMS syndrome is a rare form of plasma-cell dyscrasia characterized by the various association of Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal component and Skin changes. The most frequent skin changes such as hyperpigmentation, hypertrichosis, scleroderma-like skin thickening and angiomas are not pathognomonic but nearly constant. However, they are rarely isolated. CASE REPORT: A 57-year-old Caucasian woman presented with a 6 month history of skin thickening on both hands and feet, Raynaud's phenomenon and facial telangiectasias. Physical examination on presentation revealed hepatomegaly, signs of a sensorimotor peripheral neuropathy which was demyelinating in type on electrophysiological assessment, and ankle edemas. Initial laboratory investigations revealed a platelet count of 900 000/mm(3), a monoclonal IgG lambda gammapathy. Plasma-cells were slightly increased (10 p. 100 of marrow elements) and full skeletal radiographs showed no focal osteosclerotic or lytic lesion. A diagnosis of POEMS syndrome was made. The patient was treated with tamoxifen, methylprednisolone and plasmapheresis without improvement in polyneuropathy or in skin changes. DISCUSSION: Our patient satisfied the criteria for POEMS syndrome. The most typical feature here was the scleroderma-like skin change, which has been recognized by other authors. But, in the present case, Raynaud's phenomenon, skin thickening and facial telangiectasias were present 6 months before the diagnosis, and initially suggestive of systemic scleroderma, confirmed histologically. The pathogenesis of POEMS syndrome might be regarded as the result of a marked activation of the proinflammatory cytokine network, but an increase in serum Vascular Endothelial Growth Factor (VEGF) levels could well account for other manifestations such as skin thickening.

[Cutaneous manifestations of erysipeloid septicemia]. / Manifestations cutanées d'une maladie du rouget du porc septicémique.

BACKGROUND: Rouget du porc, or swine erysipelas, usually occurs in man as Rosenbach's erysipeloid. Septicemic forms are more uncommon and can be associated with dermal involvement far from the site of inoculation. We report a case in a patient given corticosteroid therapy for systematic lupus. CASE REPORT: A 50-year-old farmer was seen with fever, infiltrative erythema of the long finger and dorsal lesions on the ring finger which developed after a skin lesions caused by a duck. The diagnosis of septicemic rouget du porc was made after isolating the germ from blood cultures. There was no associated endocarditis. Fever and skin lesions totally regressed after treatment with ceftriaxone. DISCUSSION: The diagnosis of erysipeloid was supported by epidemiologic arguments and characteristic clinical features. The corticosteroid therapy was probably a favoring factor for development of septicemia. Positive diagnosis is usually obtained from blood culture but the germ can be isolated from skin biopsies at the site of inoculation. Our patient was free of endocarditis which should always be suspected. Endocarditis is frequent and often fatal. Intravenous high-dose penicillin G is recommanded treatment.

[Dermatitis caused by estrogens]. / Dermatite aux oestrogènes.

BACKGROUND: We report a case of sensitization to estrogen. CASE REPORT: A 40-year-old woman consulted for skin disorders which followed a cyclic pattern. At each menses, the patient developed pruritus and erythematous papulovesicular lesions over the members and trunk. Estraderm patch contact dermatitis was evident. Prick and patch tests with alcoholic solutions of estrone alone were positive. Serum tests were positive for anti-ethinyl-estradiol antibodies and anti-progesterone antibodies. DISCUSSION: Autoimmune dermatitis can be caused by sensitization to endogenous or exogenous sex hormones. Clinical manifestations and histological findings are variable and non-specific. The cyclic nature of the manifestations is however quite suggestive. Positive prick and patch tests performed with alcohol solutions of the hormones may give the diagnosis and serum tests may be positive for specific anti-steroid antibodies. These complementary explorations are however difficult to perform and interpret and definitive diagnosis is based on an association of clinical findings, skin tests, laboratory tests and the clinical course. In case of progesterone sensitization, the treatment of choice is estrogen inhibition of ovulation. For estrogen sensitization, anti-estrogen treatment appears to be more effective. Finally, bilateral ovariectomy may be required in difficult cases.

[Melanomatous meningitis. Apropos of 3 cases]. / La méningite mélanomateuse. A propos de trois observations.

UNLABELLED: The meninges are frequently involved during the dissemination of malignant melanoma. This "meningeal melanoma" ranks fairly high on the list of metastatic meningites, side by side with meningeal carcinomatosis proper (i.e. related to a malignant epithelial tumour). Meningeal melanoma may be associated with a cerebral metastasis or isolated, as in the three cases reported here. Although its prognosis is sombre, its diagnosis is important since the possibilities of treatment are limited but exist. CASE-REPORTS: Case 1. This was a 68-year old woman who had initially presented with malignant melanoma in the maxillary region (SSM level IV, thickness 2.9 mm). Two years after the primary tumor was excised, secondary lesions developed in the lymph nodes and bones. A few weeks later, the patient fell into mental confusion progressing towards delusion of persecution. Neurological examination and CT scans were normal, but numerous melanoma cells were found in the CSF. This woman died one month after the first neurological signs had appeared. Case 2. This 63-year old man presented with bilateral axillary adenopathy and inflammatory thoracic plaques which at histology had proved to be metastases from a malignant melanoma of the shoulder surgically treated 10 years previously. After 14 months of almost complete remission under multiple chemotherapy (CPDD, ACTD, VDS), headaches and lumbosacral pain developed. Standard radiography and CT of the spine gave normal results, and it was only at the third lumbar puncture that the CSF was found to contain malignant cells. The patient died 2 months after the first neurological manifestations. Case 3. A 42-year old woman developed headaches and dysesthaesia in the arms and head. This was followed by oppositional semi-mutism. All exploratory examinations were normal, except for that of the CSF which showed 18 cells/mm3, 50 p. 100 of which were melanoma cells. The patient was transferred to the Dermatology department where he underwent excision of a left scapular melanoma difficult to classify (malignant blue naevus?). Combined treatment with Fotemustine* and DTIC produced an incomplete but relatively prolonged response. Death occurred after 8 months. DISCUSSION: A. Meningeal melanoma accounts for about 10 p. 100 of all metastatic meningites. The most frequent primary tumours in meningeal carcinomas are breast cancer and lung cancer. Malignant melanoma ranks third or fourth, but when the relative frequency of each of these three malignant diseases is taken into account, it appears that the risk of malignant melanoma is higher than that of any other solid tumour. B. In 410 cases extracted from the literature, the meninges were involved in 30 p. 100 of malignant metastatic melanomas found at autopsy. There is no publication that makes it possible to evaluate the relative frequency of melanomas affecting only the meninges or coexisting with cerebral metastases. This also applies to meningeal melanoma associated with non-neuro

[Isolated mucosal ulcers disclosing idiopathic hypereosinophilic syndrome]. / Ulcérations muqueuses isolées révélatrices d'un syndrome hyperéosinophilique idiopathique.

INTRODUCTION: Idiopathic hypereosinophilic syndrome is an uncommon disease often associated with diverse non-specific skin manifestations. Mucosal ulcerations suggest a myeloproliferative from with poor prognosis due to possible progression to malignant hemopathy or visceral complications. CASE REPORT: A 28-year-old man presented idiopathic hypereosinophilia with isolated mucosal ulcerations involving the buccal and genital areas. Laboratory results (hematology, CD25) suggested a myeloproliferative form. Treatment with alpha interferon (18 months) led to regression of the mucosal lesions and a decrease in the markers of eosinophil toxicity. There was no visceral involvement. DISCUSSION: Immunosuppression with/without high-dose alpha interferon is usually used for the treatment of hypereosinophilic syndrome. In our case favorable outcome was obtained with lower doses of alpha interferon than those reported in the literature. There was objective decrease in eosinophil toxicity (regular counts of hypodense eosinophils, CD25 or interleukin 2 soluble receptor) and no progression (malignant hemopathy, mortal visceral involvement).

[Chronic urticaria and toxocara canis infection. A case-control study]. / Urticaire chronique et Toxocara canis. Etude cas-témoins.

INTRODUCTION: The cause of chronic urticaria remains unknown very often. Having noted several cases of chronic urticaria associated with antibodies to Toxocara canis, and lacking any other explanation, we set-up a case-control study. PATIENTS AND METHODS: Between November 1992 and April 1993, 51 adults or children with chronic urticaria (cases) who had been examined at least once at one of the three dermatology units of the Bordeaux University Hospital were matched to controls who had neither signs nor symptoms of chronic urticaria. The presence of antibodies to T. canis was measured by ELISA and Western blot. RESULTS: The frequency of T. canis was 64.7 p. 100 in cases and 21 p. 100 in controls (p < 0.0001) with an odds ratio of 6.9 (95 p. 100 CI: 2.9-16.3). Cases with antibodies to T. canis were more frequently in contact with pets (84 vs 50 p. 100, p < 0.001). Of the 33 cases of chronic urticaria with antibodies to T. canis, 14 have been treated with thiabendazole or ivermectin and after a one-year follow-up, 5 (36 p. 100) were cured and 4 (29 p. 100) had improvement. No improvement occurred in the 12/19 cases not specifically treated. CONCLUSION: The strong association between the presence of antibodies to Toxocara canis and chronic urticaria is unlikely to be due to chance. A causal relation is difficult to establish, however. Our findings should prompt further investigation of a role for Toxocara canis in chronic urticaria and the evaluation of therapeutic interventions. Preventive measures include deworming pets (dogs particularly), enclosing kitchen gardens, and handwashing before meals. A systematic measure of Toxocara canis in patients with chronic urticaria is recommended especially when in contact with dogs. Early and specific treatments can be applied on knowledge we already have.

[Cutaneous metastases in digestive cancers (author's transl)]. / Métastases cutanées des cancers digestifs.

Cutaneous metastases are seldom encountered in digestive cancers. The mechanism of their formation explains their low rate. The metastases spread mainly by the lymphatic system (especially in diffuse, spotty or pseudo-elephantiasic forms and in regional forms), however numerous lymph node filters found through out the lymphatic system limit the progression of neoplasic cells. On the contrary, metastases most often spread by retrograde involvement due to blocking of the lymphatic system. As for hematogenous metastases (generalized nodular forms or aberrant localizations), they are observed even less frequently than lymphatic ones; metastatic embolisms may effectively become blocked in capillaries, especially in the lungs and the liver, which constitutes a double barrier before the skin may be involved.

[Leprosy disclosed by polyarthritis]. / Lèpre révélée par une polyarthrite.

BACKGROUND: We report a case of leprosy observed in a French woman who had lived in Africa 30 years earlier. The clinical presentation was misleading, suggesting connective tissue disease. CASE REPORT: A 69-year-old woman was hospitalized in April 1996 for inflammatory joint disease. The first manifestations had developed three years earlier and the patient had been on systemic corticosteroid therapy associated with anti-malarials since 1993. The clinical presentation progressively included neurological and skin manifestations. Histology examination gave the diagnosis of lepromatous leprosy. Three-drug anti-leprosy treatment in one oral dose was initiated. DISCUSSION: Chronic Mycobacterium leprae infection usually leads to overt leprosy with neurological and cutaneous involvement. Rheumatological forms are less common and found almost exclusively during leprous reactions. The association of inflammatory join pain with neurological and skin manifestations wrongly suggested vasculitis. In addition, the general corticosteroid therapy certainly was implicated in disease activation and progression to a purely lepromatous form.

[Melanoma of soft tissues]. / Mélanome des tissus mous.

INTRODUCTION: Soft tissue melanoma was described in 1965 by Enzinger who used the term clear-cell sarcoma. In 1983, Chung and Enzinger coined the term soft tissue melanoma due to the immunohistochemical similarity with melanoma. We report a case of this rare type of melanoma. CASE REPORT: A 59-year-old woman had pain between the first two toes for 3 years. A subcutaneous tumor was found at examination. Histologically, the tumor was composed of weakly eosinophilic cell proliferation. Protein S100 and HMB45 were positive. The cells were organized in theques. Pathology diagnosis was soft tissue melanoma. Complete remission was obtained for 3 years when several local recurrences required surgery and chemotherapy then surgery and radiotherapy. Complete remission has been achieved for 9 months. DISCUSSION: This case presented the main characteristics of soft tissue melanoma as described in a review of 209 analyzable cases reported in the literature. This tumor occurs in young subjects with no sex or race predominance. It is an ubiquitous tumor which develops in close relation with tendons and aponevroses, usually in limbs (especially feet). Pain is sometimes the revealing manifestation, but the tumor is often asymptomatic, so the volume is often important at diagnosis. Pathology examination shows rather monomorphic proliferation of cells with a clear or weakly eosinophilic cytoplasm grouped in clusters or theques separated by fibrous septa. Intracytoplasmic melanin is sometimes observed, indicating interest of protein S100 and HMB45 immunohistochemistry which is almost always positive. The principle differential diagnoses are metastasic melanoma and epithelioid sarcoma. Prognosis of soft tissue melanoma is similar to that in sarcomas with a high rate of local recurrence and metastases (lymph nodes, lungs). Mortality reaches 56 p. 100. Treatment is wide surgical exeresis. CONCLUSION: Soft tissue melanoma is a rare tumor of the melanocyte. It differs from melanoma by the population involved, its clinical expression and its prognosis which is similar to that in sarcoma.

[Post-radiotherapy cutaneous neuro-endocrine carcinoma]. / Carcinome neuro-endocrine cutané en zone irradiée.

INTRODUCTION: Merkel cell carcinoma or cutaneous neuroendocrine carcinoma is an uncommon severe disease. The carcinogenic effect of ionizing radiations has been suspected in exceptional observations. We report the sixth case of Merkel cell neuroendocrine carcinoma in a patient with prior radiotherapy. CASE REPORT: An 86-year-old man underwent radiotherapy for a basal cell carcinoma of the tip of the nose and developed a highly aggressive Merkel cell carcinoma at the same location 6 years later. DISCUSSION: The development of Merkel cell carcinoma on irradiated tissue accounts for 2.6 p. 100 of the 227 publications where dermatological history was reported. This percentage may be underestimated. The similar localizations of the irradiated zone and the site of cancer development 5 years later suggest that the Merkell cell carcinoma may be a radio-induced tumor. The delay may vary from 5 to 47 years. The similarity of the carcinogenic factors involved in Merkel cell carcinoma and squamous cell or basal cell carcinomas (ultraviolet, ionizing irradiation) and the frequent association of different types favor an epidermal origin for Merkel cell carcinoma. In clinical practice, past history of radiotherapy in an area where Merkell cell carcinoma develops indicates that therapeutic management must exclude post-operative radiotherapy.

[Langerhans-cell histiocytosis and Erdheim-Chester disease: probably not a fortuitous association]. / Histiocytose langerhansienne et maladie d'Erdheim-Chester: une association probablement non fortuite.

BACKGROUND: Erdheim Chester disease (MEC) is a rare non-Langerhans cell histiocytosis characterized by multi-visceral involvement. We report a case of MEC associated with Langerhans cell histiocytosis (HCL). CASE REPORT: A 46-year-old women presented skin and vulvar localization of HCL associated with typical MEC bone involvement. Despite chemotherapy (vinblastine) and prednisone, the disease progressed to involve the central nervous system, leading to fatal outcome. Post-mortem examination showed HCL in skin, MEC in bones and central nervous system, and intermediate histiocytic proliferation in the encephalon. DISCUSSION: Usually, MEC and HCL are considered as distinct entities. MEC is characterized by a xanthogranulomatous proliferation of CD 68+ foamy histiocytes nested in fibrosis, and HCL by a proliferation of PS 100+ and CD1a+ Langerhans cells. However, our observation, as well as previous reports, suggests that MEC is part of the HCL spectrum.

[Genital herpes]. / Herpès génital.

Herpes is the first cause of genital ulcers, recurrences are unpredictable and asymptomatic viral excretion is frequent. Herpes simplex virus 2 is responsible for genital infection in 80% of cases, but the incidence of Herpes simplex virus 1 is rising. New diagnostics methods are developing, batcellular viral culture remains gold standard. Oral aciclovir is the standard treatment, and directions for use are strictly codified. In pregnant women a new preventing project is proposed, it does not prevent transmission during asymptomatic viral excretion. In immunocompromise patients Herpes is frequent severe and sometimes aciclovir resistant.

[Lepromatous leprosy after BCG vaccination]. / Lepra lepromatosa revelada por la vacuna BCG

The authors describe an eruption of cutaneons nodules after a BCG vaccination. Investigation demonstrated that the lesions were caused by Lepromata. An increased number of B lymphocytes was observed in the bloodstream. Ryphampycin was used with good results.