[Polyarthritis and papular eruption revealing leprosy]. / Polyarthrite et éruption papuleuse révélant une lèpre.

Leprosy is generally revealed by cutaneous lesions often associated to nerve impairment. Rarely, it may be revealed by polyarthritis. The diagnosis, often delayed in the cutaneous-nevritic form because of the low prevalence of the disease in metropolitan France, is very difficult in case of rheumatic presentation. We report the case of a 28 year-old woman from Mali, who was diagnosed with lepromatous borderline leprosy with reversal reaction occurring in the postpartum as she presented with polyarthritis and skin lesions.

[Extensive cutaneous candidiasis revealing cutaneous T-cell lymphoma: 2 cases]. / Candidose cutanée diffuse révélatrice de lymphome T cutané: 2 cas.

BACKGROUND: During the course of immunodeficiency diseases, severe candidiasis can occur with extensive cutaneous and mucous membrane lesions. However, blood dyscrasias are very rarely revealed by diffuse candidiasis. We report two case of cutaneous T-cell lymphoma revealed by extensive and atypical cutaneous candidiasis. PATIENTS AND METHODS: Case No. 1:A 72-year-old woman presented a pruritic rash of circinate, serpiginous patches on glabrous skin and skinfolds with multiple intertrigo and rapidly worsening palmoplantar keratoderma. All mycological skin specimens tested positive for Candida albicans. Histological examination of a biopsy sample from a serpiginous patch revealed the presence of fungal elements while palmoplantar keratoderma biopsy showed an epidermotropic lymphocytic infiltrate in the superficial dermis evocative of mycosis fungoides. Blood tests showed a white cell count of 28 600/mm3 with 14% circulating Sezary cells and a T-cell clone. The T-cell lymphoma was treated with methotrexate, but the disease worsened a few months later, progressing to CD30- large T-cell pleomorphic lymphoma. The patient died of severe sepsis. Case No 2:A 60-year-old man presented a macular rash over the face, trunk and skinfolds as well as erythematous scaly annular plaques of the glabrous skin with lymphadenopathy. Cultures of skin scrapings were all positive for Candida albicans. Blood tests showed a white cell count of 15 000/mm3 with 30% circulating Sezary cells. A trunk patch biopsy revealed the histological appearance of mycosis fungoides. There was a T-cell clone in the peripheral blood and skin. DISCUSSION: In both cases, the patients presented with widespread annular and erythematous scaly lesions of the glabrous skin and skinfolds with evidence of Candida albicans on fungal tests of all skin scrapings. The discovery of circulating Sezary cells on a systematic smear for hyperleukocytosis led us to suspect underlying cutaneous T-cell lymphoma, which was confirmed by biopsy of the skin lesions accompanying the mycoses. Widespread cutaneous candidiasis can occur in patients with cell-mediated immunodepression. Cutaneous T-cell lymphoma can enhance such candidiasis through interference with skin integrity and impairment of cell-mediated immunity, with large amounts of IL10 and TGF-B, increased secretion of soluble interleukin-2 receptors (CD25) and impaired CD8 suppressor cell function.

[Cutaneous leishmania in HIV patient in Ouagadougou: clinical and therapeutic aspects]. / Leishmaniose cutanée chez les malades infectés par le VIH. Aspects cliniques et thérapeutiques.

BACKGROUND: Immune suppression cause by HIV infection is a risk factor in the progression of leishmania diseases. In Burkina Faso atypical clinical presentations of leishmaniases have been observed among people living with HIV. The goal of this study was to describe clinical and evolutionary aspects of cutaneous leishmania and HIV co-infection among patients followed at Ouagadougou University Hospital. PATIENTS AND METHODS: This 16-month prospective study was carried out from January 2003 to April 2004 among HIV-seropositive patients with a diagnosed cutaneous leishmania infection. At baseline, infection and lesions were classified. Clinical diagnosis of cutaneous leishmania depended on finding parasites by microscopy in smears or tissue biopsies. Histological examinations were done if clinical and parasitological diagnosis were not concordant. Treatment consisted of three 21-day rounds of pentavalent antimonial, (Glucantime(R)). Clinical evolution was monitored at the end of each treatment round. RESULTS: Thirty-two HIV-1 positive patients (16 women and 16 men) were included. Mean age was 35.5 (10-67 years old). Leishmania lesions had been evolving, on average, for 12 weeks. Eleven patients were taking HAART and 21 patients were taking cotrimoxazole prophylaxis against opportunistic infections. Cutaneous lesions were found: in the face (15 cases), torso (18 cases), upperlimbs (26 cases) and lower-limbs (28 cases). Observed clinical forms were: papulo-nodular (9 cases), ulcerative (14 cases), infiltrative (12 cases), lepromatous and diffuse (15 cases), psoriasis-like (5 cases), cheloid, histioid or kaposi-like (1 case each). Some patients presented more than one clinical form. Prognosis was satisfactory in 24 patients after the first treatment. Twelve patients relapsed after the first treatment, among those 10 were only taking cotrimoxazole. At the end of the third treatment, 24 patients were cured, 3 died and 5 were lost to follow-up. CONCLUSION: Clinical polymorphism of cutaneous leishmania has been observed in HIV-patients, thereby increasing the risk of differential diagnosis.

[Feasibility of cervical smear in HIV-positive women living in Chad]. / Faisabilité du frottis cervico-utérin chez les femmes séropositives pour le VIH vivant au Tchad.

Cervical cancer is the leading cause of cancerrelated death in Sub-Saharan African women. HIV-infected women are at increased risk for cervical intraepithelial lesions and invasive cervical cancer. WHO guidelines for screening and treatment of precancerous cervical lesions are regularly actualized. There are no data on cervical squamous intraepithelial lesions in Chad. Between August 2013 and May 2015, screening for cervical squamous intraepithelial lesions was proposed to HIV-infected women living in Moundou (Chad). Cytology examination was performed after with Papanicolaou coloration. Three hundred and eleven HIV-seropositive women accepted the screening without refusal. Mean age of the patients was 38 years (95% Confidence Interval: 37.7-39.9). The women declared a mean of 4.1 pregnancies (range: 0-12). The patients had been followed-up for their seropositivity for 8 years (range: 0-25). All were on highly active antiretroviral therapy (HAART). Of the patients whose results were known (N = 231), 98% had a CD4 lymphocyte nadir count less than 350/mm(3). Cytological results were as follows: normal smear (N = 59; 19%), inflammatory or hemorrhagic smear (N = 139; 44%), low grade squamous intraepithelial lesion (N = 58; 19%), high grade squamous intraepithelial lesion (N = 28; 9%), epidermoid carcinoma (N = 13; 4%), and uninterpretable smear (N = 14; 5%). The inflammatory lesions were due to cervicitis (N = 54), vaginosis (N = 22), and trichomonas infection (N = 3). The patients' age, CD4 lymphocyte nadir count, and CD4 count at the time of the cervical smear were not different according to the cytological results. Only five patients had a cone biopsy. Three patients deceased during the study of whom two from a gynaecological cancer diagnosed too late. The screening of dysplasia and cervical cancer in HIV-seropositive women is possible in Chad. In our study, 13% of the women had highgrade dysplasia or carcinoma needing curative care. We also showed that simple cytology did not permit the interpretation of half of the smears. The performance of cervical smear would have increased if it had been preceded by the visualization of the cervix with coloration.

Occurrence of angioimmunoblastic T cell lymphoma in a patient with chronic myelomonocytic leukemia features.

In a patient with recently diagnosed chronic myelomonocytic leukemia features, the biopsy of a peripheral lymphadenopathy seven months later revealed disorganised lymphoid tissue with a few large EBER (+) LMP1 (+) B-lymphocytes before any treatment was given. At this time, a clonal TCR gamma rearrangement and very faint clonal IgH rearrangement were demonstrated, and the diagnosis of angioimmunoblastic T-cell lymphoma was made. Treatment with MOPP was started, followed by Hydroxycarbamide and CHOP but the outcome was fatal. During the evolution, there was no blastic transformation of the chronic myelomonocytic leukemia. The T-cell lymphoma extended to abdominal lymph nodes, Waldeyer ring and bone marrow and the percentage of large LMPI EBER (+) B-cells increased in the lymph nodes. These findings do not support a common stem cell abnormality leading to myelodysplasia in the bone marrow and lymphoma in peripheral lymph nodes. The lack of a clearcut light chain restriction in the EBV infected B-cell is suggestive of a persistant EBV infection in polyclonal or oligoclonal activated B-cells as described in immunodepressed patients. The association of CMML features and an angioimmunoblastic T-cell lymphoma is discussed.

Bone marrow one step fixation-decalcification in Lowy FMA solution: an immunohistological and in situ hybridization study.

The immunoreactivity of paraffin embedded bone marrow biopsies (BMB) was studied following a one step 20-hour-fixation-decalcification in Lowy formalin mercuric chlorid acid solution which permits excellent histological stainings. Antibodies reactive with myeloid, megakaryocytic, erythroid cells, T and B lymphocytes, mastocytes and metastatic cells were compared. Nearly all antibodies working on paraffin sections were demonstrated on Lowy FMA fixed BMB. Special care was taken to define an optimal working dilution. Trypsinization was not necessary. A slide microwave pre-treatment appeared essential before testing CD20 L26, CD8, CD3, CD34, MB1 Kappa and Lambda antibodies. It was suitable for UCHL1, LN2, CD30 antibodies. The same fixative allowed an m RNA Kappa or Lambda in myeloma and EBER 1 EBV RNAs in HIV lymphoma visualization by in situ hybridization. The safety handling of the toxic mercuric chloride component is discussed.

Lethal chondrodysplasia punctata, Conradi Hünermann subtype A, one case.

Lethal Conradi Hünermann disease is described in a patient with severe skin and skeletal involvement. Skin lesions, now considered as a peculiar form of ichthyosis, consisted of adnexal and pilar follicle hyperkeratosis, polymorphonuclear exocytosis, irregular keratohyaline distribution and frequent paranuclear vacuoles in stratum Malpighi. Numerous chondrocyte clusters and calcified masses were found in the epiphyseal cartilage. The growth plate was disorganised. There was a bilateral cataract. This severe lethal form of Conradi Hünermann disease corresponds to the subgroup A denomination of Spranger and may be a severe manifestation of X-linked Chondrodysplasia punctata.

Demonstration of T lymphocytes in leprous granuloma using the acid alpha naphtyl acetate esterase activity. An attempt at quantitative analysis.

In nine leprosy patients (1 TT, 1 BT, 4 BL and 3 LLp), esterase positive lymphocytes (T Lymphocytes) were studied in frozen sections of skin biopsies by alpha naphtyl acetate esterase pH 5.8 method (ANAE) Four patients had never been treated previously and five patients exhibited clinical and bacteriological evidence of relapse for inadequate therapy at first biopsy. There was an increase in ANAE (+) lymphocyte density in granulomas when second biopsies were done after efficient treatment, evaluated by bacillary index for the eight bacilliferous patients, and clinical improvement. The significance of T cells in granulomas is discussed.

Pleomorphic medium-sized T-cell lymphoma following Hodgkin's disease (nodular sclerosis type).

We describe a 32-year-old woman who presented with Hodgkin's disease, nodular sclerosis type II, subtype I, which necessitated several treatments over 11 years. The patient then developed pleomorphic, medium-sized T-cell lymphoma, which had a fatal outcome within 13 months. The role of radiotherapy, splenectomy, and chemotherapy in second tumor induction is compared with other sequential T-cell lymphomas. The significance of rare Epstein-Barr virus-infected cells during the T-cell lymphoma extension is discussed.

Peritoneal endometriosis. Report of a case with cytologic, cytochemical and histopathologic study.

The cytologic and histochemical data in a case of extensive peritoneal endometriosis are presented. The presence of macrophages heavily laden with blue and dark pigment (as demonstrated by May-Grünwald-Giemsa, Perls and Fontana stains) and scattered non-neoplastic endometrial cells in hemorrhagic ascitic fluid indicated a diagnosis of peritoneal endometriosis. Metabolized hemoglobin material was related to both recent and older hemorrhages.

[Atypical CADASIL phenotypes and pathological findings in two new French families]. / CADASIL: aspects phénotypiques inhabituels et observations anatomo-pathologiques dans deux nouvelles familles françaises.

Atypical phenotypes of CADASIL and corresponding anatomical data in two cases are described in 6 members of 2 new French families. In the first family, 4 cases in the same kindred were probably affected, two of them with a predominant psychiatric presentation, two others with dementia and a pseudo-bulbar syndrome of progressive evolution. No history of migraine or ischemic event were documented in any. In the propositus, the diagnosis was documented by skin biopsy, Notch 3 gene mutation and autopsy after the patient had died when 67 years old, 8 years after onset. Brain examination showed a widespread leukoencephalopathy with subcortical infarcts. Characteristic granular lesions of the small arteries of the brain and other organs were observed. In the second family, two cases are reported. One patient died when 63 years old after a subacute evolution mimicking intracranial hypertension. The anatomical diagnosis was retrospectively proven typical of CADASIL with Notch 3 immunostaining of arterial smooth muscle cells. The other case had a progressive evolution over 20 years of limb paresthesia with a mild spasticity diagnosed as a progressive form of multiple sclerosis. It was followed by a pseudo-bulbar syndrome and a mild subcortical dementia without acute ischemic attack. The diagnosis was confirmed by skin biopsy and mutation of the Notch 3 gene. This report illustrates

[Acute pancreatitis in rheumatoid purpura. Apropos of 2 cases]. / Pancréatite aiguë au cours d'un purpura rhumatoïde. A propos de deux cas.

Abdominal pain observed in Henoch-Schönlein purpura (HSP) is usually attributed to digestive tract involvement. Pancreatic involvement is a rare and benign complication. The authors report two cases of acute pancreatitis as a complication of HSP. Pancreatitis was confirmed in both cases by clinical presentation and increase of serum amylase levels. Abdominal echography has demonstrated ascites or alithiasic cholecystitis without pancreatic abnormality. The prognosis was favourable in each case. Pathophysiologic mechanism is presumably a vasculitis of the small vessels specially within the pancreas leading to inflammation. Abdominal pain can be explained by a digestive tract involvement but also by an acute pancreatitis. This later occurrence is not as exceptional as reported in the literature. Thus, serum amylase levels should be evaluated in patients with HSP who have intense epigastric or abdominal pain, in order to recognize a pancreatic involvement.

[The place of anatomo-pathological study in the diagnosis of enterocolitis complicated by Yersinia pseudo-tuberculosis]. / Place de l'examen anatomo-pathologique dans le diagnostic des entérocolites compliquées à Yersinia pseudo-tuberculosis.

Over a period of 3 months, 3 patients were operated on for subacute enterocolitis. The first case was a 3 years old boy with a transverse colonic perforation near the right angle and gross lymphadenopathy. The second case was a 21 years old man who had subacute terminal ileitis. Histopathological study showed specific lesions in those two cases: ovoïd or starry abscesses with preepithelioïd cells, rare giant Langhans cells, rare altered polynuclear cells in necrosis, massive lymphoïd hyperplasia. The third case was a 27 years woman with mild appendicitis showing masses of bacteria in the wall of appendix, surrounded by lymphocytes without polynuclear. Serological study showed anti YPST agglutinins significantly modified in all 3 cases. In the absence of bacteriological study, the role of pathological findings is discussed.

[Malignant intravascular lymphoma (malignant angioendotheliomatosis), with adrenal localization. Report of a case]. / Lymphome malin intravasculaire (angioendotheliomatose maligne), avec localisation surrénale. A propos d'un cas.

A past history of epilepsy and terminal dementia in a fifty five year old patient lead to the post mortem diagnosis of malignant intravascular lymphoma (malignant angioendotheliomatosis). There was a scattered involvement of capillaries and venulae in leptomeninges, peripancreatic and perirenal adipose tissue, pericardium, myocardium. The only adrenal involvement was typically tumoral at macroscopic examination. Such a localization, often reported in B intravascular malignant lymphoma, is studied according to the recent publications of this entity.

[Contribution of immunocytochemical marking of benign cells to the diagnosis of serous effusions]. / Apport du marquage immunocytochimique des cellules bénignes au diagnostic des épanchements des séreuses.

In pleural and peritoneal effusions, the distinction between mesothelial cells and histiocytic cells is fairly often difficult. This distinction is specially important in malignant effusions, where such reactive cells can be confused with tumoral ones. In order to increase the accuracy of cytological diagnosis, the immunocytochemical reactivity with 13 antibodies was studied. Four antibodies (vimentin, lysozyme, monoclonal keratin, OKM1) realise a panel specially useful for the characterization of mesothelial and histiocytic cells in most cases of benign or malignant effusions.

[Lymphadenopathy of dermatosis in the course of drug hypersensitivity. Cytologic, immunohisto- and immunocytologic, ultrastructural aspects. Report of a case]. / Lymphadénopathie des dermatoses au cours d'une hypersensibilité médicamenteuse. Aspect cytologique, immunohisto et immunocytologique, ultrastructural. A propos d'une observation.

In addition to the morphological details obtained from the imprints, a simple immunocytological study allowed us to diagnose one case of a dermopathic lymphadenopathy simulating a T cell lymphoma, following a drug-induced erythrodermia. We were able to identify the increase of CD1a+ and Prot. S100+ cells on acetone fixed imprints. The histological, immunohistological and ultrastructural investigations confirmed the value of the cytological study and that the dendritic cells were Langerhans cells (Birbeck granules+). Most of them were considered as migrating from the dermal lesions.

[Myocardial infarction caused by thrombosis of the left coronary artery in a patient with thrombocythemia]. / Infarctus du myocarde par thrombose du tronc commun de la coronaire gauche chez un patient thrombocytémique.

Myocardial infarction is not exceptional in patients with essential thrombocythaemia. This infarction is often related to the formation of in situ coronary thrombosis with no associated atheromatous lesions. The authors report the case of a thrombocythaemic patient with anterior infarction due to thrombosis of the left coronary artery. The clinical course is often more severe than in nonthrombocythaemic patients. The pathophysiological mechanisms remain unclear, but appear to be related to qualitative rather than quantitative platelet abnormalities.

[A case of bone lipoma of the femur head]. / Un cas de lipome intra-osseux de l'extrémité supérieure du fémur.

A case of intra-osseous lipoma of the upper femur is reported. A review of the literature shows that this lesion is rare, only about 33 cases having been published. The radiological appearance is that of a fibroma. The diagnosis is made at the time of surgery which reveals the presence of fat in a large cavity. Curettage and bone graft resulted in healing.