Scabietic vasculitis: Report of 2 cases.

BACKGROUND: The infectious causes of cutaneous vasculitis are well known and include streptococcal infections among others. Cases resulting from parasitic infection are less frequent. Scabies, which is currently on the increase, has only been reported in a few isolated cases. Herein, we report two noteworthy cases of profuse scabies complicated by cutaneous vasculitis. PATIENTS AND METHODS: Case 1: a 90-year-old woman, residing in a nursing home, was admitted to our dermatology department complaining of pruritus, present for one month, predominantly on the inside of the thighs and on the buttocks, associated with purpuric lesions on the lower limbs. A skin biopsy revealed leukocytoclastic vasculitis. A diagnosis of scabies was based on severe pruritus and hypereosinophilia and was confirmed by microscopic examination of the parasitology sample and the skin biopsy sample. Despite thorough investigation, no other cause of vasculitis could be found. Complete regression of the skin lesions was achieved with scabies treatment only, without any specific treatment for the vasculitis. Case 2: a 74-year-old man, living in a nursing home, was hospitalized for purpuric papules on the lower limbs, present for one month. Physical examination revealed linear patterns in the interdigital spaces associated with scabies evident on dermoscopic examination. The skin biopsy revealed signs of vasculitis. As in our first case, no aetiology of vasculitis was found and a favorable outcome was achieved by means of scabies treatment alone with no specific treatment for vasculitis. DISCUSSION: Both of our patients presented scabies and vasculitis. In view of the absence of other causes of vasculitis and of the complete regression of lesions due to vasculitis without recurrence achieved with the scabies treatment alone, a diagnosis was made of scabietic vasculitis, probably as a result of cutaneous hypersensitivity reaction to humeral mediators.

[Plaque-like leiomyomas on a chemical burn: First case report]. / Léiomyomes en plaque sur brûlure chimique : première observation.

BACKGROUND: Cutaneous leiomyomas are uncommon benign smooth muscle neoplasms of skin of unknown pathogenesis. We report a sporadic case of multiple cutaneous leiomyomas at the site of a chemical burn. OBSERVATION: A 47-year-old male presented with multiple grouped red nodules on the right calf painful to cold and to touch. The lesions were located on the site of a chemical wound that had occurred 5 years earlier. Histopathological examination revealed a proliferation of smooth muscle fibres, leading to diagnosis of leiomyoma. DISCUSSION: To our knowledge, the literature contains no previous reports of sporadic multiple cutaneous leiomyomas occurring at a chemical burn site. While a chance association cannot be ruled out, there is also the possibility of a physiopathological mechanism similar to that of vascular tumours following exposure to 4-hydrazinobenzoic acid. This compound, a hydrazine derivative of the fungus Agaricus bisporus, can cause smooth-muscle tumours in the aorta and large vessels with morphological characteristics similar to those seen in vascular leiomyomas.

[Intranodal palisaded myofibroblastome: A rare cause of inguinal lymphadenopathy]. / Le myofibroblastome palissadique intraganglionnaire : une cause rare d'adénopathie inguinale.

INTRODUCTION: Lymphadenopathies are a major cause of consultation in internal medicine, with various causes of diagnosis. Unexplained persistent lymphadenopathy must be biopsied to rule out malignant tumor. CASE REPORT: We report the case of a 53-year-old man, with inguinal lymphadenopathy evolving for more than one year. The patient had no associated symptoms and his blood tests were unremarkable. Due to the progression of the adenopathy and its hypermetabolism on PET-CT, an excisional biopsy was performed. Histological analysis revealed an intranodal proliferation of spindle cells with a palisading pattern. ß-catenine and smooth muscle actin labelling were positive, leading to the diagnosis of intranodal palisaded myofibroblastoma, a benign tumour. CONCLUSION: Intranodal palisaded myofibroblastoma is a rare benign cause of adenopathy, with often inguinal lymph node localization and slow growth and without risk of recurrence after surgical removal.

[Papular mycosis fungoides]. / Mycosis fongoïde papuleux.

BACKGROUND: Mycosis fungoides (MF) is the most common type of cutaneous T-cell lymphoma. A new form of incipient MF has recently been described: papular MF. Herein, we report a case and propose a literature review. PATIENTS AND METHODS: A 63-year-old man presented with erythematous and non-pruritic papular lesions of the trunk. The general examination was unremarkable. A skin biopsy showed moderately dense epidermotropic lymphocytic infiltration consistent with MF. Screening for CD30 was negative. Treatment with an extremely potent corticosteroid (clobetasol, one application per day) seemed effective, with almost complete disappearance of the lesions. DISCUSSION: Many clinical variants of the initial stages of MF have been described, one of the most recent of which is papular mycosis fungoides (PMF), of which 10 cases are reported in the literature. PMF begins clinically with an erythematous, non-pruritic and chronic papular rash that is not associated with the classic erythematous-squamous lesions of incipient MF. There appears to be no predominance of gender, and the age of onset ranges from 31 to 63 years. Histological examination of the PMF lesions revealed an epidermotropic subepidermal infiltrate composed predominantly of CD4+T-cells. The prognosis appeared good with the treatments conventionally used for incipient MF. PMF is likened to a form of incipient MF with a good prognosis. Associated classic MF lesions comprising erythematous-squamous plaques have been described as the condition progresses. Differential diagnoses include pilotropic MF, pityriasis lichenoides chronica, pityriasis lichenoides varioliformis acuta, and especially type B lymphomatoid papulosis, the histopathological findings of which may be close to PMF. CONCLUSION: Papular MF would appear to be a papular variant of incipient MF with a good prognosis. However, it is necessary to obtain clinical and disease progression data for a greater number of patients in order to better characterize this entity.

[Pericarditis: a giant cell arteritis manifestation]. / La péricardite: une manifestation de la maladie de Horton.

INTRODUCTION: Temporal arteritis is a vasculitis in which inflammatory manifestations mainly involve the external carotid artery area but not exclusively. Through a clinical observation and a review of the literature, we suggest that inflammatory pericarditis could represent a manifestation of temporal arteritis. EXEGESIS: A 75-year-old-woman was admitted for progressive physical deterioration which had been evolving for three months, associated with fever, frontotemporal cephalalgia and severe biological inflammatory syndrome. Chest X-ray reveals a cardiomegaly and suggests a pericarditis, which was rapidly confirmed by echocardiogram. Temporal artery biopsy concludes to the diagnosis of a giant cell arteritis. Steroids treatment is prescribed, leading to a rapid regression of the inflammatory state and the pericarditis without relapse after 6 months of follow-up. CONCLUSION: Only prospective studies on systematic echocardiography when faced with the diagnosis of giant cell arteritis, whatever clinical symptoms, will enable to appreciate the prevalence and prognosis value of this manifestation. Moreover, temporal artery analysis seems to be justified when faced with a sub-acute or chronic "idiopathic" inflammatory pericarditis occurring in the elderly patient. Physiopathogeny is unknown but some hypothesis can be proposed: inflammatory cytokines storm, immune complexes deposition, giant cell vasculitis of pericardial arteries or inflammatory interstitial lesion of the pericardium with or without granuloma.

[Malignant cutaneous adnexal neoplasms of the face and scalp: Diagnostic and therapeutic update]. / Les tumeurs cutanées malignes à différentiation pilaire de la face et du cuir chevelu : mise au point diagnostique et thérapeutique.

INTRODUCTION: Malignant cutaneous adnexal neoplasms are rare and have been characterized only recently. They can occur at any age but preferentially in elderly. There are 3 of them: trichoblastic carcinoma, trichilemmal carcinoma and malignant pilomatricoma. The aim of our study was to make a diagnostic and therapeutic update about these tumors when located at the face or at the scalp. MATERIALS AND METHODS: A bibliographic research was made on PubMed using following keywords: appendage skin carcinoma AND pathology AND/OR therapeutic. Articles published before 2000 were considered outdated and were excluded. RESULTS: Twenty-five articles met the inclusion criteria. Clinical presentation was non-specific. Histological examination only allowed for diagnosis. Lesions were locally or loco-regionally aggressive. Lymphatic or hematogenous metastasis were reported. No consensus about treatment was found. When surgery was used, it consisted in resection with safety margins ranging from 0.5 to 3cm depending on the teams. In case of metastasis, treatment consisted in chemo- and/or radiotherapy. A quarterly medical monitoring was recommended. DISCUSSION: Malignant cutaneous adnexal tumors are rare. There is nowadays no treatment consensus. An initial staging by mean of a head and neck, chest, abdominal and pelvic CT-scan is mandatory. Treatment has to be decided in a multidisciplinary cancer committee. In the absence of metastasis, the reference treatment is surgical resection, possibly by Mohs micrographic technique, with large safety margins. In case of metastasis or if the loco-regional extension does not allow for a complete excision, chemotherapy and/or radiotherapy may be proposed. A close monitoring is essential.

[Ileum inflammatory fibroid polyp revealed by intussusception. About one familial case]. / Polype fibro-inflammatoire de l'iléon révélé par une invagination. A propos d'un cas familial.

INTRODUCTION: Acute ileum intussusception is a frequent and mostly benign condition in childhood. Conversely, it is a rare condition during adulthood and generally associated with an underlying malignancy. We report a familial form of benign inflammatory fibroid polyps, revealed by an acute ileum intussusception. EXEGESIS: A 41-year-old man, whose mother had undergone three surgical procedures for acute ileum intussusception associated with inflammatory fibroid polyp, was admitted for a abdominal pain that started three month ago. The patient displayed alteration of the intestinal transit, weight loss and sub-occlusive syndrome. Upper and lower endoscopies, performed before admission, were normal. In the emergency room, the abdominal computed tomography-scan revealed an acute intussusception of the last loop of the small intestine, probably caused by a tumor and leading to an occlusive syndrome. Surgical resection and histological analysis concluded to an inflammatory fibroid polyp. Clinical outcome was excellent. CONCLUSION: Inflammatory fibroid polyp is always a benign tumor. It is usually isolated, expressing itself mainly in the form of an acute intussusception when located in the small bowel. The familial form presented here is exceptional and illustrates the possibility of an inherited transmission of this disease. However the pathogenesis of this type of polyp is still unclear.

[Treatment of cutaneous metastases of a squamous cell carcinoma of the leg with topical miltefosine]. / Traitement de métastases cutanées d'un carcinome épidermoïde de la jambe par applications de miltéfosine.

INTRODUCTION: Miltefosine (Hexadecylphosphocholine) is a chemotherapy, which when applied locally, has demonstrated efficacy in the treatment of cutaneous metastases of breast cancer. CASE REPORT: A 79 year-old woman developed recurrent in transit cutaneous metastases of a squamous cell carcinoma on her left leg. All lesions completely resolved after topical applications of miltefosine. Tolerance was excellent and the patient remained disease-free 24 months after the end of the treatment. DISCUSSION: Miltefosine, which might prove to be a simple and effective alternative for the usually heavy treatments proposed, warrants further assessment in this context.

Recurrences following primary osteosarcoma in adolescents and adults previously treated with chemotherapy.

In this retrospective analysis, we report on the detailed management of 33 recurrent osteosarcoma patients from a population of 81 adolescents and adults previously treated (between November 1979 and November 1998) at the La Timone Adults Hospital, for an extremity-localised osteosarcoma. The site of the first recurrence was limited to the lung in 24 patients (73%), was local in 4 patients (12%), at multiple sites in 4 patients (12%), and limited to the bone for 1 patient (3%). The median interval between the diagnosis of the primary osteosarcoma and the first recurrence was 16 months (range 4-108 months). For all patients, the treatment combined aggressive chemotherapy and surgical resection of the recurrences whenever possible. 19 patients (58%) achieved a second complete remission. The median follow-up time from the first recurrence was 18 months (range 4-150 months). For all patients, the median overall survival from first recurrence was 17 months (95% confidence interval (CI), 11-22 months) and the projected 3- and 5-year survival rates were 31.6 and 23.7%, respectively. Patients with a second complete remission had a better 5-year survival than patients without (44.6% versus 0%, P=0.001). The achievement of a second complete remission has an independent significant prognostic value for an improved survival. Aggressive surgery with the removal of recurrence sites combined with multi-agent chemotherapy can either cure patients with recurrent osteosarcoma or significantly prolong their survival.

Schistosomiasis as an unusual cause of appendicitis.

Millions of people originating from tropical areas now live outside the country of their birth. As a consequence, the number of cases of diseases imported from the tropics and being seen by European physicians in immigrants is growing. As an example of such diseases, schistosomal appendicitis is a specific trait of infection with Schistosoma haematobium and is an uncommon cause of appendicitis in non-endemic areas. Treatment requires anti-schistomal medication in addition to surgery. Physicians, including surgeons, need to be aware of the possibility of seeing atypical presentations of parasitic diseases in immigrant patients.

Caspases: conductors of the cell death machinery in lymphoma cells.

The present review focuses on recent insights into the regulation of caspases by other components of the apoptotic pathway, including the mechanisms by which caspase activation influence the death of lymphoma cells. In the light of our recent findings and similar observations of other investigators, it is likely that lymphoma cells possess the complete caspase machinery required for the apoptotic process. Inhibition of caspases activation appears as a potential mechanism to explain apoptotic defects of malignant B-cells, and thus may constitute the basis for new cancer therapies.

[Use of high-density filters ("DNA arrays") for gene expression analysis in human tissues]. / Utilisation de filtres à haute densité (<>) pour l'analyse de l'expression génique dans les tissus humains.

Most technical strategies for the analysis of gene expression in tissues are able to study only one protein or RNA product at the same time. A new recent method referred to as <> or <> is able to analyze simultaneously several hundreds of different genes. The DNA array is a nylon membrane on which are spotted equal amounts of cDNAs corresponding to different genes. This filter is hybridized with a <> probe synthesized with mRNA derived from the tissue analyzed. The result gives a global profile of gene expression within the tissue and allows quantitative and comparative analysis between different tissues or cell types.

[Cutaneous metastasis of pleural mesothelioma: two cases]. / Métastases cutanées de mésothéliome pleural: deux observations.

INTRODUCTION: Cutaneous manifestations of pleural mesothelioma are rare, polymorphic, and occur at various stages of the disease. CASE REPORTS: Case 1. A 60 year-old man, 6 months after the initial diagnosis of a pleural mesothelioma, developed a right latero-thoracic subcutaneous nodule. The lesion resulted from an inoculation of tumoral cells to the wall at the time of the initial thoracoscopy. No specific treatment was proposed. Case 2. A 62 year-old man, with a history of pleural mesothelioma developed an inflammatory layer of the left chest wall. The lesion, laid out like an hemi-belt, was centred by the thoracotomy scar and was associated with retraction phenomena of the thoracic wall. The lesion corresponded to a direct extension of the mesothelioma to the wall. At the same time, the pleural disease was progressing. The patient died a few weeks later. COMMENTS: Cutaneous manifestations usually occur when the diagnosis of primitive pleural cancer has been already established. Distant cutaneous metastases are exceptional and involve with predilection the cephalic extremity. The majority of cutaneous lesions are distributed near the tumoral site and represent either a direct extension of the tumor to the thoracic wall, or a malignant seeding by diffusion of the pleural fluid at the time of the diagnostic and therapeutic procedures.

[DNA-array analysis of the expression profile of apoptosis gene regulators in malignant lymphoma]. / Analyse par "biopuces" du profil d'expression des gènes régulateurs de l'apoptose des lymphomes malins.

Microarray technology has recently led to the identification of molecular prognostic subgroups in non Hodgkin's lymphomas. In order to determine the usefulness of ready-made macroarrays as routine diagnosis tools in haemato-pathology, we have analysed lymph node biopsies using a cDNA macroarray containing genes involved in apoptosis, including caspases. Nine biopsy specimens were analysed on total frozen tissues: 4 samples of B-cell follicular lymphoma (FL), two of B-cell diffuse large cell lymphoma (DLCL), and three of non-neoplastic lymph nodes from benign lymphadenitis. Eight cell populations were sorted from fresh tissues: malignant B-cells from 2 FL cases and 2 DLCL cases, reactive B-cells from 1 benign lymph nodes, reactive T-cells from 1 benign lymph node, virgin (mantle zone) B-cells and germinal center (GC) B-cells from benign tonsils. Immunohistochemistry (IHC) on paraffin sections was performed for localization of caspases 2, 3, 4, 7, 8, and 9. In the clustered array data, sorted cells from samples sharing common histological lesions grouped together, whereas the array/histology correlation was less satisfactory for tissues. The expression profiles of both array and IHC methods were correlated for most caspases and samples. Variations in array profiles of sorted cell populations can be statistically associated with specific histological features, suggesting a possible diagnostic application of ready-made "Apoptosis macroarrays" in haematopathology.

[Recurrent pericarditis revealing a systemic sarcoidosis]. / Péricardite récidivante révélant une sarcoïdose systémique.

INTRODUCTION: Sarcoidosis is a systemic granulomatous disorder of unknown cause, mainly involving the lung and the mediastinum. Involvement of the pericardium and peritoneum is rare, but can be the first manifestation of the disease. CASE REPORT: A 55-year-old female patient was followed-up for a recurrent "idiopathic" pericarditis. Diagnostis was challenged when she secondarily presented with enlarged hilar and mediastinal lymph nodes associated with pulmonary "nodules". Imaging with (18)F-FDG positron emission tomography with computed tomography showed multiple hypermetabolic foci in the mediastinum and peritoneum, which suggested a malignant disorder. Finally, histopathological evaluation of the peritoneal nodules revealed a sarcoidosis. A corticosteroid therapy was initiated and disease course was favourable. CONCLUSION: This case report highlights the importance of a unique explanation for a patient presenting with recurrent pericarditis associated with a systemic disease. Although rare, sarcoidosis should be discussed and diagnostic procedures should be performed to obtain histological confirmation.

c-Met overexpression in inflammatory breast carcinomas: automated quantification on tissue microarrays.

Inflammatory breast carcinoma (IBC) is a rare but aggressive tumour associated with poor outcome owing to early metastases. Increased expression of c-Met protein correlates with reduced survival and high metastatic risk in human cancers including breast carcinomas and is targetable by specific drugs, that could potentially improve the prognosis. In the present study, we compared c-Met expression in IBC (n=41) and non-IBC (n=480) immunohistochemically (Ventana Benchmark autostainer) in two tissue microarrays (TMA) along with PI3K and E-cadherin. The results were quantified through an automated image analysis device (SAMBA Technologies). We observed that (i) c-Met was significantly overexpressed in IBC as compared with non-IBC (P<0.001), (ii) PI3K was overexpressed (P<0.001) in IBC, suggesting that the overexpressed c-Met is functionally active at least through the PI3K signal transduction pathway; and (iii) E-cadherin was paradoxically also overexpressed in IBC. We concluded that overexpressed c-Met in IBC constitutes a potential target for specific therapy for the management of patients with poor-outcome tumours such as IBC. Automated image analysis of TMA proved to be a valuable tool for high-throughput immunohistochemical quantification of the expression of intratumorous protein markers.

Prediction of metastasis risk (11 year follow-up) using VEGF-R1, VEGF-R2, Tie-2/Tek and CD105 expression in breast cancer (n=905).

Neoangiogenesis in tumours contributes to the development of blood-borne metastases, and can be evaluated by markers of activated endothelial cells in preference to panendothelial markers. Our purpose was to document the prognostic significance of VEGF-R1, VEGF-R2, Tie-2/Tek and CD105 immunoexpression in breast carcinoma frozen samples (n=905, follow-up=11.7 years). We observed that: (i). CD105 (P=0.001) and Tie-2/Tek (P=0.025) (but not VEGF-R1 and VEGF-R2) overexpression correlated with a shorter survival, and were (Cox's model) independent histoprognostic indicators; (ii). only CD105 marked expression correlated (P=0.035) with a shorter survival of node-negative patients; (iii). three markers - CD105 (P=0.001), Tie-2/Tek (P=0.01), VEGF-R1 (P=0.001), but not VEGF-R2 - correlated with metastatic risk in node-negative patients in univariate analysis; and (iv). VEGF-R1 (P=0.01) expression correlated with high local recurrence risk. It is concluded that CD105 and to a lesser extent Tie-2/Tek and VEGF-R1, but not VEGF-R2 are endowed with prognostic significance that may be useful for patient monitoring, particularly CD105 expression for selecting node-negative patients for more aggressive postsurgery therapy.

Correlation between apoptosis microarray gene expression profiling and histopathological lymph node lesions.

AIMS: Microarray technology has recently led to the identification of molecular prognostic subgroups in non-Hodgkin's lymphomas. To determine the usefulness of ready made macroarrays as routine diagnostic tools in haematopathology, lymph node biopsies were analysed using a cDNA macroarray containing genes involved in apoptosis, including caspases. METHODS: Nine biopsy specimens were analysed using total frozen tissues: four samples of B cell follicular lymphoma, two of B cell diffuse large cell lymphoma, and three of non-neoplastic lymph nodes from benign lymphadenitis. Nine cell populations were sorted from fresh tissues: malignant B cells from two patients with follicular lymphoma and two with diffuse large cell lymphoma, reactive B cells from two benign lymph nodes, reactive T cells from one benign lymph node, and virgin (mantle zone) B cells and germinal centre B cells from benign tonsils. Immunohistochemistry (IHC) on paraffin wax sections was performed for the localisation of caspases 2, 3, 4, 7, 8, and 9. RESULTS: In the clustered array data, sorted cells from samples sharing common histological lesions were grouped together, whereas the array/histology correlation was less satisfactory for tissues. The expression profiles of both the array and IHC methods correlated for most caspases and samples. CONCLUSIONS: Variations in array profiles of sorted cell populations can be associated with specific histological features, suggesting a possible diagnostic application of ready made apoptosis macroarrays in haematopathology.