[Cutaneous chondroid syringoma]. / Syringome chondroïde cutané.

INTRODUCTION: Chondroid syringoma (CS) is a rare cutaneous tumor characterized by mixte epithelial and mesenchymal component. The confident histological diagnosis can be obtained by immuno-histochemistry study. Here we present 10 new cases with their clinico-hystological characteristics. METHODS: The 10 cases were observed between January 2000 and august 2013, in Fort-de-France and Louis-Mourier universitary hospitals. For all the cases a controlled histological study was performed by a dermatopathologist expert and immuno-histochemistry was added. Clinical and immuno-histological data were analyzed. RESULTS: The lesions were almost localized on the face (3/10) and the extremities (3/10). The size was about 1.2 to 5.2cm. Every case was treated by surgery, no malignant case was diagnosed. Histologically, all the 10 cases presented as a well-limited dermic tumor with a mixte epithelial and mesenchymal component. The stroma was myxo-chondroid, and the epithelial component consisted in epithelial cavities lined by one or two cell layers with eccrine (4/10) or apocrine (5/10) features. Immuno-chemistry study reveals positivity for EMA, ACE and CK7 for the internal cells, and positivity for S100 protein and vimentin of the extern cell layer. DISCUSSION: Chondroid syringoma is characterized by a mixte epithelial with eccrine and apocrine cells and a myxo-chondroid stroma. Our study has some clinical and histological particularities (lesions on the extremities, epidermic connecting…). The main differentials diagnoses are the other annexial tumors. The treatment is surgical. CONCLUSION: The histological diagnosis of CS is quite easy, but in case of doubt, immuno-chemistry will help, showing a double mesenchymal and epithelial differentiation.

High prevalence of small intestinal bacterial overgrowth in patients with morbid obesity: a contributor to severe hepatic steatosis.

BACKGROUND: With the increasing prevalence of obesity, non-alcoholic fatty liver disease (NAFLD) has become a major cause of liver diseases. Small intestinal bacterial overgrowth (SIBO) could be related to NAFLD. Our aim was to determine the prevalence of SIBO and its relationship with liver lesions in morbidly obese patients. METHODS: A glucose hydrogen (H(2)) breath test (positive if fasting breath H(2) concentration > 20 ppm and/or an increase of > 10 ppm over baseline within the first 2 h) was performed in obese patients referred for bariatric surgery (body mass index [BMI] > 40 kg/m(2) or > 35 in association with comorbidities) and in healthy non-obese subjects. In obese patients, a surgical liver biopsy was performed. RESULTS: One hundred and forty-six patients (129 women, age [mean+/-SE]: 40.7 +/- 11.4 years) were prospectively included in the study. The mean BMI was 46.1+/-6.4 kg/m(2). A liver biopsy was available in 137 patients and a breath test in 136. The frequency of positive breath tests was higher in obese patients (24/136, 17.1%) than in healthy subjects (1/40, 2.5%; P=0.031). In the univariate analysis, SIBO was not associated with clinical variables, but tended to be associated with more frequent severe hepatic steatosis (26.3 vs. 10.3%, P=0.127), whereas the frequency of sinusoidal or portal fibrosis, lobular necrosis and non-alcoholic steatohepatitis (NASH) were not different. In the multivariate analysis, SIBO (P=0.005) and the presence of a metabolic syndrome (P=0.006) were independent factors of severe hepatic steatosis. CONCLUSION: In morbidly obese patients, bacterial overgrowth prevalence is higher than in healthy subjects and is associated with severe hepatic steatosis.

Monoarthritis secondary to joint metastasis. Two case reports and literature review.

Synovial metastases from neoplasms are uncommon. We report two cases of knee monoarthritis due to joint metastasis. Joint fluid cytology established the diagnosis. In one patient, an epidermoid carcinoma of the ureter metastasized to the left knee. The other patient had chronic monoarthritis of the left knee unresponsive to conventional treatment and was found to have distal femoral metastases from a lung adenocarcinoma. Only 28 cases of synovial metastases from solid tumors have been reported in the literature. The knee is the most common target, the lung the most common site of the primary (12 cases), and adenocarcinoma the most common histological type (12 cases). Joint metastasis carries a poor prognosis with a mean survival of less than 5 months.

[Solitary cutaneous infantile myofibromatosis]. / Myofibromatose infantile cutanée isolée.

Infantile myofibromatosis is the most frequent fibromatosis in childhood. It is a benign proliferation of fibroblasts and myofibroblasts. This case report concerns a newborn who presented at birth with a purple cutaneous nodule on the scalp. Surgical excision was performed at the age of 16 months. Infantile myofibromatosis was diagnosed on histology. Infantile Myofibromatosis (IMF) was first described by Enzinger in 1981. Three types can exist. Solitary MFI, the most frequent, is a solitary lesion, cutaneous/subcutaneous, osseous or involving soft tissues. Multicentric disease is characterized by multiple locations and generalized form by visceral involvement. Morphological features are identical in all types. The histological diagnosis relies on the identification of two separate components, a fascicular myofibroblastic pattern at the periphery with a hemangiopericytoma like pattern in the centre. Both components are positive for alpha-smooth muscle actin. Atypia, or mitotic activity, are not observed usually but features of intravascular growth can be seen in the centre of the lesion. Infantile MF carries a good prognosis when solitary but death frequently occurs in generalized MF with visceral involvement.

[Postmenopausal frontal fibrosing alopecia. Report of 3 cases]. / Alopécie frontale fibrosante post-ménopausique.

Postmenopausal frontal fibrosing alopecia is a rare aspect of scarring alopecia concerning elderly women. It appears as a receding anterior hair line localised in the frontal and temporal regions. It is a particular pathologic and clinical form of lichen planopilaris. The histologic aspect is that of a lichenoïd inflammatory infiltrate affecting the dermal follicular junction, accompanied by a fibrous scarring aspect, the latter contributing to the diagnosis and individualization of this entity. Discoïd lupus erythematous is the main histologic differential diagnosis. Postmenopausal period is the only associated condition found in affected women. Evolution is unpredictable and does not seem to be modified by treatment.

[Pilomatrix carcinoma: histologic and immunohistochemical features. Two studies]. / Pilomatricome malin: aspects histologiques et immunohistochimiques. A propos de deux observations.

Malignant pilomatricoma is a rare malignant hair follicle tumor, that was initially described in 1980. Histologically, it shares common features with the more frequent, which benign pilomatricoma, makes its diagnosis difficult. It is a deep dermis-hypodermis epithelial tumor, well circumscribed, with no relation with the epidermis. It is composed of nodular structures with rows of basaloid cells in their periphery, as well as focal necrosis and mummified "ghost" cells in their central parts. Immunohistochemistry is of little value and can not confirm malignancy. The diagnosis remains essentially morphological. Histological examination must stress on the evaluation of the degree and extent of infiltration of the surrounding tissues, the degree of necrosis, the presence of atypical mitotic figures, and the presence or not of peri-neural or vascular invasion. Surgical wide resection is the recommended treatment. It reduces the risk of local recurrence by 50%. Malignant pilomatricoma carries a high risk of metastases to the bones, lungs, and lymph nodes. No feature is specific to confirm wether a malignant pilomatricoma arises de novo, or whether it is a malignant transformation of a pre-existing benign pilomatricoma.

[Unusual parosteal osteochondromatous proliferation or Nora's tumor. A clinicopathological analysis of 4 cases]. / Prolifération ostéocartilagineuse parostéale bizarre ou tumeur de Nora. A propos de quatre observations.

Nora's lesion, also known as bizarre parosteal osteochondromatous proliferation (B.P.O.P.), involves mostly the small tubular bones of the hands and feet. Histologically, it is characterized by a proliferation of chondroid, bony and fibrous tissues, sometimes with high cellular density, bizarre chondrocytes but is devoid of cellular atypia and necrosis. Distinct blue color is noted at the interface of bone and cartilage. The most important lesions that present differential diagnostic problems are chondrosarcoma, parosteal osteosarcoma and florid reactive periostitis. The lesion is benign but may recur locally in as many as 55%. The clinical and pathological findings of four cases of Nora's lesion are presented.

Targeted skin overexpression of the mineralocorticoid receptor in mice causes epidermal atrophy, premature skin barrier formation, eye abnormalities, and alopecia.

The mineralocorticoid receptor (MR) is a transcription factor of the nuclear receptor family, activation of which by aldosterone enhances salt reabsorption in the kidney. The MR is also expressed in nonclassical aldosterone target cells (brain, heart, and skin), in which its functions are incompletely understood. To explore the functional importance of MR in mammalian skin, we have generated a conditional doxycycline-inducible model of MR overexpression, resulting in double-transgenic (DT) mice [keratin 5-tTa/tetO-human MR (hMR)], targeting the human MR specifically to keratinocytes of the epidermis and hair follicle (HF). Expression of hMR throughout gestation resulted in early postnatal death that could be prevented by antagonizing MR signaling. DT mice exhibited premature epidermal barrier formation at embryonic day 16.5, reduced HF density and epidermal atrophy, increased keratinocyte apoptosis at embryonic day 18.5, and premature eye opening. When hMR expression was initiated after birth to overcome mortality, DT mice developed progressive alopecia and HF cysts, starting 4 months after hMR induction, preceded by dystrophy and cycling abnormalities of pelage HF. In contrast, interfollicular epidermis, vibrissae, and footpad sweat glands in DT mice were normal. This new mouse model reveals novel biological roles of MR signaling and offers an instructive tool for dissecting nonclassical functions of MR signaling in epidermal, hair follicle, and ocular physiology.

Vitamin E uncouples joint destruction and clinical inflammation in a transgenic mouse model of rheumatoid arthritis.

OBJECTIVE: Reactive oxygen species are thought to play a role in rheumatoid arthritis (RA) in humans. We postulated that antioxidant treatment could have a beneficial effect in this disease. We therefore investigated the effects of vitamin E in the transgenic KRN/NOD mouse model of RA. METHODS: Mice were treated by gavage with oral vitamin E (alpha-tocopherol). Clinical, histologic, and biochemical parameters were assessed for 6 weeks. RESULTS: Vitamin E treatment did not modify the clinical features of the disease (date of onset or disease intensity, as measured by the articular index), but it did prevent joint destruction, as measured by qualitative and semiquantitative analyses. Redox status did not differ between treated and control mice. White blood cell chemiluminescence was higher in transgenic KRN/NOD mice than in controls, but vitamin E treatment attenuated this difference. Vitamin E treatment of the transgenic animals led to a significant decrease in the levels of interleukin-(IL-1beta) but not tumor necrosis factor alpha. CONCLUSION: Vitamin E seems to uncouple joint inflammation and joint destruction in this model of RA, with a beneficial effect on joint destruction. Since many investigations are currently in progress to evaluate the benefit of interventions targeted toward anti-IL-1beta, our findings suggest opportunities of therapeutic interest in human RA.

Blockade of vascular endothelial growth factor receptor I (VEGF-RI), but not VEGF-RII, suppresses joint destruction in the K/BxN model of rheumatoid arthritis.

It was recently shown that vascular endothelial growth factor (VEGF), a growth factor for endothelial cells, plays a pivotal role in rheumatoid arthritis. VEGF binds to specific receptors, known as VEGF-RI and VEGF-RII. We assessed the physical and histological effects of selective blockade of VEGF and its receptors in transgenic K/BxN mice, a model of rheumatoid arthritis very close to the human disease. Mice were treated with anti-mouse VEGF Ab, anti-mouse VEGF-RI and -RII Abs, and an inhibitor of VEGF-RI tyrosine kinase. Disease activity was monitored using clinical indexes and by histological examination. We found that synovial cells from arthritic joints express VEGF, VEGF-RI, and VEGF-RII. Treatment with anti-VEGF-RI strongly attenuated the disease throughout the study period, while anti-VEGF only transiently delayed disease onset. Treatment with anti-VEGF-RII had no effect. Anti-VEGF-RI reduced the intensity of clinical manifestations and, based on qualitative and semiquantitative histological analyses, prevented joint damage. Treatment with a VEGF-RI tyrosine kinase inhibitor almost abolished the disease. These results show that VEGF is a key factor in pannus development, acting through the VEGF-RI pathway. The observation that in vivo administration of specific inhibitors targeting the VEGF-RI pathway suppressed arthritis and prevented bone destruction opens up new possibilities for the treatment of rheumatoid arthritis.