Relapsing lymphomatoid papulosis after allogenic bone-marrow transplant.

Lymphomatoid papulosis (LyP) is a rare cutaneous lymphoproliferative disorder in children, which can rarely be associated with a cutaneous or systemic lymphoma. We report a 13-year-old girl who presented with typical LyP and pathological features of subtype A. Six months later, the patient presented with rapidly progressive peripheral and systemic lymphadenopathy. On examination of a lymph-node biopsy, a lymphoid infiltrate negative for anaplastic lymphoma kinase (ALK) and positive for CD30 was found, suggestive of systemic anaplastic large T-cell lymphoma (S-ALCL). The patient was treated with chemotherapy, followed by allogeneic bone-marrow transplant (BMT). Over the following 6 years, she presented with biopsy-confirmed LyP relapses with complete cutaneous, peripheral-blood and bone-marrow chimerism. This is only the third reported paediatric association of S-ALCL with LyP to our knowledge, and seems to be the first paediatric case of recurrent relapses of LyP after bone-marrow allograft for S-ALCL with total (100%) cutaneous and bone-marrow chimerism. LyP occurring after allogenic BMT does not appear to be donor-derived.

Clinical features and outcome of sphenoid sinus aspergillosis: A retrospective series of 15 cases.

INTRODUCTION: Sphenoid sinusitis is uncommon, and aspergillus infections represent a minority of these cases. This study was designed to describe the characteristics of this disease and present a review of the literature. PATIENTS AND METHOD: Retrospective study from 2004 to 2010 based on 15 patients managed at Nantes University Hospital. Risk factors and history, symptoms, intranasal examination, imaging, histological and mycological results as well as analysis of the response to treatment and outcome were analysed. RESULTS AND DISCUSSIONS: Patients were aged between 14 to 78 years, almost 75% of patients were older than 50 and 73% of patients were women. The most common symptoms were headache [80%], nasal blockage or discharge [33%], and recurrent mild epistaxis [20%]. Predisposing factors were immunodepression in three patients, with no cases of diabetes. Two patients had a history of intranasal surgery and one had a history of facial trauma. Nasal endoscopy was normal in 40% of cases. CT was suggestive of sphenoid sinus aspergillosis in more than one half of cases [8/15] and demonstrated osteolysis in four patients. An incorrect preoperative diagnosis of mucocele was proposed in three patients. Histological examination demonstrated spore-forming structures in every case, but culture was positive in only four cases. Only two patients required antifungal therapy, including one patient with invasive aspergillosis. CONCLUSION: Chronic noninvasive sphenoid sinus aspergillosis appears to be a benign disease, essentially affecting women and patients over the age of 50 years. Symptoms are fairly non-specific. Imaging and histological examination are essential for management. The invasive form is a serious disease requiring rapid, multidisciplinary management.

Juvenile ossifying fibroma of the maxilla.

INTRODUCTION: Juvenile ossifying fibroma is a rare, benign fibro-osseous tumour. In the light of a clinical case, the authors review the diagnosis, treatment and histological classification of these tumours. CASE REPORT: A seven-year-old child presented with a naso-orbito-ethmoidal trabecular juvenile ossifying fibroma. Complete surgical resection via a transfacial approach was performed after a preoperative work-up comprising head and neck CT and MRI. DISCUSSION: A review of the literature reveals that treatment of this aggressive tumour must comprise complete surgical resection via an incision determined by local tumour extension. CONCLUSION: Patients with juvenile ossifying fibroma tumour require long-term follow-up due to the high recurrence rate.

[Xanthelasma associated with Wegener's granulomatosis]. / Xanthélasmas et granulomatose de Wegener.

BACKGROUND: There have been rare reports of normolipidaemic xanthelasma in Wegener's granulomatosis. Herein, we describe another case associated with a review of the literature. CASE REPORT: A 62-year-old woman was hospitalized for Wegener's disease with cutaneous, ENT, renal, joint and neurological signs. Physical examination showed bilateral palpebral xanthelasma with infiltrated lacrimal glands that had appeared simultaneously to the other clinical signs. No hyperlipidaemia was noted. Biopsy of the xanthelasma revealed a typical infiltrate of foamy histiocytes and focal vasculitis. On treatment with corticoids and cyclophosphamide, the yellow lid lesions gradually disappeared. DISCUSSION: Normolipidaemic xanthelasma is rarely reported in association with Wegener's granulomatosis. It accompanies local ophthalmological inflammation and its appearance during the course of this disease must be dealt with carefully.

[Congenital cyst and fistula of the face and neck]. / Kystes et fistules congénitaux de la face et du cou.

The authors propose an update on cervicofacial congenital cysts and fistulas' symptomatology. Embryological data, epidemiology and clinical manifestations are described. A reminder of the therapeutic principles is proposed as well as the evolution of these congenital pathologies, which may or may not involve the branchial system. branchial.

[Pyodermatitis-pyostomatitis vegetans with nasal involvement]. / Pyostomatite-pyodermatite végétante avec atteinte nasale.

BACKGROUND: Pyodermatitis-pyostomatitis vegetans (PPV) is a rare chronic disorder often associated with inflammatory bowel disease. We report an isolated case involving the oral, labial and nasal mucosa. PATIENTS AND METHODS: A 28-year-old man, in good general condition, presented with a 2-year history of painless stomatitis. The physical examination revealed pustular and exophytic lesions of the jugal, gingival and palatine mucosa on an erythematous background, as well as some pustular and crusted lesions of the lower lip and nostrils. Histopathological analysis revealed epithelial hyperplasia and a suprabasal cleft with some signs of acantholysis and numerous neutrophils and eosinophils. Direct and indirect immunofluorescence assay was negative. There was no associated bowel disease. We concluded on a diagnosis of PPV of younger subjects. The lesions disappeared with oral corticosteroids but with steroid dependency. DISCUSSION: PPV is a rare dermatosis associated in more than 75% of cases with inflammatory bowel disease, usually ulcerative colitis. Lesions of the oral mucosa are a constant finding and are characterised by aseptic pustules on an erythematous background. Skin lesions are pustular and more or less exophytic. To our knowledge, there have been no reports to date of intranasal lesions of PPV.

Mixed adenocarcinoma and squamous cell carcinoma arising in a gastric duplication cyst.

Malignant transformation of duplication cyst is a rare condition. The authors report the original case of a degenerated gastric duplication cyst in a 67-year-old patient. The histologic examination revealed a gastric duplication cyst infiltrated with both adenocarcinoma and squamous cell carcinoma. Local carcinomatosis was found at laparotomy. The patient died six months after complete macroscopic resection of the lesion, with metastatic disease.

[Recurrent adenomas in ileal pouch following ileoanal anastomosis for familial adenomatous polyposis]. / Adénomatose récidivante du réservoir iléal après anastomose iléoanale pour polypose adénomateuse familiale.

Polyadenoma occuring on ileal pouch is a rare event. The treatment and the frequency of the endoscopic examinations is still matter of controversies. The authors report the case of recurrent endoileal pouch adenoma.

[Rating of tumoral growth in non-operated primary or recurrent extra-abdominal aggressive fibromatosis]. / Etude de la croissance tumorale des tumeurs desmoïdes extra-abdominales (FDEA) primitives ou récidivées non opérées.

PURPOSE OF THE STUDY: Extra-abdominal aggressive fibromatosis (EAAF) is a benign desmoid tumor with a potentially aggressive behavior. Surgical treatment is compromised by a very high rate of recurrence, sometimes with significant morbidity. We conducted a prospective surveillance of our patients (clinical and MRI) with EAAF to search for prognostic factors. MATERIAL AND METHODS: This cohort included 17 patients with EAAF. For nine patients, biopsy alone was performed. For eight, the tumor was a recurrence after surgical removal. Patients were seen for a clinical assessment and MRI every six months. RESULTS: Median follow-up was 42 months (range 6-114). Three patients worsened clinically with pain or functional impairment. One patient required neurosurgery to control pain (good stable outcome). MRI showed progression for two tumors (12%) but with a short follow-up since diagnosis (9 and 14 months), in one case despite medical treatment. Three tumors regressed and twelve remained stable on successive MRI. On average the tumor growth lasted ten months. DISCUSSION: Tumor growth was never noted beyond 36 months. This notion of an interruption in tumor growth is mentioned sporadically in reports on EAAF, which have generally included recurrent tumors. To our knowledge this is the first series reporting tumors left in place a followed with modern imaging techniques. The high rate of spontaneous interruption of tumor growth must be counterbalanced with the difficult task of local treatment: the risk of recurrence is particularly high after surgery and functional sequelae can be significant when wide resection is proposed in an anatomically difficult localization. The precise role for surgery, and combined radiotherapy, remain to be determined. There are only scarce reports on general treatments. Considering these facts, we propose that surgical resection should not be considered the only solution for the treatment of EAAF. Further work is needed to define the useful contribution of simple surveillance of these benign tumors.

Human submucosal neurones regulate intestinal epithelial cell proliferation: evidence from a novel co-culture model.

The role of the human enteric nervous system (ENS) in the control of the intestinal epithelium organization and proliferation is unknown. To address this issue, we developed a novel co-culture model, consisting of human submucosa containing the submucosal plexus and a human colonic epithelial monolayer. After 3 days in basal conditions (i.e. in absence of neuronal activation) epithelium disorganization and proliferation occurred. In contrast, electrical activation of submucosal neurones maintained monolayer organization and decreased cell proliferation. These effects were blocked by tetrodotoxin and a vasoactive intestinal peptide (VIP) receptor antagonist, and reproduced by VIP. In conclusion, our study suggests that the human ENS is involved in the control of epithelial cell proliferation.

[Systemic necrobiotic xanthogranuloma with initial pericardial and pulmonary involvement]. / Xanthogranulome nécrobiotique révélé par une atteinte péricardique et pulmonaire.

INTRODUCTION: Necrobiotic xanthogranuloma is a rare disease, usually associated with a monoclonal gammapathy. We report a case with pericardial and pulmonary involvement that preceded the appearance of the cutaneous lesions. OBSERVATION: A 58 year-old woman was hospitalized for cardiac tamponade. She has a past history of a ductal carcinoma of the right breast that had been completely cured. Pericardial and pulmonary samples showed a predominantly histiocyte monomorphous infiltrate. Twelve months later, indurated papular-like cutaneous lesions appeared around the eye orbits and on the thorax. The histological examination of the skin revealed a necrobiotic xanthogranuloma and the diagnosis of cutaneous and visceral necrobiotic xanthogranuloma was retained. The patient exhibited an IgG Kappa monoclonal gammapathy of undetermined significance. Sequential treatment with melphalan and general corticosteroids moderately improved the cutaneous lesions but did not modify the monoclonal peak. DISCUSSION: To our knowledge, this is the first case of pericardial involvement of a necrobiotic xanthogranuloma. The discovery of the disease, revealed by a visceral manifestation at the onset is uncommon. The subsequent appearance of typical cutaneous lesions permits the retrospective diagnosis of systemic necrobiotic xanthogranuloma. Because its diagnosis is difficult in the absence of concomitant cutaneous involvement, visceral localizations of the disease must be recognized.

Tk, a new colon tumor-associated antigen resulting from altered O-glycosylation.

Erythrocyte polyagglutination antigens T and Tn are truncated O-glycan chains that are also carcinoma-associated antigens. We investigated whether Tk polyagglutination antigen could similarly be a carcinoma-associated marker and a target of immunotherapy. Monoclonal antibody LM389 was raised against Tk erythrocytes and tested by immunohistochemistry. LM389 strongly reacted with 48% human colorectal carcinomas. Labeling of normal tissues was visible on epithelial cells, mainly digestive, but was confined at a supranuclear level. Expression of the antigen on cloned human carcinoma cells correlated with sialosyl-Tn expression. O-Sialoglycoprotein endopeptidase treatment revealed that on carcinomas and cell lines, the epitope was present on O-glycans. Antibody specificity was determined using synthetic carbohydrates. Direct binding and inhibition studies indicated that LM389 best ligands were terminated by two branched N-acetylglucosamine units. Screening of murine cellular cell lines with LM389 allowed development of an experimental model with Tk-positive and -negative cells in syngeneic BDIX rats. Vaccination of rats with Tk erythrocytes provided a protection against growth of rat Tk-positive, but not of Tk-negative, tumor cells in association with the development of antibodies. Taken together, the results indicate that Tk polyagglutination antigen is a new colorectal carcinoma-associated antigen, absent from the normal cell surface, resulting from alteration of O-glycans biosynthesis and with potential as a target of immunotherapy.

[Systemic vasculitis]. / Les vascularites systémiques.

Systemic vasculitis is a general term designating a wide range of sometimes overlapping vascular conditions with marked tropism for peripheral constituting a complex nosological structure. Angiologists must be aware of the different telltale clinical signs including purpura, polyarthritis, glomerulonephritis, polyneuritis and unexplained inflammatory syndrome. Medium-sized and small-sized vessels are involved in a wide range of visceral damage. Several typical clinical presentations have led to the individualization of recognized syndromes such as rheumatoid type purpura, Wegener disease, Churg disease, Strauss disease, or peri-arteritis nodosa. Anti-nuclear serologies (pANCA and cANCA) contribute to diagnosis and have been helpful in individualizing a new entity: microscopic polyangeitis. In angiological practice, macroscopic expression of systemic vasculitis can lead to Takayasu type granulomatous angeitis in yong women and giant-cell arteritis in the elderly. These different arteriopathies should be treated by corticosteroid therapy with regular laboratory tests to follow inflammation and angiograhic exploration in case of occlusive arterial complications. Although these conditions are quite rare, a better understanding of their pathogenesis and their clinical course should help the angiologist make the initial diagnosis and the therapist to monitor treatment efficacy.

[Eruptive xanthogranulomatosis in a trisomy 21 patient with acute lymphoblastic leukemia]. / Xanthogranulomatose éruptive au cours d'une leucémie aiguë lymphoblastique chez un malade trisomique 21.

BACKGROUND: Juvenile xanthogranulomas may be associated with myeloproliferative disorders, usually juvenile chronic granulocytic leukemia. CASE REPORT: We report the case of a 25-year-old man with Down's syndrome who presented eruptive xanthogranulomas. At this time, the patient was being treated for acute lymphocytic leukemia. The eruption increased with an extensive unusual "satellite" arrangement despite apparent remission of the acute leukemia. Leukemia relapse occurred some months later. DISCUSSION: This case had an unusual clinical presentation. It also suggests their might be a link between the clinical course of lymphocytic leukemia and juvenile xanthogranuloma.

[Disclosure of Behcet disease by concomitant dental infection]. / Poussée inaugurale de maladie de Behçet concomitante d'un foyer infectieux dentaire.

BACKGROUND: Behçet's disease can be aggravated by chronic infection. Our case illustrates how the disease can be disclosed during an acute activation of a chronic infectious focus. CASE REPORT: A 40-year-old man had Behçet's syndrome associating fever, bipolar aphtosis, cutaneous pseudo folliculitis, anterior uveitis, and meningitis following an acute episode of a dental infection, with periapical granuloma. Antibiotic therapy was ineffective in calming this first flare-up of Behçet's disease. Corticosteroid and colchicine therapy were effective. DISCUSSION: Clinical and experimental work would implicat buccal streptococcal flora in the pathogenesis of Behçet's disease. Antibiotics offer interesting perspectives but further studies are needed to define their role in the treatment of Behçet's disease.