[Cutaneous manifestation of lymphoid variant of hypereosinophilic syndrome: One case]. / Un cas de manifestation cutanée d'un syndrome hyperéosinophilique lymphoïde.

INTRODUCTION: Lymphoid hypereosinophilic syndrome (HES) is a reactive HES, related to the presence of an abnormal circulating T cell clone. Cutaneous manifestations are frequent and sometimes inaugural, however few studies describe them specifically. CASE REPORT: We report the case of a 63-year old patient, in good general condition, with no previous history and taking no treatment, who was being followed for non-specific skin lesions. Blood and skin examinations showed hypereosinophilia, the presence of an aberrant CD3-CD4+ phenotype and a positive T-clonality test. There was no differential diagnosis or argument for a systemic lymphoma. CONCLUSION: Cutaneous manifestations of lymphoid HES are variable, non-specific, and may differ according to lymphocyte phenotype. The discovery of SHE requires an extension workup and the risk of evolution towards a systemic lymphoma justifies a close surveillance. Treatment is adapted to the severity of the symptoms.

Treatment of Eosinophilic Annular Erythema: Retrospective multicenter study and literature review.

BACKGROUND: Eosinophilic annular erythema (EAE) is a rare eosinophil-related skin disease which typically manifests with annular erythematous plaques and severe pruritus. Besides the diagnosis, the treatment of EAE is challenging since relevant published data are sparse. METHODS: The aim of this study was to assess the underlying diseases, treatments and outcomes of patients with EAE. To this end, we conducted a retrospective multicenter study and a systematic review of the MEDLINE database. RESULTS: We included 18 patients with EAE followed in 8 centers. The MEDLINE database search yielded 37 relevant publications reporting 55 cases of EAE with 106 treatment sequences. The most common and efficient treatments included topical or systemic corticosteroids, hydroxychloroquine and dapsone. In refractory patients, a combination of systemic corticosteroids with hydroxychloroquine was associated with 88% of complete clinical response. DISCUSSION: To improve the management of EAE patients, we discuss the following treatment strategy: in topical steroid-resistant patients, hydroxychloroquine can be given as first-line systemic treatment. Dapsone, hydroxychloroquine or systemic corticosteroids are second-line options to consider. Last, monoclonal antibodies or JAK inhibitors targeting type 2 inflammation could represent promising last-resort options in refractory patients.

[Dermatomyositis-like syndrome revealing statin-induced necrotizing autoimmune myopathy with anti-HMGCR antibodies]. / Myopathie nécrosante auto-immune à anticorps anti-HMGCR induite par les statines et révélée par un tableau évocateur de dermatomyosite.

BACKGROUND: Statin-induced necrotizing autoimmune myopathy (NAM) has been recently characterized. Herein we report an accurate description of the clinical and histological characteristics of cutaneous rash associated with NAM. PATIENTS AND METHODS: A 61-year-old woman presented a skin rash involving the face, the chest and the back of the hands with heliotropic distribution coupled with proximal symmetrical muscle weakness. Rosuvastatin had been introduced 8 months earlier. Creatinine kinase levels were dramatically raised. Screening for lupus and dermatomyositis antibodies were negative. The cutaneous histology was consistent with neutrophilic lupus while a muscle biopsy revealed no inflammation but showed necrotic and regenerative myofibres. Finally, antibodies directed against 3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) were found at high levels (1658UA/ml vs. normal<13.0UA/ml), resulting in diagnosis of necrotizing autoimmune myopathy (NAM). Intensive immunosuppressive therapy resulted in excellent improvement. DISCUSSION: NAM is a severe acquired autoimmune myopathy characterised by severe proximal weakness and specific positive antibodies (anti-HMGCR or anti-signal recognition particle). It is classically associated with statin use. Some extra-muscular symptoms have been described in previous studies. We report the third accurate description of cutaneous rash associated with statin-induced NAM involving HMGCR antibodies. The skin rash was evocative of connective tissue disease and our diagnosis was based on immunology and muscle histology. CONCLUSION: Dermatologists must be able to recognise this rare entity of "pseudo-dermatomyositis" and then discontinue statin intake if present and carry out further investigations consisting of muscle biopsy and serological tests.

Carotid diaphragm: Atypical fibromuscular dysplasia or atheromatous lesions?

Atypical fibromuscular dysplasia (FMD) is an underdiagnosed cause of ischemic stroke. The typical angiographic pattern of a septum on the posterolateral side of a carotid megabulb is highly suggestive of atypical FMD. We report here on two patients with this highly suggestive pattern of carotid atypical FMD, but which histological examination revealed to be atheromatous lesions. Interestingly, contrast-enhanced ultrasound of the carotid artery showed enhancement of the spur, which should never be the case with an FMD lesion, which has no vasa vasorum. Our findings suggest that the results of studies reporting stenting of atypical FMD in cervical arteries should be interpreted with caution.

[Granulomatous slack skin associated with metastatic testicular seminoma]. / Mycosis fongoïde chalazodermique associé à un séminome testiculaire métastatique.

BACKGROUND: Granulomatous slack skin (GSS) is an extremely rare subtype of T-cell lymphoma, a variant of mycosis fungoides (MF). Herein, we describe the first reported case of GSS associated with metastatic testicular seminoma. PATIENTS AND METHODS: A 28-year-old male patient presented with circumscribed erythematous loose skin masses, especially in the body folds and which had been relapsing for 4years. Skin biopsy showed a loss of elastic fibers and an atypical granulomatous T-cell infiltrate with epidermotropism, enabling a diagnosis of GSS to be made. A biopsy of a retroperitoneal lymphadenopathy showed testicular seminoma metastasis. DISCUSSION: Patients suffering from GSS have a statistically higher risk of developing a second primary cancer, especially Hodgkin's lymphoma. The association found between GSS and a lymphoproliferative malignancy requires long-term follow-up and determines the patient's prognosis. CONCLUSION: It is not possible to prove a formal link between GSS and testicular seminoma. However, this case illustrates the value of screening for a second cancer, particularly where extra-cutaneous lesions appear during GSS treatment. Lymph node biopsy should be performed routinely in the event of GSS with possible lymph node involvement.

Infraorbital nerve involvement on magnetic resonance imaging in European patients with IgG4-related ophthalmic disease: a specific sign.

OBJECTIVES: To measure the frequency of infraorbital nerve enlargement (IONE) on magnetic resonance imaging (MRI) in European patients suffering from an IgG4-related ophthalmic disease (IgG4-ROD) as compared to patients suffering from non-IgG4-related ophthalmic disease (non-IgG4-ROD). METHODS: From January 2006 through April 2015, 132 patients were admitted for non-lymphoma, non-thyroid-related orbital inflammation. Thirty-eight had both pre-therapeutic orbital MRI and histopathological IgG4 immunostaining. Fifteen patients were classified as cases of IgG4-ROD and 23 patients as cases of non-IgG4-ROD. Two readers performed blinded analyses of MRI images. The main criterion was the presence of an IONE, defined as the infraorbital nerve diameter being greater than the optic nerve diameter in the coronal section. RESULTS: IONE was present in 53% (8/15) of IgG4-ROD cases whereas it was never present (0/23) in cases of non-IgG4-ROD (P < 0.0001). IONE was only present in cases where, on MRI, the inflammation of the inferior quadrant was present and in direct contact with the ION canal. CONCLUSIONS: In European patients suffering from orbital inflammation, the presence of IONE on an MRI is a specific sign of IgG4-ROD. Recognition of this pattern may facilitate the accurate diagnosis for clinicians and allow for the adequate management and appropriate care of their patients. KEY POINTS: • IONE on an MRI is a specific sign of IgG4-ROD. • IONE recognition allows for a quicker diagnosis and appropriate management. • IONE appears when inflammation is in direct contact with the ION canal.

Merkel cell carcinoma: value of sentinel lymph-node status and adjuvant radiation therapy.

BACKGROUND: Sentinel lymph-node (LN) biopsy (SLNB) is a valuable tool to assess the regional LN status in Merkel cell carcinoma (MCC). However, its prognostic value is still debated. This study was undertaken to assess SLNB usefulness for MCC management and to determine the impact of SLNB status on disease-free survival (DFS) and overall survival (OS) by comparing SLNB-positive versus -negative patients according to demographic, clinical and treatment characteristics. PATIENTS AND METHODS: In this retrospective, multicenter observational study, SLNB was proposed to all patients referred for clinically N0 MCC. Treatment schedule consisted of wide-margin surgical resection of primary MCC followed by adjuvant radiation therapy (aRT) to the primary site and, for SLNB-positive patients, radical LN dissection followed by regional aRT. Univariate and multivariate analyses determined factors associated with DFS and OS. RESULTS: Among 87 patients with successful SLNB, 21 (24.1%) were SLNB-positive. Median follow-up for the entire series was 39 months; respective 3-year DFS and OS rates were 73% and 81.4%, respectively. Univariate analysis (all patients) identified SLNB-negativity as being associated with prolonged OS (P = 0.013) and aRT (all sites considered) was associated with longer DFS (P = 0.004) and OS (P = 0.018). Multivariate analysis (all patients) retained SLNB status and aRT (all sites considered) as being associated with improved DFS (P = 0.014 and 0.0008) and OS (P = 0.0020 and 0.0019). Moreover, for SLNB-negative patients, tumor-bed irradiation was also significantly associated with prolonged DFS (P = 0.006) and OS (P = 0.014). CONCLUSIONS: The present study demonstrates that SLNB-negativity is a strong predictor of longer DFS and OS in stage I and II MCC patients. The similar benefit for aRT on tumor bed observed in this study has to be confirmed by a prospective study. The results advocate for SLNB being considered to all MCC patients.

A new direct laser photo-induced fluorescence method coupled on-line with liquid chromatographic separation for the simultaneous determination of anilides pesticides.

A new direct laser photo-induced fluorescence high performance liquid chromatography (DL-PIF-HPLC) method is developed for the simultaneous determination of three anilide pesticides, namely carboxin, monalide and propanil. DL-PIF-HPLC uses a tunable Nd:YAG-OPO laser to obtain fluorescent photoproduct(s) and to simultaneously analyze their fluorescence in a short acquisition time with an intensified CCD camera, which improves the selectivity (by choosing the suitable excitation wavelength), increases the sensitivity (due to the high energy of the laser beam) and reduces the time of analysis, relative to the classical PIF methods. However, one of the main drawbacks of PIF methods is the presence of interferences with other compounds, such as other pesticides from the same group yielding similar fluorescent photoproducts, which reduces their selectivity. The analytical interest of DL-PIF-HPLC to avoid these interferences is demonstrated. The DL-PIF spectra, chromatographic conditions and analytical performances of DL-PIF-HPLC are presented for the simultaneous determination of three anilide pesticides. The calibration curves are linear over one order of magnitude and the limits of detection are in the ng mL(-1) range. The new DL-PIF-HPLC system has the advantage to combine the performances of both techniques, DL-PIF and liquid chromatography, and to improve the analysis selectivity.

Determination of phenylurea pesticides by direct laser photo-induced fluorescence.

A direct Laser Photo-Induced Fluorescence (DL-PIF) method is developed for the determination of two phenylurea pesticides, namely fenuron and diflubenzuron. The DL-PIF method uses a tunable Nd:YAG-OPO Laser to obtain the photoproduct(s) and to simultaneously analyse their fluorescence in a short acquisition time on an intensified CCD camera. Compared to classical PIF methods, the use of a tunable laser improves the selectivity (by choosing the suitable excitation wavelength), increases the sensitivity (due to the high energy of the beam) and also reduces the time of analysis. The analytical performances of this method for the determination of both pesticides are satisfactory in comparison to other classical PIF methods published for the determination of phenylurea pesticides. The calibration curves were linear over one order of magnitude and the limits of detection were in the ng mL(-1) range. Satisfactory recoveries were obtained in the analysis of both pesticides in river and sea water spiked samples.

[Diagnosis of skin cancer by dermatologists in the French West Indies: a prospective study]. / Étude prospective de l'incidence des cancers cutanés dépistés en pratique dermatologique aux Antilles françaises.

BACKGROUND: The incidence of skin cancer is not well established in the French West Indies, aside from old data concerning cutaneous melanoma. METHOD: A prospective study was performed over a 3-month period in late 2007 in the French West Indies. The number of new cases of histologically confirmed skin cancer was determined using a questionnaire. RESULTS: The rate of participation of dermatologists was 82 %. During the study period, 166 skin cancers were diagnosed in 134 patients (66 women and 68 men: mean age=63.3 years, SD=2.5), including 137 basal cell carcinomas, 12 melanomas, seven squamous cell carcinomas, six Bowen's disease, one B lymphoma and one Paget's disease. The raw incidence of skin cancers detected by dermatologists was calculated as 64.6 cases/100 000 inhabitants/year for basal cell carcinoma, 5.7 cases/100 000 inhabitants/year for melanoma, 3.3 cases/100 000 inhabitants/year for squamous cell carcinoma, and 2.8 cases/100 000 inhabitants/year for Bowen's disease. Seven melanomas (almost exclusively of plantar topography) and seven basal cell carcinomas were diagnosed in patients of phototype V or VI. DISCUSSION: Although lower than in metropolitan France, the number of skin cancers diagnosed by dermatologists in the French West Indies is fairly high. In addition, this study indicates the significant incidence of basal cell carcinomas and melanomas in subjects with phototype V or VI, underreported in the literature. These findings highlight the need to begin longer-term studies and to include skin carcinomas in the cancer registry of the French West Indies in order to better identify their characteristics among the Caribbean population.

Pancreatocolonic fistula complicating noninvasive intraductal papillary mucinous tumor of the pancreas.

Intraductal papillary mucinous tumors (IPMT) of the pancreas are a distinct clinicopathological entity that is increasingly recognized and whose natural history and clinical presentation are now better understood. Nevertheless, only rare cases of pancreatobiliary or pancreatodigestive fistulas complicating IPMT have been described so far and their clinicopathological significance and association with cancer remain controversial. We report a case of pancreatocolonic fistula complicating a noninvasive IPMT, and review the published literature. Unlike previous reports, IPMT complicated by fistula in nearby organs does not seem to be more often associated with invasive carcinoma: frequency is comparable in resected IPMT with or without internal fistula. Since fistulas are not a reliable clinicopathological predictor of invasive malignancy, en-bloc resection should not be routinely performed especially if extended resection increases the immediate risks or the long-term risks of surgery.