Epidemiological changes in cutaneous lymphomas: an analysis of 8593 patients from the French Cutaneous Lymphoma Registry.

BACKGROUND: Primary cutaneous lymphomas (PCLs) are a heterogeneous group of T-cell (CTCL) and B-cell (CBCL) malignancies. Little is known about their epidemiology at initial presentation in Europe and about potential changes over time. OBJECTIVES: The aim of this retrospective study was to analyse the frequency of PCLs in the French Cutaneous Lymphoma Registry (GFELC) and to describe the demography of patients. METHODS: Patients with a centrally validated diagnosis of primary PCL, diagnosed between 2005 and 2019, were included. RESULTS: The calculated incidence was unprecedently high at 1·06 per 100 000 person-years. The number of included patients increased yearly. Most PCL subtypes were more frequent in male patients, diagnosed at a median age of 60 years. The relative frequency of rare CTCL remained stable, the proportion of classical mycosis fungoides (MF) decreased, and the frequency of its variants (e.g. folliculotropic MF) increased. Similar patterns were observed for CBCL; for example, the proportion of marginal-zone CBCL increased over time. CONCLUSIONS: Changes in PCL frequencies may be explained by the emergence of new diagnostic criteria and better description of the entities in the most recent PCL classification. Moreover, we propose that an algorithm should be developed to confirm the diagnosis of PCL by central validation of the cases.

[Spontaneous choroidal hemorrhage associated with novel oral anticoagulants: A report of two cases and literature review]. / Hémorragie choroïdienne spontanée associée à la prise de nouveaux anticoagulants oraux : cas cliniques et revue de la littérature.

New direct oral anticoagulants (DOAC) have been approved for treatment and prevention of some thromboembolic diseases: acute and chronic phase of thromboembolic disease, deep venous thrombosis prophylaxis in orthopedic surgery and prevention of stroke in patients with atrial fibrillation. These molecules are an alternative to heparins and vitamin K antagonists. Among these, rivaroxaban (Xarelto®, Bayer Schering Pharma) is a direct factor Xa inhibitor, and dabigatran etexilate (Pradaxa®, Boehringer Ingelheim) is a direct free thrombin inhibitor. These molecules are almost the ideal anticoagulant: oral administration, few drug and food interactions, wide therapeutic target, and especially no lab monitoring. However, their use remains associated with hemorrhagic complications such as gastrointestinal, intracranial or urinary hemorrhages. We describe two clinical cases of spontaneous choroidal hemorrhage in patients treated with direct oral anticoagulants (rivaroxaban and dabigatran etexilate) for atrial fibrillation. These cases show that an ocular hemorrhagic risk exists with these drugs. Patients treated with DOAC should have the therapeutic dose adjusted based on creatinine clearance. Special monitoring should be performed in patients with age-related macular degeneration or with hypertension even though meta-analysis shows that the risk of intraocular bleeding is reduced by 22% compared with warfarin.

Oesophageal involvement in 26 consecutive patients with mucous membrane pemphigoid.

BACKGROUND: Oesophageal involvement of mucous membrane pemphigoid (MMP) has not yet been thoroughly described. OBJECTIVES: To characterize systematically the endoscopic lesions of a series of patients with oesophageal symptoms seen at a referral centre for autoimmune bullous diseases. METHODS: Clinical, endoscopic and immunological findings of consecutively referred patients with MMP with oesophageal involvement, systemic and endoscopic treatments, and follow-up are described. RESULTS: Of 477 consecutive patients with MMP consulting between 2002 and 2012, 26 (5·4%) had symptomatic oesophageal involvement. Dysphagia, observed in 23 (88%) patients, was the most frequent symptom. Oesophageal symptoms could be the first sign of MMP. Patients with oesophageal involvement had a mean of three other involved sites. At initial oesophageal endoscopy, 17 of 26 patients had active lesions (intact bullae, erosions and/or erythema), 15 had stricture(s) and 12 had other cicatricial lesions. Systemic therapy alone achieved oesophageal symptom relief for five patients. Dilatation was combined with systemic therapy for 12 patients and was successful in nine; one perforation occurred. CONCLUSIONS: Symptomatic oesophageal involvement affected 5·4% of patients with MMP. Dermatologists and gastroenterologists should be aware of these mucocutaneous diseases and their oesophageal involvement, as it could lead to earlier diagnosis and better care. Oesophageal dilatation could be a therapeutic option for symptomatic stricture not relieved by optimized systemic therapy alone.

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.

Frequency and prognostic value of cutaneous molecular residual disease in mycosis fungoides: a prospective multicentre trial of the Cutaneous Lymphoma French Study Group.

BACKGROUND: Monoclonal T-cell receptor (TCR) rearrangement is detected in 57-75% of early-stage mycosis fungoides (MF) at diagnosis. A retrospective study showed molecular residual disease (MRD) in 31% of patients in complete clinical remission (CR) after 1 year of treatment. OBJECTIVES: To confirm the frequency of MRD at 1 year and to determine its prognostic value for further relapse. METHODS: Patients with T1-, T2- or T4-stage MF were prospectively included in this multicentre study. At diagnosis, clinical lesions and healthy skin were biopsied. After 1 year of topical treatment, previously involved skin of patients in CR was biopsied for histology and analysis of TCR-γ gene rearrangement. The results were compared with the clinical status each year for 4 years. RESULTS: We included 214 patients, 133 at T1, 78 at T2 and three at T4 stage. At diagnosis, 126 of 204 cases (61·8%) showed TCR clonality in lesional skin. After 1 year, 83 of 178 patients (46·6%) still being followed up were in CR and 13 of 63 (21%) showed MRD. At 4 years, 55 of 109 patients (50·5%) still being followed up were in CR and 44 of 109 (40·4%) were in T1 stage. MRD did not affect clinical status at 4 years (CR vs. T1/T2, P = 1·0; positive predictive value 36·4%; negative predictive value 67·6%). CONCLUSIONS: T-cell clonality at diagnosis and MRD at 1 year are not prognostic factors of clinical status at 4 years.

[Acanthamoeba keratitis]. / Kératites amibiennes.

Early diagnosis and appropriate therapy are key elements for a good prognosis in Acanthamoeba keratitis (AK). AK should be considered in any case of corneal trauma complicated by exposure to soil or contaminated water, and in all contact lens (CL) wearers. A presumptive diagnosis of AK can be made clinically and with in vivo confocal microscopy, although a definitive diagnosis requires identification of Acanthamoeba on direct scraping, histology, or identification of Acanthamoeba DNA by polymerase chain reaction (PCR). We use cysticidal drugs for treating AK because encysted forms are more resistant than trophozoites to treatment. The treatment protocol used a biguanide (PHMB 0.02% or chlorhexidine 0.02%) and a diamidine (propamidine 0.1% or hexamidine 0.1%). New diagnostic modalities and more specific topical anti-amoebic treatments would substantially benefit patients with AK.

Guidelines on the use of extracorporeal photopheresis.

BACKGROUND: After the first investigational study on the use of extracorporeal photopheresis for the treatment of cutaneous T-cell lymphoma was published in 1983 with its subsequent recognition by the FDA for its refractory forms, the technology has shown significant promise in the treatment of other severe and refractory conditions in a multi-disciplinary setting. Among the major studied conditions are graft versus host disease after allogeneic bone marrow transplantation, systemic sclerosis, solid organ transplant rejection and inflammatory bowel disease. MATERIALS AND METHODS: In order to provide recognized expert practical guidelines for the use of this technology for all indications the European Dermatology Forum (EDF) proceeded to address these questions in the hands of the recognized experts within and outside the field of dermatology. This was done using the recognized and approved guidelines of EDF for this task. RESULTS AND CONCLUSION: These guidelines provide at present the most comprehensive available expert recommendations for the use of extracorporeal photopheresis based on the available published literature and expert consensus opinion.

[Ocular manifestations of arachnoid cysts in children: report of two cases]. / Kystes arachnoïdiens et manifestations ophtalmologiques chez l'enfant : à propos de deux cas.

Arachnoid cysts are congenital benign intracranial collections of cerebrospinal fluid. Most arachnoid cysts are small and asymptomatic. They are usually located in the temporal fossa. Classically described complications result from compression of adjacent structures and include neurologic involvement, headaches and seizures. There are few cases of arachnoid cysts with ophthalmic manifestations reported in the literature. We describe two pediatric cases of arachnoid cyst with ocular complications. A 12-month-old girl with a history of acquired nystagmus and enlarged head circumference was referred for ophthalmologic examination. An MRI study showed a 7cm×7cm arachnoid cyst in the left temporal fossa. Six months after cystocysternostomy, the nystagmus resolved. A 2-year-old girl with esotropia and severe amblyopia of the left eye was referred for ophthalmologic examination. Optic nerve hypoplasia was detected on left fundus examination. MRI demonstrated a supracellar arachnoid cyst with mass effect on the optic chiasm as well as hydrocephalus. She was referred to neurosurgery for shunt surgery. In the case of clinical signs such as nystagmus, optic nerve hypoplasia, papilledema and oculomotor palsy, an arachnoid cyst may be a probable cause.

[Epithelial basement membrane dystrophy diagnosed following LASIK surgery]. / Dystrophie de Cogan révélée après chirurgie réfractive de type Lasik.

A 48-year-old woman with no significant past history underwent bilateral simultaneous laser in situ keratomileusis for correction of her myopia. On the tenth postoperative day, the patient complained of visual decrease and photophobia. Slit lamp exam showed corneal epithelial irregularities. Confocal microscopy was performed and revealed a characteristic appearance of epithelial basement membrane dystrophy (EBMD). The patient was successfully treated with artificial tears and autologous serum eyedrops. EBMD may be missed before LASIK surgery, even after a careful pre-operative examination. Exacerbation of EBMD after LASIK surgery is rare. It should be considered when unexplained corneal epithelial defects or irregularities occur following LASIK. Confocal microscopy is very useful to confirm the diagnosis.

[Unilateral isolated partial third nerve palsy and sphenoiditis in a child: A case report]. / Paralysie partielle du III isolée d'origine sinusienne chez un enfant : à propos d'un cas.

We report a case of unilateral mydriasis associated with sphenoid sinusitis and mucocele in a child. An 11-year-old girl with a history of unilateral mydriasis was referred for ophthalmologic examination. She complained of a mild headache over the past four days. The right pupil was dilated and nonreactive to light. The left pupil was normal and reactive. There was no ptosis or other focal neurological deficit. She was orthotropic. Visual acuity in both eyes, ocular motility and fundi were normal. Magnetic resonance imaging (MRI) showed a sphenoid sinus mucocele with sphenoiditis. The diagnosis was partial third nerve palsy without ophthalmoplegia. Treatment with antibiotics was initiated and led to complete resolution of the mydriasis. Sphenoid sinus mucoceles are relatively rare. Failure to diagnose and treat can lead to serious neurologic sequellae such as third nerve palsy, compressive optic neuropathy, cavernous sinus thrombosis, meningitis or brain abscess. Head imaging by reconstructed CT and MRI can lead to the diagnosis of mucocele. Isolated unilateral mydriasis as a sign of third nerve palsy may be caused by a slowly enlarging lesion. In a child with isolated unilateral mydriasis, head MRI should be performed to rule out a compressive lesion of the oculomotor nerve.

[Recurrence of an idiopathic vasocentric epiretinal membrane: clinical and surgical particularities]. / Récidive d'une membrane épirétinienne idiopathique dite « vasocentrique ¼: particularités cliniques et chirurgicales.

The vasocentric epiretinal membranes (ERM) are idiopathic ERM centered on retinal blood vessels, described mainly in young patients. We report a case of a 70-year-old patient who presented with a decrease in visual acuity secondary to a vasocentric epiretinal membrane. A successful vitrectomy and ERM removal were performed. Four years after surgery, a contractile ERM centered on the superior temporal blood vessel occurred and was associated with retinal distortions at the posterior pole. The second surgery combined removal of the recurrent ERM, which was adherent to the temporal vessels, and peeling of the internal limiting membrane in the macular area. Although there was visual recovery, the patient is still suffering from metamorphopsia 2 years after surgery. The vasocentric ERM have a poor visual outcome and a high risk of recurrence in comparison with other ERM disorders. This case report describes the main clinical and surgical characteristics of this type of membrane.

[Unilateral purpura of a limb revealing a periprosthetic abscess in an HIV-infected patient]. / Purpura unilatéral d'un membre révélateur d'un abcès périprothétique chez un patient infecté par le VIH.

BACKGROUND: Many diagnoses may be evoked in the presence of purpuric lesions of the lower limbs in HIV-positive patients. We report here on a Staphylococcus aureus abscess around a vascular prosthesis revealed by unilateral purpuric lesions. PATIENTS AND METHODS: A 43-year-old HIV-positive man was referred to us with febrile purpura of the left lower limb. His past medical history included a crossover iliofemoral arterial bypass. Clinical examination revealed fever and infiltrated purpuric lesions on the left ankle associated with cyanotic left toes. A skin biopsy showed leucocytoclastic vasculitis. A voluminous right iliac abscess was demonstrated by abdominal and pelvic CT scans. S. aureus was isolated from the skin biopsy, two blood cultures and the periprosthetic abscess. The abscess was drained without replacing the prosthesis and antibiotic therapy consisting of oxacillin was given for 6 months, resulting in complete healing. DISCUSSION: Vascular prosthetic infections are rare events that can occur early or late after surgery. S. aureus is usually the causative infectious agent. Clinical signs are non-specific and include purpuric lesions, which rarely reveal these infections. The organism may generally be isolated from prosthetic materials and blood cultures. CT scan is the recommended test to visualize prosthetic impairment. Treatment comprises prolonged antibiotic therapy adapted in accordance with the bacterial antibiogram, along with surgical debridement and, preferably, prosthetic replacement. CONCLUSION: This case report describes a unilateral purpura revealing a periprosthetic abscess. Dermatologists must be aware of this sign as a potential indicator of prosthetic infection.

[Intraocular foreign bodies (IOFB) of the posterior segment: retrospective analysis and management of 57 cases]. / Corps étrangers intraoculaires (CEIO) du segment postérieur : analyse rétrospective et prise en charge à propos de 57 cas.

PURPOSE: To determine the influence of prognostic factors and the type of tamponade agent in surgical management of intraocular foreign bodies (IOFBs) for better visual outcome. PATIENTS AND METHODS: Fifty-seven consecutive cases were retrospectively reviewed at the XV-XX National Hospital (Paris) between 1 January 2004 and 31 December 2007. Univariate and multivariate analyses were performed to identify prognostic variables. Several parameters were measured: pre- and postoperatively: best corrected visual acuity (BCVA), material and size of the foreign body, entry site, time from trauma to surgical removal, IOFB location, initial retinal detachment, choice of vitreous tamponade agent (none, gas, silicone oil), postoperative visual outcome, and complications (siderosis, endophthalmitis, and vitreoproliferative retinal detachment). RESULTS: Metal IOFBs accounted for the majority with 80.7% (n=46). The inferior retinal segment was the most frequent location found: 47.4% (n=27). Initial and final mean BCVAs were, respectively, 1.47 (±0.99) and 1.03 (±0.96) (LogMar scale). The mean follow-up was 20.7 months (range, 12-60 months). Initial retinal detachment was found in 24.56% (n=14), with a statically worse prognosis. The BCVA was better in the group with gas tamponade (n=16) than in the group with silicone tamponade (n=21) and the group without a tamponade agent (n=20). Initial BCVA was the most important predictive factor for final BCVA. Time to surgery was a predictive factor of final visual outcome with a cut-off in the first week. The scleral or corneoscleral entry site had a better prognosis than the corneal site. Five cases of siderosis (8.7%) related to delayed management and two cases of endophthalmitis (3.5%) were found (despite use of prophylactic systemic antibiotics). No statistical difference was found regarding the IOFB location on the retina, its size, or the material. Finally, the retina remained detached in nine cases (15.78%). A final BCVA of 20/40 or more was obtained in 39.3 and 17.5% had light perception or worse. CONCLUSION: The prognosis of an IOFB injury is for the most part uncertain due to a complex combination of parameters. Nevertheless, good postoperative results can be achieved without a silicone tamponade agent. The main prognostic factors related to better visual outcome were initial BCVA, time to surgery (first week), initially attached retina, and the scleral entry site. The main complications were vitreoproliferative retinal detachment, endophthalmitis, and siderosis. The location, type, and size of IOFBs were not statistically significant predictive factors in this study.

[Long-term results of the treatment of optic disc pit associated with serous macular detachment: a review of 20 cases]. / Résultats à long terme du traitement des fossettes colobomateuses de la papille compliquées de décollement séreux rétinien maculaire : à propos de 20 cas.

INTRODUCTION: The pathogenesis of the macular serous retinal detachment (SRD) associated with congenital optic disc pit remains controversial. The treatment is also discussed. Through this study, which includes the majority of the techniques available, we report our experiment in the treatment of this pathology. PATIENTS AND METHODS: This was a retrospective single-centre study of 20 patients who presented with macular SRD associated with optic disc pit between 1983 and 2009. Various treatments were provided. At the beginning of the study, patients were treated only by juxtapapillary laser photocoagulation. After laser failure then as first-line treatment, laser photocoagulation was associated with intravitreal gas (C3F8) injection with postoperative facedown positioning for 2 weeks. During the past few years, all patients have been systematically treated with vitrectomy with or without internal limiting membrane (ILM) peeling, laser, and gas (C2F6) tamponade. RESULTS: This series consisted of 20 patients: nine men and 11 women. The patients' mean age at presentation was 29 years (range, 9-60 years). The mean time between the onset of the decrease in visual acuity (VA) and treatment was 6.1 months. None of these patients had a posterior vitreous detachment at the time of diagnosis. Six patients were treated by laser photocoagulation alone, which was successful only in two cases. Eleven patients (with laser treatment failure in three) were treated by laser and intravitreal gas injection, with a 72% success rate. We performed vitrectomy with posterior hyaloid dissection, laser, and gas tamponade in eight cases (with laser-gas treatment failure in two) with 87% success rate and no recurrence. Five of these patients had ILM peeling during the vitrectomy. The mean follow-up period was 60 months (range, 2 months to 17 years). CONCLUSION: This study shows that early treatment of macular SRD associated with optic disc pit by vitrectomy, ILM peeling, juxtapapillary photocoagulation, and gas tamponade is followed by good anatomical and functional results. This treatment is superior to the other less invasive procedures. Optical coherence tomography is an important exam for diagnosis and postoperative follow-up of patients.

[Clinical, microbiological and therapeutic features of severe bacterial keratitis]. / Caractéristiques cliniques, microbiologiques et thérapeutiques d'une série de 111 kératites bactériennes sévères.

PURPOSE: To analyze risk factors and prognosis factors of severe bacterial keratitis. METHODS: Retrospective study of 111 eyes from 105 patients hospitalized from 2005 to 2006 for bacterial keratitis proven by microbiological assessment or suspected (favorable outcome after antibiotic treatment). RESULTS: The main risk factors were contact lens wear (39.6%), ocular surface diseases (36.9%), a history of ocular surgery (27.9%), and ocular trauma (11.7%). Gram-positive cocci were found in 46.8% of cases, Gram-negative bacilli in 19.8%, Gram-positive bacilli in 7.2%, Gram-negative cocci in 2.7%, and Gram-negative coccobacilli in 0.9%. No infectious agents were found in 22.5% of the cases. Two or more bacteria were found in 25.6%. The mean follow-up time was 6.5 months. Resolution of infection was obtained in 77.5% with only medical treatment and in 99.1% with further surgical treatment. Amniotic membrane transplantation was performed in 16.2% and emergency keratoplasty in 8.1%. The mean LogMAR visual acuity was 1.43 initially and 0.84 at the last examination. The final visual acuity was 1.03 for Gram-positive and 0.35 for Gram-negative organisms (p=0.03). CONCLUSION: Bacterial keratitis is a sight-threatening infection. Gram-positive keratitis is more frequent, except for contact lens wearers, and is also more severe.

[Rejection of corneal allografts]. / Le rejet d'allogreffe de cornée.

Corneal graft rejection is the major cause of penetrating keratoplasty failure. It is a complex immunological process that involves recognition of alloantigens from the corneal graft by the host's immune system, leading to an efferent immune response against the graft. Each layer of the cornea can undergo rejection, endothelial rejection being the most severe form. In some cases, rejection will lead to corneal graft failure. Many donor- and host-related risk factors contribute to corneal graft rejection. Corticosteroid therapy, topical or systemic, is the gold-standard in the preventive and curative treatment of rejection. Other immunosuppressive agents are promising but require further evaluation. Early detection of rejection is essential to establish an aggressive treatment and reduce the risk of graft failure. Prevention of rejection is also based on tissue matching between donor and recipient. In high-risk patients, ABO compatibility decreases the risk of rejection. HLA compatibility could positively influence corneal graft survival in some cases.