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Introduction: Poikilodermatous mycosis fungoides is a rare and indolent clinical variant of mycosis fungoides (MF). It can be difficult to distinguish from poikilodermatous parapsoriasis, a group of chronical dermatoses that may sometimes progress to MF. We aimed to specify the clinical, histopathological and developmental features of these entities by means of a retrospective study of 12 cases followed in our center., Patients and Methods: We identified cases of poikiloderma for which a diagnosis of MF or parapsoriasis was made by the physician. Photographs and histological slides were reviewed, and a final diagnosis of MF was made if the International Society for Cutaneous Lymphoma criteria for the diagnosis of early MF were fulfilled., Results: Twelve patients were included, 10 of whom met of the MF criteria. 5 patients had large poikilodermatous patches or thin, well-defined plaques ; 3 patients had the same lesions associated with classical MF lesions ; finally, 4 patients had widespread ill-defined erythematous lesions in a net-like pattern, described as parakeratosis variegata, including 3 MF. 2 patients with well-defined lesions (one associated with classical MF lesions) progressed to the tumoral stage whereas none of the patients with parakeratosis variegata presented such progression. A total of 5 patients had a high skin phototype (IV and V). Two patients had squamous cell carcinoma on poikilodermatous lesions., Discussion: Our study suggests that poikilodermatous MF covers a heterogeneous clinical spectrum comprising on one hand a presentation of delimited lesions sharing classical MF risk of progression, and on the other, an entity similar to parakeratosis variegata, an entity overlooked in the French nomenclature, which was particularly benign in our small series, raising the question of its affiliation to the MF group. This question merits further investigation in a larger-scale study., (Copyright © 2020 Elsevier Masson SAS. All rights reserved.)

Introduction: Streptococcal infections can cause various skin manifestations related to the direct action of the offending organism itself or to a reactional mechanism. Reactional manifestations are less well known and understood, and they include generalized acute pustulosis belonging to the spectrum of neutrophilic dermatoses. We report a case of generalized acute pustulosis followed by Sweet syndrome and erythema nodosum occurring after a streptococcal infection., Patients and Methods: A 60-year-old woman was consulting for a diffuse pustular rash after a throat infection, with high levels of anti-streptolysin (337 U/L) and anti-streptodornase (2560 U/L). The biopsy showed folliculitis and a neutrophilic infiltrator of the dermis, and bacteriological and mycological cultures were sterile. The patient then developed papules evoking Sweet syndrome followed by nodules typical of erythema nodosum after 20 days. A favourable outcome was achieved under colchicine., Discussion: Generalized acute pustulosis is a form of neutrophilic dermatosis whose mechanisms, area predilection and treatment are poorly known. The clinical presentation of this patient was initially typical and the secondary progression to lesions like those in Sweet syndrome is consistent with the pathophysiological continuity and overlap of these entities., (Copyright © 2019 Elsevier Masson SAS. All rights reserved.)

Background: Granuloma annulare (GA) is a benign granulomatous skin disorder that is generalized (GGA) in 15 % of cases. Although many case reports describe a relationship between GGA and systemic diseases, few large series have been published, and their association is debated. We present herein a series of GGA in order to describe their clinical and histological features., Patients and Methods: We included all biopsy-proven cases of GA presenting at the dermatopathology laboratory of Strasbourg where generalized (i.e. over 10 lesions). Clinical features were obtained from patients' medical files., Results: We included 35 GGA, with a sex ratio of 0.5. The mean age was 54 years. Lesions were annular or non-annular in equal measure and were symptomatic in 25 % of cases. Most patients (77 %) had an associated disease, already known in 60 % of cases, including dyslipidemia (27 %), diabetes mellitus (20 %), immunosuppressive drugs (17 %), atopy (17 %), auto-immune disease (17 %), hematological disease (14 %), and cancer (9 %). Histological analysis revealed the predominant pattern to be interstitial (54 %) rather than palisading (20 %), having no correlation with clinical type. Eosinophils were frequent (46 %) in GA but were not correlated with systemic disease or drug taking. Among the 40 % of patients treated, 50 % had a successful outcome on topical corticosteroids, doxycycline, antimalarial drugs or phototherapy., Discussion: GGA differs from localized GA, which is mostly associated with an already known systemic disease, whether metabolic, infectious or neoplastic, uncorrelated with clinical or histological features, and screening is necessary., (Copyright © 2020 Elsevier Masson SAS. All rights reserved.)

Introduction: For inflammation of a tattoo occurring decades after its creation, sarcoidosis should be considered first of all. Two case of extremely delayed hypersensitivity tattoo reaction have been recently reported in patients treated with BRAF and MEK inhibitors. We report a similar new case strongly suggesting a specific effect of this drug combination., Patients and Methods: A 58-year-old man bearing 20-year-old tattoos was treated with dabrafenib and trametinib for advanced melanoma. A painful erythematous swelling appeared on all the patient's tattoos two months later, while his general tolerance of the treatment was poor. Skin biopsy demonstrated perivascular lympho-histiocytic infiltrate without granuloma, but with prominent pigment-loaded macrophages. Inflammatory signs quickly regressed after drug discontinuation., Discussion: Great similarity exists between this new case and the first reported case, in which a female patient presented painful infiltration of old tattoos following repeated reintroduction of dabrafenib and trametinib in a setting of advanced melanoma. The immunomodulatory properties BRAF/MEK inhibition may enhance loss of tolerance to tattoo ink, accounting for the extremely long time to reaction. This third case militates in favour of a specific drug-induced reaction, of which patients with tattoos should be informed when anti-BRAF/MEK therapy is being considered., (Copyright © 2019 Elsevier Masson SAS. All rights reserved.)

The key diagnostic tool for hyperpigmentation is histopathology, which may be accompanied by certain laboratory tests. Hyperpigmentation may result from excess melanin (hypermelanosis), cutaneous iron deposits (hemosiderosis), cutaneous carotene deposits (carotenoderma), or cutaneous deposits of a substance not normally found in the skin (dyschromia). The different types of hypermelanosis may be classified as either localised or generalised. The former generally correspond to skin tumours and may form a cutaneous expression of complex syndromes, which most notably include cardiac abnormalities, or to pigmented forms of inflammatory and/or infectious dermatoses. Diffuse hypermelanosis is frequently a sign of systemic disease, generally metabolic or endocrine disease, or else it may result from pharmaceutical therapy. Herein we review the various causes of hyperpigmentation and the corresponding therapy., (Copyright © 2019 Elsevier Masson SAS. All rights reserved.)

Introduction: Impairment of dermal elastic tissue occurs in different entities associated with immunoglobulins or immunoglobulin-derived protein-secreting clonal plasma cell proliferations, such as amyloid elastosis, anetodermic nodular amyloidosis or monoclonal gammopathy-associated cutis laxa. We report a case of cutaneous immunoglobulinemic amyloidosis revealed by a unique chalazodermic presentation and we review elastic tissue impairment in patients with monoclonal gammopathies., Observation: A 67-year-old woman consulted for non-infiltrated anetodermic lesions on the upper left quadrant of her abdomen present for ten years. She also had a chalazodermic plaque with abnormal skin wrinkling and laxity in her right axilla. Biopsies revealed deep dermal and subcutaneous amyloid deposits. Immunohistochemistry with lambda light chain was positive. Orcein staining and electron microscopy showed extensive elastolysis. The patient presented no signs of systemic involvement, but a very small amount of monoclonal IgGλ gammopathy was detected during follow-up., Discussion: This is a unique chalazodermic presentation of immunoglobulinemic amyloidosis that does not fit into a clearly-defined nosological setting. It highlights the complex interactions between immunoglobulin-derived proteins, including light and heavy chains, and elastic tissue components, leading to different types of impairment of the latter. We therefore suggest the unifying concept of immunoglobulinemic elastopathy, underscoring the need to screen for monoclonal gammopathy in patients presenting elastic tissue impairments., (Copyright © 2018. Published by Elsevier Masson SAS.)

Background: Subcutaneous glatiramer acetate, commercialized under the name of Copaxone ® , is licensed for the treatment of relapsing multiple sclerosis. Its major adverse effects are skin reactions at the injection site. Nicolau syndrome is a rare but serious iatrogenic accident. Herein we report a case seen in a setting of change of dosage and administration rate of Copaxone ® ., Patients and Methods: A 64-year-old woman, treated since 2010 with daily sub-cutaneous injections of Copaxone ® 20mg/L, reported the appearance of a painful, indurated and erythematous plaque in the suprapubic area following changeover to 40mg/mL injections three times weekly. The suprapubic injections were continued and ugly greyish spots with stellate purpuric borders appeared. Fournier gangrene was ruled out by means of a soft tissue scan., Discussion: We report this latest case of Nicolau syndrome to alert readers to the non-exceptional nature of this complication associated with use of glatiramer acetate, particularly at a dosage of 40mg/L injections three times weekly. In our case, onset of Nicolau syndrome appears to have been favored by continued injection in areas already showing inflammation. Re-injection of the drug in these areas should thus be proscribed., (Copyright © 2018 Elsevier Masson SAS. All rights reserved.)

Competing Interests: C. Lenormand déclare avoir perçu de manière ponctuelle une rémunération sous forme d’honoraires de la part des laboratoires BMS, Zambon et Galderma, pour des interventions en tant qu’orateur au cours de séances de FMC.

Brachytherapy is a possible treatment for localized low risk prostate cancer. Although this option is minimally invasive, some side effects may occur. Acute retention of urine (ARU) has been observed in 5% to 22% of cases and can be prevented in most cases by alpha-blocker treatment. Several alternatives have been reported in the literature for the management of ARU following brachytherapy: prolonged suprapubic catheterization, transurethral resection of the prostate and also intermittent self-catheterization. The authors report an original endoscopic approach, using urethral endoprosthesis, with a satisfactory voiding status., (Copyright © 2013. Published by Elsevier Masson SAS.)

Background: IL-1 antagonists are used in the treatment of patients with rheumatoid arthritis and cryopyrinopathies. As yet anecdotal observations suggest that they may allow effective treatment of patients with different types of inflammatory skin disease. This review focuses on our current knowledge of the use of IL-1 antagonists in dermatology., Patients and Methods: A Medline search was performed combining the keywords: "anakinra; canakinumab; rilonacept" AND "skin; neutrophilic dermatoses; Sweet syndrome; pyoderma gangrenosum; hidradenitis suppurativa; Schnitzler syndrome; Still disease". The precise dermatological phenotype of patients with IL-1 antagonist-responsive auto-inflammatory disorders was analysed in order to compare it to related complex disorders., Results: Double-blind randomized controlled trials have demonstrated the efficacy of these treatments in cryopyrinopathies with dermatological involvement including chronic infantile neurological cutaneous and articular (CINCA) syndrome, Muckle-Wells syndrome and familial cold urticaria. Anakinra is the only treatment for Schnitzler syndrome that is almost constantly efficacious, even in refractory disease, as attested by numerous case reports. It is also efficacious in the treatment of patients with adult-onset Still disease and systemic juvenile arthritis. Neutrophilic dermatoses constitute the cutaneous hallmark of IL-1-responsive auto-inflammatory disorders, and neutrophilic dermatoses could thus form an indication for this treatment. However, to date, only 9 reports have been published showing efficiency in patients with Sweet syndrome, in one case of neutrophilic panniculitis, and in two cases of pustular psoriasis. Anakinra appears less efficacious in patients with pyoderma gangrenosum., Conclusion: IL-1 antagonists are a first-line treatment in patients with Schnitzler syndrome and cryopyrinopathies. They could become important alternatives in patients with acute and febrile neutrophilic dermatoses either unresponsive to or with contraindications to conventional treatments, but this requires confirmation by further clinical trials., (Copyright © 2012 Elsevier Masson SAS. All rights reserved.)

Transoesophageal echocardiography has shown a high incidence on non-obstructive thrombosis after mitral valve replacement with a mechanical prosthesis. The unpredictable outcome and the period during which the complication arises make treatment difficult. The aim of this study was to assess the tolerance and efficacy of the association of long-term heparin and oral anticoagulation, as recommended in this indication. All patients undergoing mitral valve replacement with a mechanical prosthesis between June 1999 and July 2001 were systematically included and studied by transoesophageal echocardiography in the immediate postoperative period. Those with non-obstructive thrombosis at least 5 mm in size were treated by heparin and oral coagulation until the thrombus disappeared on transoesophageal echocardiography. One hundred and fourteen patients undergoing 120 mitral valve replacements (6 reoperations) underwent transoesophageal echocardiography and non-obstructive thrombi measuring at least 5 mm were found on 26 occasions (21.7%). The association of heparin and oral coagulation was maintained for 7 to 115 days (average 20 days). No thromboembolic or haemorrhagic complications and no deaths were observed during this period. Two patients were treated with danaparoid and oral anticoagulation because of heparin-induced thrombocytopenia before the diagnosis. None of the patients died during follow-up (average 49 months); there were 4 recurrent non-obstructive thromboses, three of which were complicated by thromboembolic events with no sequellae in the first 8 months, again treated effectively with the association of heparin and oral anticoagulants; two cerebral embolic events without sequellae were observed without a demonstrable non-obstructive thrombus on transoesophageal echocardiography. The authors conclude that the association of heparin and oral anticoagulants seems well tolerated and effective in this small population and this would justify a large scale clinical trial.

The authors report two cases of pulmonary valve endocarditis which required emergency surgical treatment. A 74 year old patient with trivalvular endocarditis (pulmonary, aortic, mitral), due to Sptreptococcus D bovis, developed cardiogenic shock with acute pulmonary oedema and underwent double aortic and pulmonary valve replacement with Carpentier-Edwards prostheses and simple resection of a mitral valve vegetation. Another 36 year old drug addict developed isolated pulmonary valve endocarditis due to Staphylococcus aureus infection complicated by pulmonary regurgitation with right ventricular failure and by septic pulmonary embolism with persistent sepsis: he underwent pulmonary valve replacement with a Bravo 300 bioprosthesis. The postoperative course was uncomplicated in both cases, with interruption of the infection and normalisation of the haemodynamic status. The insidious and severe nature of pulmonary valve endocarditis is demonstrated by these two cases, confirming previous reports which have underlined the poor prognosis of this condition. Surgery has been shown to be effective and well tolerated and should be integrated early in the therapeutic strategy, the results being all the better when an aggressive attitude is taken.

In order to assess the prevalence of cardiac involvement in the primary antiphospholipid syndrome (PAS), a syndrome which associates thromboembolism, recurrent abortion, the presence of antiphospholipid antibodies and thrombocytopenia, transthoracic (TTE) and trans-esophageal echocardiography (TEE) was performed in 15 patients, 10 women and 5 men with a mean age of 38.8 +/- 11 years, with the PAS but without systemic lupus erythematosus. The presentation of the PAS was a thrombotic event (6 arterial and 7 venous) in 13 cases, and recurrent abortion in 3 cases. Twelve patients had high anticardiolipin antibody levels (> or = 15 U GPL) and 12 had a raised anti-prothrombinase antibody title. Valvular heart disease was detected in 9 patients (60%) as a valve thickening (> or = 5 mm for the mitral and > or = 3 mm for the aortic valve) or nodule. Mitral regurgitation was observed in 4 cases both on TTE and TEE and was mild in 3 cases and severe in 1 case. Aortic regurgitation was diagnosed in 6 patients, in 3 cases by TTE and in 6 cases by TEE. It was mild in 5 cases and moderate in the other cases. Pericardial effusion was observed in 3 patients (20%), alone in 1 case and associated with valvular disease in the other two cases. No abnormality of left ventricular systolic or diastolic function could be demonstrated. In conclusion, cardiac involvement seems to be common in the PAS, and TEE is a sensitive and accurate method for describing the valvular, especially aortic valve, abnormalities.

The aim of this study was to evaluate the continuity equation in the quantification of mitral valve area in mitral stenosis, the area being considered as the product of the area of the left ventricular outflow tract multiplied by the ratio of the velocity time integrals of the aortic or pulmonary flow to that mitral flow. The continuity equation was compared to two other echocardiographic methods, planimetry and Hatle's method, and to the results obtained at catheterization using the Gorlin formula in a population of 44 patients with mitral stenosis. All were in sinus rhythm; twelve had Grade I mitral regurgitation and 9 patients had Grade I aortic regurgitation. Excellent correlation were observed between the values obtained by the continuity equation and planimetry (r = 0.91; SEE = 0.19 cm2; p less than 0.001) and Hatle's method (r = 0.87; SEE = 0.20 cm2, p less than 0.001). The correlation with the catheter values were also excellent (r = 0.83; SD = 0.22 cm2, p less than 0.001), better than those observed with Hatle's method (r = 0.73; SEE = 0.27 cm2, p less than 0.001) and very similar to those obtained with planimetry (r = 0.87; SEE = 0.23 cm2, p less than 0.001). The sensibility and specificity of the continuity equation for the diagnosis of severe mitral stenosis (surface less than 1.5 cm2) were 90% and 100% respectively, when those of Hatle's method were 88% and 91% respectively. The continuity equation in the evaluation of mitral valve area in mitral stenosis seems to be reliable and accurate compared with catheter data, and superior to Hatle's method.