[The five more frequent inflammatory vulvar lesions]. / Les cinq lésions inflammatoires les plus fréquentes en anatomie pathologique vulvaire.

Lichen sclerosus, lichen planus, psoriasis and plasma cell vulvitis are the most common diagnosis amongst inflammatory vulval diseases, the most frequently suspected by the clinicians and the most frequently diagnosed by the pathologist. We expose their clinical and pathological aspects and detail the most recurrent diagnosis difficulties.

Vulvar Fordyce adenitis: A cohort of 45 women.

BACKGROUND: Recurrent episodes of painful papules and nodules, mostly located on the labia minora, have been reported under the denominations of vulvar sebaceous adenitis (14 published cases) and vulvar acne (16 published cases). OBJECTIVES: The primary aim of this study was to delve further into the clinical and pathological features of this condition. The secondary aim was to collect therapeutic data. METHODS: In this retrospective cohort study, files and photographs of patients with papules or nodules on the labia minora or the inner labia majora were extracted from a vulvar clinic database. Clinical, pathological and therapeutic data were analysed. RESULTS: Forty-five women were included from 2002 to 2018. The median age at the time of diagnosis was 36 years (range: 16-60). The median time to diagnosis was 6.5 years. Clinical features included recurrent painful papules, pustules or nodules, suppuration (n=22), and pitted scars (n=10) on the labia minora (n=41), the inner labia majora (n=19), the outer labia majora (n=1), and the clitoral hood (n=1). Associated acne vulgaris was seen in 17 out of 26 patients for whom data were available. Hidradenitis suppurativa, androgenic alopecia and hirsutism were observed in 3, 1 and 1 cases respectively. Neutrophilic infiltrates were observed in the 4 available biopsies within or around the Fordyce sebaceous glands in 2 patients. Treatment with tetracyclines and oral zinc was not consistently effective. Isotretinoin led to complete remission in 4 patients unresponsive to tetracyclines. DISCUSSION: Vulvar sebaceous adenitis/vulvar acne is a clinically identifiable cause of painful recurrent inflammatory lesions affecting the labia minora and the inner labia majora, which are coincidentally sites of Fordyce granules. We therefore suggest a more specific denomination: "vulvar Fordyce adenitis". The relationship with acne vulgaris and hidradenitis suppurativa requires further investigation. We suggest use of an oral anti-acne therapeutic strategy to reduce inflammation and to decrease the secretion of the sebaceous glands.

[Eruptive granuloma after injection of Ellansé® successfully treated using methotrexate]. / Granulomes après injection d'Ellansé®, résolutifs sous méthotrexate.

BACKGROUND: Collagen stimulators such as Ellansé® are soft tissue fillers able to induce nucleogenesis. We describe a case of eruptive foreign body granulomas following injection of Ellansé® that were successfully treated using methotrexate. CASE REPORT: A 47-year-old woman received injections of Ellansé® for the wrinkled aspect of her cheeks. She had previously undergone injections of hyaluronic acid on the nasolabial folds. Nine months after the Ellansé® injections, the patient consulted for the recent appearance of multiple nodules on her face. Histological analysis of one of these nodules confirmed the presence of foreign-body granulomas developed in contact with spherical gaps of a size substantially identical to the Ellansé® vacuoles. Methotrexate 10mg per week for 3 months followed by 20mg per week for 9 months resulted in complete regression of the nodules. DISCUSSION: Ellansé® is composed of two biocompatible and bioabsorbable polymers: carboxymethylcellulose, responsible for immediate volume creation, and polycaprolactone, which promotes collagen synthesis. However, any injected product can cause varying degrees of granulomatous reaction. Hyaluronic acid was previously injected at several other sites on the patient's face. These lesions were not the result of poor injection technique. CONCLUSION: Although collagen stimulators are biocompatible and bioabsorbable substances, the development of foreign-body granulomas, while rare, is still possible. Methotrexate resulted in significant regression of nodules as of the third month of treatment.

[Anal and vulvar hidradenoma papilliferum are similar: A study of 14 cases]. / Les hidradénomes papillifères de l'anus ressemblent à ceux de la vulve : étude de 14 cas.

INTRODUCTION: Hidradenoma papilliferum (HP) is an adenomatous proliferation of mammary-like glands. These glands are located preferentially on the vaginal labia, the perineum and the anal skin. About ninety percent of HP occur on the vulva, with anal localization being much less common. AIM OF THE STUDY: To analyze the clinical and histological characteristics of anal HP and compare them to those seen on the vulva based on the literature. METHOD: A monocentric retrospective analysis (in the medical and surgical proctology department of the Saint-Joseph Hospital Group, Paris) of patients for whom a diagnosis of anal HP was made based on pathological analysis of a resected sample. RESULTS: A total of 14 female patients were included between 2012 and 2018. The mean age was 48.2 years (22-70). The tumor, single in all cases, was asymptomatic with very slow progression. It was located on the anal skin in all patients. It generally consisted of a round nodule measuring under 1cm that was barely prominent, translucent, depressible and mobile. In 15% of patients the tumor was ulcerated. Histologically, the tumor displayed the usual characteristics of HP. Only one relapse was seen, six years after resection. CONCLUSION: The clinical and histological aspects of anal HP are the same as those of the vulva, which are better documented, namely a small, rounded, pink, translucent or bluish, and mobile tumor, with a smooth surface, and more rarely ulceration or budding, and in most cases asymptomatic. All HP were diagnosed in women and were located at the anal margin. Histopathological examination of an excised sample confirms the diagnosis and rules out a malignant tumor. The frequency of anal HP may be underestimated.

[Miescher's granulomatous vulvitis]. / Vulvite granulomateuse de type Miescher.

BACKGROUND: Miescher's cheilitis, whether occurring alone or as part of Melkersson-Rosenthal syndrome, is a rare type of granulomatous inflammation of unknown cause with an even rarer genital equivalent. Herein, we describe a case of the latter condition developing over a 12-year period. PATIENTS AND METHODS: A 27-year-old woman presented episodes of unilateral vulvar oedema, which initially regressed but resulted over time in permanent vulvar and perineal tumefaction. Histology revealed epithelioid histocytic granulomas so mild that they were not noticed at the start of the disease. The oedema remained the sole sign for 12years and two gastrointestinal screening tests for Crohn's disease proved negative, diagnosis of the latter condition was ruled out. DISCUSSION: Miescher's granulomatous vulvitis requires differential diagnosis, essentially with regard to Crohn's disease. Our case illustrates the difficulty in diagnosing this rare disease as well as the uncertainties surrounding its physiopathology. Diagnosis rests upon repeated sampling and biopsies with repeated levels of sections in order to identify the characteristic perivascular granulomas, which may be very mild.

Thermoresponsive gold nanoshell@mesoporous silica nano-assemblies: an XPS/NMR survey.

This work provides a detailed study on the physico-chemical characterization of a mechanized silver-gold alloy@mesoporous silica shell/pseudorotaxane nano-assembly using two main complementary techniques: XPS and NMR (liquid- and solid-state). The pseudorotaxane nanovalve is composed of a stalk (N-(6-aminohexyl)-aminomethyltriethoxysilane)/macrocycle (cucurbit[6]uril (CB6)) complex anchored to the silica shell leading to a silica/nanovalve hybrid organic-inorganic interface that has been fully characterized. The stalk introduction in the silica network was clearly demonstrated by XPS measurements, with the Si 2p peak shifting to lower energy after grafting, and through the analysis of the C 1s and N 1s core peaks, which indicated the presence of CB6 on the nanoparticle surface. For the first time, the complex formation on nanoparticles was proved by high speed (1)H MAS NMR experiments. However, these solid state NMR analyses have shown that the majority of the stalk does not interact with the CB6 macrocycle when formulated in powder after removing the solvent. This can be related to the large number of possible organizations and interactions between the stalk, the CB6 and the silica surface. These results highlight the importance of using a combination of adapted and complementary highly sensitive surface and volume characterization techniques to design tailor-made hybrid hierarchical structured nano-assemblies with controlled and efficient properties for potential biological purposes.

[Angiomatoid fibrous histiocytoma in children: 6 cases]. / Histiocytome fibreux angiomatoïde de l'enfant : 6 cas.

BACKGROUND: Angiomatoid fibrous histiocytoma (AFH) is a soft-tissue tumour of uncertain differentiation most often arising in the extremities of children and young adults. AFH is a little-known neoplasm and its rarity may result in it being misdiagnosed as either a reactive lesion or a benign or higher-grade tumour. We report 6 cases of AFH in children and we review the clinicopathological and molecular features of this neoplasm published in the literature. PATIENTS AND METHODS: The children (aged 4 to 16 years) presented a single nodule involving the forearm (4/6), the trunk or the buttock, and all 5 nodules appeared spontaneously. Microscopic examination revealed well-circumscribed nodular lesions comprising a fibrous pseudo-capsule, haemorrhagic non-endothelial-lined pseudocystic spaces, and sheets of spindle and ovoid cells with dense surrounding lymphoplasmacytic infiltrate. Tumours were positive for desmin, CD68, CD99 and smooth-muscle actin markers. A fusion gene (EWSR1-ATF1) was found in the 3 cases in which molecular investigation was performed. DISCUSSION: In our series, a diagnosis of AFH had in no event been evoked after clinical examination and radiological investigation. The diagnosis was based in all cases on recognition of characteristic features during histological examination and it was confirmed in 3 cases by the recognition of fusion genes. Complete excision with wide margins allowed complete cure in all cases, supporting a good prognosis of AFH, although long-term follow-up is still mandatory to rule out relapse or metastases, which although rare, are responsible for fatal cases. To avoid unnecessary surgery in patients with AFH, an ultrasound core-needle biopsy should be performed as a first step in order to provide precise diagnosis enabling complete excision to be performed, with the margins being decided in multidisciplinary meetings involving teams specialised in soft-tissue tumours.

[Lipoatrophic panniculitis in adults: treatment with hydroxychloroquine]. / Panniculite lipoatrophiante de l'adulte : traitement par hydroxychloroquine.

BACKGROUND: Lipoatrophic panniculitis is generally considered to be a rare disease affecting children. We report a case involving this condition in an adult patient presenting with striking clinical features and responding to hydroxychloroquine therapy. We discuss the nosological relationship between lipoatrophic panniculitis and connective tissue panniculitis. PATIENTS AND METHODS: A 62-year-old woman was referred to our institution with a six-month history of painful erythematous nodules and plaques on the calves, thighs, buttocks, breasts, abdomen and arms. With each outbreak of new lesions, she felt unwell and experienced fever, chills and sweating. After a few weeks, the lesions progressed circumferentially and led to large areas of subcutaneous atrophy showing a central depression covered with a yellowish, supple skin and surrounded by an annular, infiltrated, erythematous and tender inflammatory margin. Deep subcutaneous biopsy specimens showed typical features of lobular and septal lipophagic panniculitis, with a dense inflammatory infiltrate composed of large histiocytes, multinucleated giant cells and few neutrophils, without vasculitis. The patient was started on hydroxychloroquine 400 mg daily. Three weeks later, her pain and tenderness had completely resolved and the inflammatory margin of the lesions had clearly regressed. DISCUSSION: This unique condition, with distinctive clinical and histological features, is similar to cases described under the term "lipophagic panniculitis", seen mostly in children, but also "connective tissue panniculitis". Their clinical resemblance and response to hydroxychloroquine therapy leads us to think that these two entities, previously subsumed under the eponym of Weber-Christian disease or Rothman-Makai syndrome, are closely related. CONCLUSION: Dermatologists and dermatopathologists should be made aware of this unusual entity, and of the fact that it can arise in adult patients, so that they may make an early diagnosis and thus prevent the unsightly consequences of lipoatrophy.

Analysis of the orientational order effect on n-alkanes: Evidences on experimental response functions and description using Monte Carlo molecular simulation.

Short-range correlations of the molecular orientations in liquid n-alkanes have been extensively studied from depolarized Rayleigh scattering and thermodynamic measurements. These correlations between segments induce structural anisotropy in the fluid bulk. This phenomenon, which is characteristic of linear chain molecules when the constituting segments are nor freely jointed, but interact through a given angular potential, is then present in the linear n-Cn series, increasing its magnitude with chain length, and it is therefore less relevant or even completely absent in branched alkanes. This intermolecular effect is clearly revealed in second-order excess magnitudes such as heat capacities when the linear molecule is mixed with one whose structure approaches sphericity. The mixing process of different aspect ratio chain molecules is thought to modify the original pure fluid structure, by producing a diminution of the orientational order previously existing between pure n-alkane chains. However, second-order thermodynamics quantities of pure liquids C(P), ( partial differentialv/ partial differentialT)(P), and ( partial differentialv/ partial differentialP)(P) are known to be very sensitive to the specific interactions occurring at the microscopic level. In other words, the behavior of these derived properties versus temperature and pressure can be regarded as response functions of the complexity of the microscopic interactions. Thus, the purpose of the present work is to rationalize the orientational order evolution with both temperature and molecular chain length from the analysis of pure fluid properties. To this aim, we focused on two linear alkanes, n-octane (n-C(8)) and n-hexadecane (n-C(16)), and two of their branched isomers, i.e., 2,2,4-trimethylpentane (br-C(8)) and 2,2,4,4,6,8,8-heptamethylnonane (br-C(16)). For each compound, we propose a combined study from direct experimental determination of second-order derivative properties and Monte Carlo simulations. We performed density rho, speed of sound c, and isobaric heat capacity C(P) measurements in broad ranges of pressure and temperature allowing a complete thermodynamic characterization of these compounds. Monte Carlo simulations provide a link between the molecular scale model and the experimental thermodynamic properties. Additional information about the microscopic structure of the simulated fluid model was derived, through the calculation of the radius of gyration and average end-to-end distances. Orientational order is clearly revealed by the experimental residual heat capacity trend of pure linear alkanes. The close agreement observed between this experimental macroscopic property and the calculated theoretical structural parameters support the conclusion that the orientational order between segments of linear molecules should be regarded as a conformational effect due to the flexibility of the chain.