Androgen receptor expression in epidermal and adnexal tumours.

INTRODUCTION: Androgen receptor (AR) immunohistochemistry is used in general pathology and in dermatopathology, particularly for sebaceous tumours. The goal of this study was to quantify AR expression in benign and malignant epidermal tumours and adnexal tumours. METHODS: We studied AR expression in 301 skin lesions using standard immunohistochemistry and compared 10 trichoblastomas, 10 sebaceomas and 10 hidradenomas using 5 markers (cytokeratin 7 and 8, PHLDA1, BerEp4 and AR). RESULTS: The rates of AR expression were: 22% in basal cell carcinomas, 3% in squamous cell carcinomas, 92% in sebaceous tumours, 10% in follicular tumours and 22% in sweat gland tumours. Benign sebaceous tumours were AR+ in 97% of cases. Only 12% of sebaceous carcinomas showed no AR staining. The immunohistochemical profiles of the comparative study were as follows: sebaceoma: AR+, CK7-, CK8-, PHLDA1-, BerEp4-; hidradenoma: AR-, CK7+, CK8+, PHLDA1+, BerEp4+; trichoblastoma: AR-, CK7-, CK8-, PHLDA1+, BerEp4+. DISCUSSION: AR staining was positive in 92% of sebaceous tumours, including sebaceomas, in some cases indicative of Muir-Torre syndrome. AR staining is therefore highly sensitive for the diagnosis of sebaceous tumours, but it is non-specific and is best used in combination with other antibodies, notably anti-CK8 and PHLDA1, particularly to distinguish sebaceoma from hidradenoma or trichoblastoma.

[A case of cutaneous tuberculosis evolving for 50 years]. / Un cas de tuberculose cutanée évoluant depuis plus de 50 ans.

INTRODUCTION: Tuberculosis is an infection caused by Mycobacterium (M.) tuberculosis. It is rare in France. Clinical presentations vary, making demonstration of the cause of M. Tuberculosis difficult and rendering diagnosis and management difficult. PATIENTS AND METHODS: A 58-year-old man, born in Morocco, consulted for ulceration of the right forefoot that had been present since the age of 3 years. He had previously consulted at several dermatology departments. He had undergone numerous skin biopsies and bacteriological and mycobacteriological cultures but these did not contribute to the diagnosis. Slow extension and oozing were observed over time and resulted in functional disability. Given the evocative clinical aspect and despite further negative screening for mycobacteria, anti-TB quadrotherapy was prescribed and resulted in complete cure of the lesion. DISCUSSION: This case underscores the difficulty of diagnosing cutaneous tuberculosis. Such a diagnosis must be clinically suspected in the presence of long lasting destructive or verrucous skin lesions that fail to heal, even where cultures are negative, and anti-TB therapy should be putatively prescribed.

Épidémiologie des kératoses actiniques: Epidemiology of actinic keratosis.

Actinic keratosis is a premalignant skin lesion resulting from proliferation of atypical epidermal keratinocytes. Actinic keratoses are very frequent and their prevalence is increasing. Risk factors for actinic keratosis include intrinsic and environmental factors, particularly exposure to ultraviolet radiation and advanced age. The main factor is the exposition to ultraviolet radiation. Better sun protection decreases the risk of actinic keratosis and also the risk of progression to squamous cell carcinoma, even though not all actinic keratoses progress to invasive squamous cell carcinoma. A diagnosis of actinic keratosis should encourage patients to do an annual dermatological screening of skin cancers. Given the economic cost of actinic keratoses, a global approach of health authorities could be interesting for their management. © 2019 Elsevier Masson SAS. All rights reserved. Cet article fait partie du numéro supplément Kératoses actiniques : comprendre et traiter réalisé avec le soutien institutionnel de Galderma International.

Place de la radiothérapie dans le traitement du carcinome basocellulaire: Radiation therapy for basal cell carcinoma.

Radiotherapy is an available but not well-known treatment for management of basal cell carcinoma. National organizations have established that standard therapy is complete surgical removal and radiation therapy is an option for inoperable tumors or those where the post-operative defect would be cosmetically disfiguring or functionally disabling. These therapeutic options, with histological sample, should be considered in the multidisciplinary management of patients with basal cell carcinoma. There are several types of radiotherapy: external radiation or interstitial brachytherapy, unfortunately, there is no consensus in the literature and the range of radiation regimens in common use is large. Very few randomized studies have been conducted to defi the optimum treatment in terms of recurrence rate, cosmetic outcome and side-eff In most of studies, the overall local control rate was between 80-100% and over 90% of patients reported good or excellent cosmetic outcome. Side-eff of radiotherapy most commonly reported are minor but in young patients one must be alert to the theoretical possibility of the induction of secondary malignancies. Cet article fait partie du numéro supplément Prise en charge des carcinomes basocellulaires difficiles à traiter réalisé avec le soutien institutionnel de Sun Pharma.

[Rapidly growing squamous cell carcinoma after ingenol mebutate treatment]. / Carcinome épidermoïde de croissance rapide après traitement par mébutate d'ingénol.

INTRODUCTION: Ingenol mebutate is an actinic keratosis treatment, which has a dual action mechanism. It allows a rapid cellular death and a severe inflammation. OBSERVATION: We report the case of a 75 years old patient with a rapidly growing tumor 5 weeks after application of ingenol mebutate on typical actinic keratosis. Histological analysis after surgical excision showed an invasive squamous cell carcinoma (SCC); with aggressiveness signs: perineural infiltration and vascular permeation. DISCUSSION: Ingenol mebutate's common side effects are benign and regressive within 2 to 4 weeks. There are erythema, edema, crusts, and ulcerations/erosions. Squamous cell carcinoma development was rarely reported. We have tried to collect other cases in the literature and in pharmacovigilance centres: three similar cases were recently published in the literature, 21 cases were notified to the European Medicines Agency and we asked French pharmacovigilance centres and found 5 cases of SCC after ingenol mebutate application. The role of the molecule in SCC development is currently unknown. Induced inflammation could take part in the development of these tumors. We compare this case with other situations of inflammation, such skin graft donor site or surgical incision, complicated of rapidly growing SCC. Our case, literature's and pharmacovigilance's cases encourage us to follow ingenol mebutate's side effects. Careful follow-up and registration of such cases are important to gain further insight on this topic.

Amicrobial pustulosis of the folds: Where have we gone 25years after its original description?

BACKGROUND: Twenty-five years ago at the Journées Dermatologiques de Paris, Prof. Béatrice Crickx described a new association, "antimicrobial pustulosis and systemic lupus erythematosus", a condition now known as amicrobial pustulosis of the folds (APF). The aim of this study is to analyse the clinical and laboratory characteristics of APF and to outline the gradual advancement of knowledge regarding this disease. MATERIALS AND METHODS: Based on a case of APF seen in our department, we carried out a review of the literature since 1991 by searching the Medline database for scientific articles using the following keywords: "Amicrobial Pustulosis" or "Pustular Dermatosis" and "Folds". RESULTS: We collated 63 cases of APF. In total, 90% involved women and the mean age was 30 years. Cutaneous lesions consisted of pustules in an erythematous setting, frequently erosive and affecting the skinfolds, anogenital region, scalp, external auditory meatus and umbilicus. Histological examination revealed spongiform subcorneal pustulosis associated with mixed inflammatory infiltrate in the dermis. DISCUSSION: APF belongs to the spectrum of neutrophilic dermatoses. Its complex physiopathology involves an auto-inflammatory mechanism. It is associated with a variety of autoimmune diseases, in most cases systemic lupus erythematosus. Diagnosis of the condition is difficult and is based on histological correlation and negative microbiological culture. The most effective treatment is still systemic corticosteroids, although new therapeutic alternatives are emerging such as anakinra and anti-TNF-alpha drugs.

[Muir-Torre syndrome and Turcot syndrome]. / Syndrome de Muir-Torre et syndrome de Turcot.

INTRODUCTION: Lynch syndrome (LS) is a syndrome that carries a genetic predisposition to certain cancers associating, either in a single individual or in a family, a visceral tumour, mainly colorectal, with a high risk of other synchronous or metachronous cancers. LS is linked with mutations in the genes coding for proteins in the DNA repair system. Phenotypic variants of SL exist, including Muir-Torre syndrome (MTS) and Turcot syndrome (TS), both of which predispose to colorectal cancer. They may be distinguished by the presence of benign or malignant sebaceous tumours in MTS, and tumours of the central nervous system in TS. PATIENTS AND METHODS: A 59-year-old man, with a history of right colon cancer at the age of 36 years, consulted for a nose lesion shown by histopathological examination to be a sebaceous tumour. Immunohistochemistry revealed loss of expression of proteins MSH2 and MSH6, strongly suggesting a diagnosis of MTS. Eight years earlier, the man's son had developed a fatal glioblastoma; given the paternal phenotype of MTS, the hypothesis of TS in the son is probable. DISCUSSION: This case suggests that several variants of Lynch syndrome may be seen within the same family. It raises the issue of screening for cerebral tumours in patients with MTS and in their family members, even though such a recommendation does not exist; current recommendations in fact consist primarily of gastrointestinal and gynaecological monitoring.

Clinicopathological study of 47 cases of sebaceoma.

BACKGROUND: Sebaceoma is a rare and poorly understood form of sebaceous tumour, and it is of great significance since it may reveal Muir-Torre syndrome (MTS). Herein, we present a series of cases with details of the histopathological appearance. PATIENTS AND METHODS: We examined records of cases labelled as sebaceous tumour recorded at the Strasbourg Dermatopathology Laboratory between 1991 and 2015. We include cases of benign sebaceous tumour predominantly involving immature basophilic cells. The clinical and histological data were collected as well as screening for a history of MTS. RESULTS: We studied 47 cases of sebaceomas (26 men), in patients of mean age 67.6years, located primarily in the head or neck (32 cases). Of the 17 patients followed up, 6 had MTS. Different types of architecture were seen: dermal nodule (9 cases) or cystic nodule (9 cases), multiple dermal nodules (22 cases), exophytic tumour (4 cases) and an appearance intermediate with sebaceous adenoma (3 cases). The cells involved were basophilic, with the presence of round ducts exhibiting an eosinophilic cuticle and, in rare cases, mature sebocytes. Mitoses were observed: mean 6.6/10 fields (0 to 19). In all cases, there was expression of CK17, EMA and androgen receptors, but not of BerEP4. DISCUSSION: Sebaceoma is a small benign tumour but identification is imperative due to association with MTS. A knowledge of the associated cytological and architectural elements - particularly cysts and labyrinthine patterns - and immunolabelling enable differential diagnosis with respect to other tumours. The extra-facial and cystic forms in particular require screening for MTS. If there is any doubt, immunolabelling of androgen receptors provides a precious tool.

[Muir-Torre syndrome associated with Waldenstrom's macroglobulinemia]. / Syndrome de Muir-Torre chez un patient atteint de maladie de Waldenström.

BACKGROUND: Muir-Torre syndrome (MTS), a cutaneous variant of Lynch syndrome, consists of hereditary predisposition to cutaneous tumours and gastrointestinal and gynaecological neoplasms, with autosomal dominant transmission. It is associated with mutations in genes coding for proteins in the DNA mismatch repair system. PATIENTS AND METHODS: Herein, we report a case of a male patient presenting Waldenstrom's macroglobulinemia since the age of 50 and which, after the age of 65 years, developed into sebaceous tumours (5 sebaceous adenomas, 1 sebaceoma, 1 sebaceous carcinoma) and colonic lesions (4 adenomas). The clinical phenotype was consistent with MTS. Somatic analysis carried out on one sebaceous tumour showed instability of the microsatellites with loss of expression of MSH2 and MSH6 although constitutional genetic analysis showed no germline mutations known to be harmful. DISCUSSION: This noteworthy case raises a number of questions, including the possibility of association between STM and Waldenstrom's macroglobulinemia, which is discussed herein.

[Red blood transfusion in palliative care situation]. / Transfusion de globules rouges en situation oncologique palliative.

Anemia is frequent in oncology. We debate the decision-making process of erythrocyte transfusion in palliative care situation from a case report. A patient with a prostatic metastatic cancer was in palliative situation with asthenia and coronary symptom. We analyze, in this particular case that does not describe reality of normal practice, the decision-making process of erythrocyte transfusion. These transfusions were based, in this case, on the evaluation of oncology prognosis, the short-term vital threats, life project and clinical safety of the transfusion. The patient has received 5 erythrocyte transfusions in 4 months until a multidisciplinary meeting decided to stop transfusion because of poor prognostic situation and bad tolerance of the act. This patient could be a collegial model used to measure the reasonable nature of prescription depending on the purpose and the goal of the patient but does not allow generalization. Although there is low risk of erythrocyte shortage, it seems important to train doctors to reduce abusive transfusion and define transfusion thresholds. Different levels of erythrocyte transfusion security would raise the issue of management of several stocks. Erythrocyte transfusion in palliative care can be considered subject to prognostic information and the palliative aim of the transfusions, multidisciplinary decision-making, during short hospitalizations and with evaluation of the act and consequences for the patient.

[Cutaneous panniculitis]. / Panniculites cutanées.

Panniculitis is an inflammation of the subcutaneous fat. Skin biopsy plays a critical role in the differential diagnosis of panniculitis. The most common approach to diagnosis relies on the differentiation between predominantly septal or lobular panniculitis, followed by the distinction between lesions with and without vasculitis. It is also very important to submit a part of the skin biopsy for microbiological analysis and for T-cell clonal expansion if T-cell lymphoma is suspected. Erythema nodosum, the most frequent septal panniculitis, has many causes and in its typical clinical presentation, does not require skin biopsy. In other panniculitis, diagnosis is based on the integration of the clinical and histological data, which renders a deep cutaneous biopsy necessary. Periarteritis nodosa, a septal panniculitis with vasculitis characterized by subcutaneous nodules and livedo racemosa, can be associated with systemic involvement. Nodular thrombophlebitis needs search for associated coagulopathy, Behçet's disease, periarteritis nodosa or Buerger's disease. Lobular panniculitis are classified according to the nature of cells present in the inflammatory infiltrate. If there is a lymphocytic infiltration, lupus panniculitis is difficult to differentiate from subcutaneous panniculitis-like T-cell lymphoma. If there are histiocytes, it can be a sarcoidosis, a cytophagic histiocytic panniculitis or, in newborn, a subcutaneous fat necrosis. Neutrophilic panniculitis needs careful clinic-pathologic correlation. Treatment of panniculitis can be challenging and is based on the histopathological findings. Frequently, the precise cause of panniculitis cannot be established from the outset, so it is important to follow-up patients and not hesitate to repeat the skin biopsy.

[Advance directives in dermato-oncology: The current situation and future prospects]. / Directives anticipées en dermato-oncologie : situation actuelle et perspectives.

INTRODUCTION: Patient information and advance directives (AD) are described in the French laws of 4 March 2002 and 22 April 2005, which concern the decisions of subjects regarding end-of-life treatment. At present, practitioners rarely seek the opinion of patients on this matter. The Claeys-Leonetti law requires doctors to identify any advance directives by patients, which are binding upon medical staff. The present study sought to analyse the extent of application of the laws of 2002 and 2005 and to collect the observations of clinicians in dermato-oncology regarding the new legislation. METHODS: We contacted members of the French dermato-oncology group by email and asked them to assess their practices with regard to information provision, patient surrogates and advance directives. RESULTS: To 111 requests we received 34 replies from hospital dermatologists, i.e. a response rate of 31 %. In all, 85 % of clinicians informed patients with metastasis that their disease was incurable, and 94 % stated that they have procedures in place concerning the appointment of a patient surrogate. One third of respondents reported having a procedure in place for provision of information or collection of advance directives. According to 91 % of clinicians, the binding nature of advance directives did not constitute any loss of chance for the patient in question; 59 % felt that the new law would affect their practices, but of these, paradoxically, 60 % felt that this would have no impact on their therapeutic decision-making. In all, 26 clinicians (76.5 %) did not intend to modify their decision-making process. CONCLUSION: The law of 2002 is generally better known than that of 2005. Dermato-oncologists are not aware of the practical consequences of the new binding nature of advance directives with regard to the doctor-patient relationship and the actual decision-making process.