Chronic trophic disorders in cocaine and heroin injectors: A multicenter retrospective observational descriptive study.

AIMS: To describe the demographic characteristics of heroin and cocaine injectors with chronic injection-related trophic disorders, as well as the clinical and progressive characteristics of these disorders. METHODS: A descriptive, observational, multicenter and retrospective study over the last 15 years. Patients were recruited via a call for cases and by consulting the health data warehouse of the university hospital center. RESULTS: The population comprised 39 injection drug users, of whom 79.5% were male, with a median age of 41 years. Subjects had numerous co-addictions and 70.5% were infected with hepatitis C virus. Trophic disorders were multiple in some cases: 43.5% of patients had lymphoedema, 87% had ulcers, and 56.5% had injection-related scars. Ulcers were multiple, large, and present for a median of 3 years. They were located on the upper limbs in 32.5% of cases. Ulcers constituted a source of complications in 64.5% of cases and these were infectious in 91% of cases (local, osteoarticular or systemic). During follow-up, 8 patients died and 21.5% of patients requiring ulcer care were lost to follow-up. CONCLUSIONS: This study showed a high rate of complications, particularly infections, of ulcers in injection drug users. Localization of these ulcers to the upper limbs, although rare in the general population, is relatively frequent in this population. Follow-up is difficult and cooperation between dermatologist and addictologist is essential to improve patient care.

[Primitive myxoid melanoma: An unusual histological aspect]. / Mélanome myxoïde primitif : une forme histologique inhabituelle.

BACKGROUND: Myxoid melanoma is a rare variant of melanoma that must be recognised. Herein we describe a new metastatic case. PATIENTS AND METHODS: A 78-year-old woman consulted for a firm, pinkish nodule measuring 25mm and present for six months on her left leg. Analysis of the biopsy revealed achromic fusiform tumour cells separated by large myxoid plaques. Labeling of SOX10, HMB45 and PS100 was diffuse and of moderate to strong intensity. A diagnosis of myxoid melanoma was considered, with Breslow thickness of 9mm. Surgery was carried out with a 2-cm margin and confirmed the diagnosis. Dermatological follow-up at one year revealed metastatic spread to the ganglia, pleura, liver and bone. DISCUSSION: Few cases of primary myxoid melanoma have been described, and the condition is probably underdiagnosed. The classic clinical presentation of this condition consists of a solitary achromic nodule found chiefly on the limbs. The microscopic appearance is relatively non-specific. Immunohistochemical analysis may indicate melanocytic involvement: cells exhibit expression of SOX10, diffuse expression of protein S100, and less consistent and more variable expression of HMB45. The increasingly common use of anti-SOX10 is of value since it is expressed in the nucleus of melanocytes. Mastocytes and TGF-ß secretion appear to be involved in myxoid stroma production. In the absence of specific codification, management of myxoid melanoma is comparable to that of other types of melanoma. There is uncertainty about the prognosis, with the involvement of TGF-ß possibly indicating the aggressive potential of this type of tumour.

[Skin and mucosal erosions revealing secondary syphilis with otologic and eye disorders]. / Érosions cutanéo-muqueuses révélant une syphilis secondaire avec atteintes otologique et ophtalmologique.

BACKGROUND: The clinical polymorphism of syphilis leads to diagnostic issues. We report a case of secondary syphilis revealed by skin and mucosal erosions, and responsible for sensorineural hearing loss and asymptomatic papillitis. PATIENTS AND METHODS: A 55-year-old man presented oral and peri-anal erosions as the initial symptoms of secondary syphilis. He reported hypoacusis and a pure-tone audiogram revealed bilateral sensorineural hearing loss. Ophthalmological investigation revealed isolated right papillitis and superior temporal scotoma with blind-spot enlargement. TPHA-VDRL serology was strongly positive for plasma (TPHA 1/10,240 and VDRL 1/64) but doubtful for cerebrospinal fluid. For his hearing and eye disorders, considered as related to neurosyphilis, the patient received a 14-day course of intravenous penicillin G, associated with systemic corticosteroids with gradual reduction over a period of fifteen weeks. The patient's skin and mucosal erosions resolved, as did his papillitis. His hearing loss remained stable. Serological monitoring at three months showed a sixteen-fold decrease in VDRL titre. DISCUSSION: The re-emergence of syphilis has led to increasing incidence of related ophthalmological and otological disorders. This report highlights the first-line role of the dermatologist in systematic diagnosis and in screening for associated involvement.

[Chronic lymphocytic leukaemia presenting with specific cutaneous infiltrates in the absence of lymphocytosis: Two cases]. / Leucémie lymphoïde chronique révélée par des infiltrats cutanés spécifiques en l'absence d'hyperlymphocytose : deux observations.

BACKGROUND: Specific cutaneous infiltrates of chronic lymphocytic leukaemia (CLL) are rare. They occur after a mean disease duration of 3 years. CLL skin infiltrates as the primary manifestation of the disease have been reported, but a normal lymphocyte count at diagnosis is rare. We present two cases of CLL initially presenting in the skin, without lymphocytosis. PATIENTS AND METHODS: A 53-year-old man developed papulonodular lesions of the face and infiltrated plaques of the scalp, and an 85-year-old woman presented erythematous nodules of the face and neck. Histopathology revealed a lymphocytic infiltrate, consisting of small mature B-cells CD20+, CD79+, with an aberrant phenotype CD5+. CD23 was positive in one case. The two patients had no lymphocytosis, but immunophenotyping was characteristic of CLL. In the second case, there was a sub-mental adenopathy, histologic analysis of which was consistent with CLL. The CLL was classified as Binet stage A in the two cases. No disease progression was noted at follow-up. DISCUSSION: The unusual feature of these cases is the lack of lymphocytosis at diagnosis. Thus, the skin lesions resulted in further evaluations for CLL, although the diagnosis was not suggested by the blood count. CONCLUSION: Skin involvement in CLL does not appear to be a poor prognostic indicator, arguing in favour of recruitment of circulating monoclonal B-cells rather than an additional tumour mass.

[Multiple basal cell carcinomas after etanercept treatment for psoriasis]. / Carcinomes basocellulaires multiples après traitement d'un psoriasis par étanercept.

BACKGROUND: Several cases of skin cancer have been reported after treatment with etanercept although the causal relationship remains uncertain. We report the case of a patient who rapidly developed multiple basal cell carcinomas (BCC) after discontinuation of this treatment. PATIENTS AND METHODS: A 42-year-old man presented severe plaque psoriasis after receiving topical therapy, less than 100 sessions of PUVA-therapy, retinoids and repeated solar exposure. Severe worsening of the psoriasis led us to use etanercept for seven months with excellent results. However, 11 BCCs gradually appeared within a year starting one month after the end of treatment. DISCUSSION: There is some controversy about the risk of non melanoma skin cancer associated with etanercept treatment. However, even the most recent studies are contradictory and they mostly concern rheumatological indications. In the past four years, a dozen cases of BCC have been reported following treatment for cutaneous psoriasis. As regards our patient, a genetic predisposition is possible but a potentiating effect of solar exposure is strongly suspected. This observation should lead to reinforced screening for BCC and restriction of anti-TNFalpha therapy to patients who have received less than 1000 J of PUVA-therapy, as recommended by the British Society of Rheumatology for psoriatic rheumatism. Levels of natural solar exposure must be also be taken into account.

An unexpected asymptomatic epiglottal site of Kimura disease.

INTRODUCTION: Kimura disease (KD) is a chronic lymphoproliferative disorder of unknown etiology that affects the skin and lymph nodes, mostly observed in males of Asian descent. The natural history of asymptomatic epiglottal KD remains unknown. This rare site of KD is often only diagnosed when tumor growth starts to obstruct the upper airways. OBSERVATION: A 34-year-old North African male presented with fatigue and multiple, slowly progressive, fluctuating skin nodules in the right mandibular and retroauricular regions. Computed tomography of the head and neck revealed a large soft tissue tumor close to the right mandibular body and unexpected thickening of the epiglottis. Transnasal laryngoscopy confirmed the CT findings and showed thickening of the epiglottis. The diagnosis of KD was based on histological examination of biopsy specimens taken from the right mandibular tumor, a cervical lymph node, and the epiglottis. DISCUSSION: Most cases of KD with epiglottal involvement present with dysphonia and dysphagia. No consensus guidelines are available concerning the complementary investigations that should be performed. This case report raises the question of whether patients with suspected KD should be systematically screened for lesions in unusual and potentially dangerous anatomic sites.

Stressful life events and pemphigus.

BACKGROUND: Stress might be a triggering factor causing pemphigus. We studied 11 consecutive cases of pemphigus over 5 years. OBJECTIVE: Studying and looking for a link between severe life events and the history of the disease. METHODS: An epidemiological retrospective and prospective study was carried out, including an interview and a collection of the clinical history; then the life events were integrated into the clinical history with the patient blind. Two scales were used: Paykel's inventory (assessing the negative impact of life events) and the Mini International Neuropsychiatric Interview DSM-IV (MINI). RESULTS: 10 patients out of 11 were included. With the MINI, 2 patients presented anxiety. Paykel's inventory showed type 3 life events for numerous patients, life event type 4 for 7 patients and type 5 for 3 patients, happening from 1 to 6 months before the first signs or worsening of pemphigus. We found stressful life events before the start or worsening of pemphigus for all patients with no other risk factors. CONCLUSION: Stressful life events can worsen or trigger off a pemphigus. Psychological care, associated with the immunosuppressive treatment, should entail a better management of these patients.

[Buschke's scleredema successfully treated with extracorporeal photopheresis]. / Sclérœdème de Buschke traité efficacement par photophérèse extracorporelle.

BACKGROUND: Scleredema adultorum (Buschke's scleredema) is a cutaneous mucinosis of unknown origin, clinically characterized by a diffuse induration of the skin usually involving the neck, shoulders and back, which limits patients' mobility. CASE REPORT: We report a case of a 50-year-old woman who presented a chronic sclerodermiform syndrome for 2 years associated with type 1 diabetes. Physical examination revealed an extensive skin induration involving the shoulders, neck and back. Histologic examination confirmed the diagnosis of scleredema adultorum. The patient was treated with extracorporeal photopheresis (EPP) twice a month for two months. At follow-up, mobility was highly improved after two months. Beneficial effect of EPP was maintained on the long term while sessions were spaced. DISCUSSION: EPP is an unconventional treatment of Buschke's scleredema. We described a case of Buschke's scleredema successfully treated with EPP which may represent a therapeutic option for the treatment of scleredema.

Numerical optimization of cell colonization modelling inside scaffold for perfusion bioreactor: A multiscale model.

Part of clinically applicable bone graft substitutes are developed by using mechanical stimulation of flow-perfusion into cell-seeded scaffolds. The role of fluid flow is crucial in driving the nutrient to seeded cells and in stimulating cell colonization. A common numerical approach is to use a multiscale model to link some physical quantities (wall shear stress and inlet flow rate) that act at different scales. In this study, a multiscale model is developed in order to determine the optimal inlet flow rate to cultivate osteoblast-like cells seeded in a controlled macroporous biomaterial inside a perfusion bioreactor system. We focus particularly on the influence of Wall Shear Stress on cell colonization to predict cell colonization at the macroscale. Results obtained at the microscale are interpolated at the macroscale to determine the optimal flow rate. For a macroporous scaffold made of interconnected pores with pore diameters of above 350 µm and interconnection diameters of 150 µm, the model predicts a cell colonization of 325% after a 7-day-cell culture with a constant inlet flow rate of 0.69 mL·min-1. Furthermore, the strength of this protocol is the possibility to adapt it to most porous biomaterials and dynamic cell culture systems.

Subcorneal pustulosis and Pyoderma gangrenosum associated with a biclonal gammopathy.

Pyoderma gangrenosum and subcorneal pustulosis are two neutrophilic dermatoses. Their occurrence in the same patient is rare and may be related to an IgA dysglobulinemia. We report a case presenting these two conditions associated with a biclonal benign IgA and IgG gammopathy. A 67-year-old man exhibited typical pyoderma gangrenosum associated after three years duration with subcorneal pustulosis lesions, confirmed by cutaneous biopsy. Laboratory results showed a biclonal benign IgA and IgG kappa gammopathy. Therapeutic management was difficult: Pyoderma gangrenosum responded well to corticosteroids but subcorneal pustulosis management was harder and treatments were poorly effective.Pyoderma gangrenosum and subcorneal pustulosis are a part of the neutrophilic spectrum. Their association has been only reported in eleven patients. In eight cases, an IgA dysglobulinemia was associated suggesting its responsibility in the occurrence of both dermatoses. Treatments are various and not fully effective. If the Pyoderma gangrenosum usually responds to corticosteroids, the subcorneal pustulosis treatments are not well defined and often not efficient. Our case illustrates the dissociated evolution of these two dermatoses and their difficult global management. During the follow-up, a regular search for dysglobulinemia is required in order to detect malignant transformations.

[Neonatal maculopapular exanthema revealing septicemia due to Klebsiella pneumoniae via maternofetal infection]. / Exanthème maculo-papuleux néonatal révélateur d'une septicémie à Klebsiella pneumoniaee par infection maternofoetale.

BACKGROUND: Neonatal exanthema of bacterial origin is caused very rarely by Gram-negative bacilli. We report a case of neonatal maculopapular exanthema evocative of Klebsiella pneumoniae septicemia resulting from maternofetal infection. OBSERVATION: On the fourth day of life, a newborn infant presented incipient morbilliform maculopapular exanthema on the face. During delivery, the mother had presented hyperthermia and meconium was found in the amniotic fluid. Clinical examination of the newborn was normal. No clinically obvious site of entry of infection was seen. Laboratory tests revealed major inflammatory syndrome. Blood cultures were positive for K. pneumoniae, which was also found in blood cultures from the mother. Screening for other causes of infection was negative. Parenteral antibiotics for 10 days yielded favorable results with simultaneous resolution of the exanthema, normalization of laboratory values and negative blood cultures. DISCUSSION: Exanthemas of infectious origin are not associated with any specific organism. The most common causative micro-organisms are Listeria monocytogenes, B streptococci, colibacilli, and more rarely, staphylococci. There have been reports of a number of cases of neonatal septicemia due to Gram-negative bacilli responsible for maculopapular exanthemas but the causative organism was not identified. To our knowledge, Klebsiella pneumoniae has never been incriminated in the appearance of this type of rash via maternofetal transmission. Consequently, the presence of neonatal exanthema should prompt screening for sepsis, even in the absence of other evocative signs, and in particular in settings of apyrexia.

Numerical and ex vivo studies of a bioprobe developed for laser-induced thermotherapy (LITT) in contact with liver tissue.

This work is based on the production of a bioprobe that is compatible with magnetic resonance imaging (MRI) for laser-induced thermotherapy (LITT) in liver cancer laser therapy. This probe is made of an alumina tube (3-mm diameter) in which an optical fibre is centred and fixed. A shooting window (20mm) is created using a mechanical rectifier. The device is then consolidated by the injection of a transparent and heat-resistant resin. Through numerical modelling, the thermal power damping of the laser source is evaluated as well as the propagation of the heat in the ex vivo liver tissue according to different heating scenarios. These analyses allow for an estimation of the irradiated volume. Ex vivo tests were performed on bovine liver to confirm the adequacy of the bioprobe for LITT and of the irradiated volumes predicted by the numerical model. There was a difference of 8% between the simulations and ex vivo experiments. The pulsed mode heating scenario was the most effective under the experimental conditions.

[Topical haemotherapy as treatment for necrotic angiodermatitis: a pilot study]. / Autohémothérapie locale dans l'angiodermite nécrotique: étude pilote.

INTRODUCTION: Necrotic angiodermatitis is a variety of leg ulcer, characterized by very painful and rapidly spreading lesions. Healing takes time, a mean of 4 to 11 months. Current treatment is difficult and rarely satisfactory. Only early skin grafts lead to rapid sedation of pain and stop the lesion from extending. However, this technique, performed during a progressive phase, enhances the risk of losing a certain number of grafts. We wanted to confirm the efficacy of local auto-haemo-therapy proposed in 2 non-controlled studies. PATIENTS AND METHODS: Eleven patients with necrotic angiodermatitis were included in our non-controlled prospective study. The topical hemotherapy was applied three times a week for one month, with initial hospitalization for 15 days. The criteria of assessment were: evolution of pain, surface of the ulcer, colorimetric scale and the date of disappearance of the purple halo. RESULTS: Ten patients were relieved by the treatment, with progressive withdrawal of the analgesics. The progression of the lesion had stopped and the purple halo had disappeared in general by D6 (range: 2 to 21 days). The percentage of granulation tissue with regard to the surface of the ulcer was of 45.4 p. 100 on D0, 80 p. 100 on D14 and 85 p. 100 on D28. CONCLUSION: Topical hemotherapy applied during the initial phase of treatment would reduce the pain and prepare the lesion for a secondary skin graft.

[Primitive cutaneous neuroendocrine carcinomas or Merkel's tumor. Clinical and therapeutic aspects in 22 patients]. / Carcinomes neuroendocrines cutanés primitifs ou tumeurs de Merkel. Aspects cliniques et thérapeutiques chez 22 malades.

INTRODUCTION: Primitive cutaneous neuroendocrine carcinoma (PCNC) is a rare tumor with poor prognosis. Surgery is the treatment of choice, but radiotherapy is taking a larger place in the management of these patients. METHODS: The files of 22 patients were studied retrospectively over a period of 14 years. RESULTS: The study included 17 women and 5 men with a mean age of 75.5 years. The localization of the tumor was the cephalic extremity in 12 cases. At the initial stage, the tumor in 3 of the 22 patients was of glandular or visceral localization. Thirteen stage I patients were treated surgically. Adjuvant radiotherapy was performed in 10 of these patients and only one relapsed (incomplete initial exeresis). The other three relapsed. Exclusive radiotherapy led to complete response in 4 patients who could not undergo surgery and in 2 with local relapses. Seven patients exhibited glandular involvement and 6 of them died (median 7 months) after the adenopathy had been discovered. DISCUSSION: Our series illustrates the clinical characteristics of this tumor of the elderly, which is predominantly cephalic and of rapid progression. From a therapeutic point of view, our results underline the benefit of radiotherapy as adjuvant to surgery. When surgery is impossible, and in the case of local relapse, radiotherapy gives good results.

[Lichenoid drug reaction to leflunomide]. / Toxidermie lichénoïde au léflunomide (Arava).

INTRODUCTION: Leflunomide (Arava) is an immunomodulator, recently introduced for systemic treatment of rheumatoid arthritis. We report the first case of lichenoid drug reaction due to this drug. CASE REPORT: A sixty-four year-old woman received leflunomide for rheumatoid arthritis. Two months after initiation of treatment, pruritus and lichenoid papules appeared on her hands and subsequently on her arms and her trunk, with a few bullous lesions. A skin biopsy was evocative for the diagnosis of drug induced lichenoid eruption. The treatment was stopped, and a wash out with colestyramine and topical corticotherapy resulted in dramatic improvement. No relapse was observed. Two months later, patch-tests with leflunomide diluted to 30 p. 100 in white petrolatum were negative. DISCUSSION: Side effects of leflunomide are frequent, generally benign for the cutaneous features. In our case, the delay, clinical and histological aspect and improvement on withdrawal of the drug emphasize the imputability of leflunomide. Few cases have been reported with others immunomodulators.

[Giant primitive neuroendocrine cutaneous carcinoma of the leg]. / Carcinome neuroendocrine cutané primitif géant de la jambe.

INTRODUCTION: Primitive cutaneous neuroendocrine carcinoma or Merkel cell carcinoma is a tumor characterized by rapid evolution. The size at the time of diagnosis is a mean of 2 to 3 centimeters. We report a case of a particular clinical and anatomopathological presentation. OBSERVATION: A 76 year-old woman had a voluminous tumoral plaque on the left leg with multiple ulcero-necrotic lesions involving the bone structure without palpable, inguinal lymphadenopathy. The histological and immunocytochemistry examination led to the diagnosis of cutaneous neuroendocrine carcinoma. The normality of the chest, abdominal and pelvis investigations confirmed the primitive cutaneous origin. The patient died a few months later without lymph involvement. DISCUSSION: To the best of our knowledge, such a presentation of primitive cutaneous neuroendocrine carcinoma has never been reported. The absence of lymphadenopathy despite the tumor volume and extensive skin involvement suggest the hypothesis of a non lymphophilic form of primitive cutaneous neuroendocrine carcinoma. This case report shows that lymphophily depends neither on the tumoral size nor to the degree of differentiation of tumoral cell proliferation, but on biological factors that require specification.

[Rhabdomyosarcoma at site of pacemaker implantation]. / Rhabdomyosarcome sur site d'implantation d'un stimulateur cardiaque.

BACKGROUND: Malignant proliferation at the site of implantation of a pacemaker generator is uncommon. We report the case of a patient who developed rhabdomyosarcoma. CASE REPORT: A 85-year-old man presented with a voluminous and rapidly evolving tumor localised beneath the right clavicle. This inflammatory and necrotic lesion developed on the area where a titanium pacemaker had been implanted five years earlier. Rhabdomyosarcoma was diagnosis on the basis of immunohistochemistry findings. In spite of a wide surgical excision of this primitive tumour, visceral dissemination appeared, rapidly leading to the patient's death. DISCUSSION: This observation rises the question of the role of the pacemaker implantation in tumor development. The excellent in vivo tolerance and the widespread utilization of titanium as biomaterial is an argument against its carcinogenic action. Inversely, a metal-related chronic inflammatory reaction could favor the neoplastic process in predisposed subjects as has been observed with prosthetic materials used in orthopaedics.