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Resume Introduction Quelques cas de melanomes sur tatouages ont ete rapportes. Le role du tatouage dans le developpement de tumeurs cutanees malignes est cependant tres discute. Nous rapportons deux nouvelles observations de melanomes se developpant sur des tatouages, completees d’une revue systematique de la litterature. Observations Cas 1. Un patient de 61 ans, de phototype IV, consultait pour une lesion nodulaire pigmentee de 4,5 × 3 cm apparue depuis un an, developpee sur un tatouage noir et rouge du dos. L’examen histologique de la lesion objectivait un melanome de type nodulaire ulcere, indice de Breslow a 7 mm. Le bilan d’extension ne trouvait pas de localisations secondaires. Cas 2. Un patient de 39 ans, de phototype II, portant un tatouage noir du bras consultait pour une lesion pigmentee cliniquement suspecte evoluant depuis plusieurs mois. L’exerese etait d’emblee realisee, confirmant le diagnostic de melanome de type SSM, developpe sur naevus preexistant, indice de Breslow 0,9 mm. Une reprise a 1 cm etait realisee et, avec un recul de 9 ans, il n’etait pas note de recidive. Discussion La revue systematique de la litterature trouvait 34 cas de melanomes survenus sur tatouage. Il existait une forte prevalence masculine (90,3 %) et un âge moyen relativement jeune (45,9 ans). Il s’agissait majoritairement de tatouages monochromes noir/bleu fonce (71,0 %). Le delai moyen entre la realisation du tatouage et l’apparition du melanome etait de 13,2 ans. La localisation la plus frequente du melanome etait le membre superieur (53,1 %) suivi du tronc (34,4 %). A l’examen anatomopathologique, il s’agissait dans 2 cas de melanomes in situ, et dans 13 cas, l’indice de Breslow etait inferieur ou egal a 1 mm. Dans 5 observations, une metastase ganglionnaire macroscopique ou microscopique (ganglion sentinelle) etait trouvee au diagnostic et dans une de ces observations etaient aussi observees des metastases cutanees en transit au diagnostic. L’hypothetique role du tatouage dans la carcinogenese est discute.

Median raphe cysts are rare benign lesions of the male genitalia. They may be present along the entire raphe, from the urethral meatus to the anus, in the median position. We report herein a case of median raphe cyst with an atypical clinical and histological presentation.An 11-year-old boy consulted in dermatology for an asymptomatic perineal lesion. The clinical examination revealed a linear cystic lesion with clear content extending from the base of the scrotum to the anus. The histological diagnosis was a superficial dermal glandular cyst suggestive of a possibly apocrine origin in this topography, or possibly due to malformation.Different clinical and histological features of median raphe cysts have been reported in the literature. Treatment is mainly surgical.

Guidelines for the diagnosis and treatment of cutaneous squamous cell carcinoma and precursor lesions J.J. Bonerandi, C. Beauvillain, L. Caquant, J.F. Chassagne, V. Chaussade,** P. Clavere, C. Desouches, F. Garnier, J.L. Grolleau, M. Grossin,*** A. Jourdain, J.Y. Lemonnier, H. Maillard, N. Ortonne, E. Rio,**** E. Simon, J.F. Sei,** J.J. Grob, L. Martin,* for the French Dermatology Recommendations Association (aRED) Department of Dermatology, La Timone University Hospital, Marseille, France Department of ENT, Nantes University Hospital, Nantes, France Department of Maxillofacial Surgery, Saint-Etienne University Hospital, Saint-Etienne, France Department of Maxillofacial Surgery, Nancy University Hospital, Nancy, France **Department of Dermatology, Ambroise-Pare University Hospital, Boulogne-Billancourt, France Department of Oncoradiotherapy, Limoges University Hospital, Limoges, France Plastic Surgery, Private Practice, Marseille, France General Practitioner, Angers, France Department of Plastic Surgery, Toulouse University Hospital, Toulouse, France ***Department of Pathology, Louis-Mourier University Hospital, Colombes, France ENT, Private Practice, Laval, France Department of Gerontology, Orleans Hospital, Orleans, France Department of Dermatology, Le Mans Hospital, Le Mans, France Department of Pathology, Henri-Mondor University Hospital, Creteil, France ****Department of Oncoradiotherapy, CLCC Nantes, Nantes, France Department of Dermatology, Angers University Hospital, Angers, France *Correspondence: Prof. Ludovic Martin. E-mail: lumartin@chu-angers.fr

Resume Introduction Au stade de transformation en lymphome a grandes cellules, le mycosis fongoide est de mauvais pronostic avec une mediane de survie de 36 mois. L’autogreffe constitue une alternative therapeutique rarement proposee dans cette indication. Nous rapportons l’observation d’un jeune homme en remission complete des lesions transformees 14 ans apres une autogreffe. Observation Un homme de 25 ans, suivi depuis l’enfance pour une dermatose eczematiforme et traite par chimiotherapie pour une polyadenopathie dans l’hypothese d’une maladie de Hodgkin, consultait pour l’apparition recente de tumeurs cutanees diffuses associees a des plaques erythemateuses infiltrees. L’examen histologique des tumeurs montrait une infiltration lymphocytaire T atypique comprenant 25 % de grandes cellules CD30+ et un epidermotropisme. Les plaques non infiltrees correspondaient a un lymphome T epidermotrope a petites cellules. La relecture des biopsies ganglionnaires initialement considerees comme maladie de Hodgkin confortait en fait le diagnostic de mycosis fongoide transforme. Malgre plusieurs lignes de polychimiotherapie, la maladie progressait avec, en particulier, l’apparition d’une atteinte neurologique. Une greffe de cellules souches autologues etait alors realisee, entrainant une regression rapide des tumeurs, des adenopathies et des troubles neurologiques. Des plaques de mycosis fongoide non transforme persistaient cependant, repondant favorablement a la mechlorethamine topique. Ce patient est toujours en remission des lesions tumorales a 14 ans de l’autogreffe. Discussion Le cas presente correspond probablement a un mycosis fongoide de l’enfant diagnostique a l’âge adulte au moment de sa transformation en lymphome a grandes cellules. L’atteinte neurologique du mycosis fongoide est rare et habituellement de pronostic sombre. La tres longue remission observee chez notre patient souligne l’interet potentiel de l’autogreffe dans les mycosis fongoides transformes agressifs, en particulier chez les patients non eligibles pour l’allogreffe.

We report two cases of intravenous leiomyomatosis, a rarely described benign tumor, which illustrate potential complications resulting from metastatic dissemination. Both cases were fortuitously discoveries. We discuss the diagnostic problems, etiological factors, therapeutic management and prognosis. Hysterectomy with bilateral salpingo-oophorectomy and regular follow-up with cardiac and pulmonary monitoring were performed. Pelvic pain disappeared and the patients are recurrence-free. The main difficulties concerned early diagnosis, complete resection and prognosis. These cases illustrate the importance of careful pathology examination of all uterine myoma resection specimens.

It was recently shown that vascular endothelial growth factor (VEGF), a growth factor for endothelial cells, plays a pivotal role in rheumatoid arthritis. VEGF binds to specific receptors, known as VEGF-RI and VEGF-RII. We assessed the physical and histological effects of selective blockade of VEGF and its receptors in transgenic K/BxN mice, a model of rheumatoid arthritis very close to the human disease. Mice were treated with anti-mouse VEGF Ab, anti-mouse VEGF-RI and -RII Abs, and an inhibitor of VEGF-RI tyrosine kinase. Disease activity was monitored using clinical indexes and by histological examination. We found that synovial cells from arthritic joints express VEGF, VEGF-RI, and VEGF-RII. Treatment with anti-VEGF-RI strongly attenuated the disease throughout the study period, while anti-VEGF only transiently delayed disease onset. Treatment with anti-VEGF-RII had no effect. Anti-VEGF-RI reduced the intensity of clinical manifestations and, based on qualitative and semiquantitative histological analyses, prevented joint damage. Treatment with a VEGF-RI tyrosine kinase inhibitor almost abolished the disease. These results show that VEGF is a key factor in pannus development, acting through the VEGF-RI pathway. The observation that in vivo administration of specific inhibitors targeting the VEGF-RI pathway suppressed arthritis and prevented bone destruction opens up new possibilities for the treatment of rheumatoid arthritis.

Objective Reactive oxygen species are thought to play a role in rheumatoid arthritis (RA) in humans. We postulated that antioxidant treatment could have a beneficial effect in this disease. We therefore investigated the effects of vitamin E in the transgenic KRN/NOD mouse model of RA. Methods Mice were treated by gavage with oral vitamin E (α-tocopherol). Clinical, histologic, and biochemical parameters were assessed for 6 weeks. Results Vitamin E treatment did not modify the clinical features of the disease (date of onset or disease intensity, as measured by the articular index), but it did prevent joint destruction, as measured by qualitative and semiquantitative analyses. Redox status did not differ between treated and control mice. White blood cell chemiluminescence was higher in transgenic KRN/NOD mice than in controls, but vitamin E treatment attenuated this difference. Vitamin E treatment of the transgenic animals led to a significant decrease in the levels of interleukin-1β (IL-1β) but not tumor necrosis factor α. Conclusion Vitamin E seems to uncouple joint inflammation and joint destruction in this model of RA, with a beneficial effect on joint destruction. Since many investigations are currently in progress to evaluate the benefit of interventions targeted toward anti-IL-1β, our findings suggest opportunities of therapeutic interest in human RA.

Mesenteric trauma is one of the possible injuries caused by the use of seat belts in case of motor vehicle crash. We report here a rare case of rectal bleeding by rupture of a mesosigmoid haematoma. An emergent laparotomy revealed a mesosigmoid haematoma with a centimetric rectal perforation. The wearing of safety belts added some specific blunt abdominal trauma, which directly depends on lap-and-sash belts. Mesenteric injuries are found out up to 5% of blunt abdominal traumas. "Seat belt mark" leads the surgical team to strongly suspect an intra-abdominal trauma. When "seat belt mark" sign is found, in patients with mild to severe blunt car injuries, CT-scan has to be realised to eliminate intra-abdominal complications, including mesenteric and mesosigmoid ones. In case of proved mesenteric haematoma associated to intestinal bleeding, a surgical treatment must be considered as first choice. Conservative approach remains possible in stable patients but surgical exploration remains necessary in unstable patients with active bleeding.

Objective We assessed the clinical and histologic features of angiogenesis inhibition in a transgenic mouse model of arthritis that closely resembles rheumatoid arthritis (RA) in humans. Methods KRN/NOD mice, which spontaneously develop arthritis, were treated with TNP-470, an angiogenesis inhibitor. Disease was monitored by use of clinical indices and histologic examinations; circulating blood levels of vascular endothelial growth factor were determined by enzyme-linked immunosorbent assay. Results In the preventive protocol, with TNP-470 administration at a dosage of 60 mg/kg of body weight, the onset of arthritis was delayed and its clinical intensity was rather mild; 100% of placebo-treated transgenic mice developed arthritis that led to severe articular destruction. At a dosage of 90 mg/kg of TNP-470, the appearance of clinical signs was delayed for a longer period of time and disease was almost abolished. The therapeutic regimen alleviated clinical signs only when given during the very early stage of disease. Reductions in cartilage and bone destruction by TNP-470 treatment were observed histologically, a feature that was still evident at 30 and 80 days after injections were withdrawn. Conclusion Our demonstration that in vivo administration of an angiogenesis inhibitor suppresses arthritis and protects from bone destruction provides new insight into the pathogenesis of the disease and opens new possibilities in the treatment of RA in humans.

Introduction L’association de tumeurs sebacees a des cancers visceraux fait classiquement evoquer le diagnostic de syndrome de Torre et Muir (STM). Nous presentons une malade chez qui le diagnostic final etait celui de constitutional mismatch repair deficiency (CMMRD), anciennement decrit sous le nom de syndrome de Turcot. Observations Une patiente de 37 ans, nee de parents cousins germains, avait pour antecedents un cancer du colon (a 23 ans puis 26 ans), un glioblastome (a 34 ans), un adenocarcinome de l’endometre (a 36 ans) et un adenocarcinome duodenal (a 37 ans). Sa mere avait eu un cancer du colon a 46 ans, il n’y avait pas d’autre antecedent familial. La patiente presentait depuis un an une masse sous-cutanee du thorax, adherente et inflammatoire. L’examen histologique montrait un sebaceome kystique dermo-hypodermique. La recherche d’instabilite des microsatellites etait negative sur ce sebaceome mais positive sur la piece de colectomie. L’immunohistochimie (IHC) ne montrait pas de perte de MLH1, MSH2 et MSH6 sur le colon ; en revanche l’anticorps anti-PMS2 ne marquait ni la tumeur, ni les tissus sains avoisinants. L’analyse genetique trouvait une mutation constitutionnelle bi-allelique de PMS2 , confirmant le diagnostic de CMMRD. Discussion Les tumeurs sebacees sont connues dans le STM, maladie autosomique dominante liee a une mutation heterozygote d’un des genes MMR (genes de reparation des mesappariements de l’ADN : MLH1 , MSH2 , MSH6 et PMS2 ), se manifestant surtout par des cancers du colon et de l’endometre. En cas de suspicion de STM, on recherche une instabilite des microsatellites et une perte d’expression d’une des proteines MMR sur la tumeur, alors qu’elle est normale dans les tissus sains, le mecanisme de cancerogenese etant une inactivation somatique de l’allele normal dans la tumeur. Dans le CMMRD, autosomique recessif, les deux alleles sont mutes de facon constitutionnelle, expliquant des cancers plus precoces, plus varies (notamment des tumeurs cerebrales et des hemopathies lymphoides), et parfois resistants aux chimiotherapies habituelles. Sur la peau, on peut voir des tumeurs sebacees, mais aussi des signes de neurofibromatose (taches cafe-au-lait, lentigines, neurofibromes), des macules hypochromes et des pilomatricomes. En cas de suspicion de CMMRD, l’IHC doit tester les 4 proteines MMR ( PMS2 etant le plus souvent en cause). Chez notre malade elle etait negative en tissu tumoral et sain pour PMS2, ce qui doit faire rechercher une mutation bi-allelique du gene. Il est important de poser le diagnostic de CMMRD pour proposer un genotypage aux apparentes, realiser un depistage des tumeurs plus large et plus frequent que dans un STM, et tenir compte de ce diagnostic dans la prescription de chimiotherapie. Conclusion Le diagnostic de CMMRD doit etre connu des dermatologues, et evoque devant l’association de cancers precoces multiples avec des tumeurs sebacees ou des signes de neurofibromatose.

Diffuse sclerosing osteomyelitis of the mandible has been described as a localized disease of unknown origin. We offer evidence that mandibular involvement in the recently described synovitis acne pustulosis hyperostosis osteitis syndrome exactly fits the accepted description for diffuse sclerosing osteomyelitis of the mandible. The clinical, radiologic, and pathologic findings in seven such cases are presented and discussed.

Mycosis fungoides during large cell transformation to lymphoma has a poor prognosis with mean survival of 36 months. Autologous stem cell transplantation is rarely proposed in this indication. We report the case of a young man still in complete remission for transformed mycosis fungoides 14 years after autologous stem cell transplantation.A 25-year-old man presenting eczema-like patches since childhood was treated by chemotherapy for multiple lymphadenopathies considered as Hodgkin's lymphoma. He was referred with diffuse skin tumours and infiltrated patches. Histology of tumour samples revealed atypical T-cell infiltrate with epidermotropism and presence of more than 25% of large CD30-positive cells. Non-infiltrated patches showed small T-cell lymphoma with epidermotropism. Histological verification of a previous lymphadenopathy confirmed the diagnosis of transformed mycosis fungoides. Despite multiple courses of chemotherapy, the disease progressed, with neurological involvement in particular. Because of tumour aggressiveness, autologous stem cell transplantation was performed and resulted in rapid regression of the tumours, lymphadenopathy and neurological symptoms. Non-transformed mycosis fungoides patches persisted but were controlled with topical mechlorethamine. This patient is still in complete remission for tumour and extracutaneous lesions 14 years after the autograft.This was probably a case of juvenile mycosis fungoides diagnosed and transformed in adult age. Neurological involvement by mycosis fungoides is rare and usually carries a drastic prognosis. To our knowledge, this is the longest remission of transformed mycosis fungoides seen after autograft. It highlights the value of this method in aggressive transformed mycosis fungoides, especially in patients ineligible for allograft.

Usually, temporal arteritis progresses as a chronic disease.The authors report the observation of a 74-year-old woman who presented with two acute flares of temporal arteritis with headache, fever and inflammatory syndrome, which have spontaneously resolved.The observations of auto-inflammatory attacks of arteritis disease are rare, but maybe underestimated. The pathophysiology remains unclear.

We report two cases of intravenous leiomyomatosis, a rarely described benign tumor, which illustrate potential complications resulting from metastatic dissemination. Both cases were fortuitously discoveries. We discuss the diagnostic problems, etiological factors, therapeutic management and prognosis. Hysterectomy with bilateral salpingo-oophorectomy and regular follow-up with cardiac and pulmonary monitoring were performed. Pelvic pain disappeared and the patients are recurrence-free. The main difficulties concerned early diagnosis, complete resection and prognosis. These cases illustrate the importance of careful pathology examination of all uterine myoma resection specimens.

Mycobacterium marinum is responsible for "fish tank granuloma", a chronic and sometimes disabling skin infection, which in France would be the most frequent "atypical" cutaneous mycobacteriosis. Tropical fish salesmen are concerned about this disease because they might sell contaminated fish and be infected themselves. A review of the literature indicated that one quarter of the patients were aquarium workers. This original study tested the salesmen's knowledge and behaviour regarding "fish tank granuloma".This national study consisted in an anonymous questionnaire with 24 questions.Among forty salesmen, six of them (15 p. 100) knew this human disease well. Thirty (75 p. 100) ignored it. Among the few salesmen (22.5 p. 100) who had studied to become aquarium workers, only one third were taught about Mycobacterium marinum. Eight salesmen (20 p. 100) were concerned about the human disease: three of them were personally infected and five others had known infected colleagues or purchasers. Most of the aquarium workers immersed their hands without gloves in the fish tanks every day. Only a few destroyed all the fishes from a contaminated tank.Most of the tropical fish salesmen ignored "fish tank granuloma" although 20 p. 100 of them were concerned. This chronic disease, sometimes disabling, should be better known by aquarium salesmen. The national tropical fish salesmen were informed of the results of this study.

Prostatic adenocarcinoma is exceptionally associated with cutaneous lesions. We describe a patient with cutaneous lymphangitis and paraneoplastic ichtyosis related to prostatic cancer.A 92 year-old man had been treated for five years for a prostatic carcinoma. An angiomatous lesion developed with in 3 months near the right breast. Physical examination revealed axillary node enlargement, a large skin angiomatous lesion, and ichtyosis. The skin biopsy of the angiomatous skin lesion demonstrated its prostatic origin with carcinomatous metastases in the lymphatic vessels. The ichtyosis was considered as paraneoplastic.Cutaneous metastases from prostatic carcinoma are rare. Less than 1 p. 100 of cutaneous metastases are of prostate origin despite the high frequency of this cancer in the general population. The clinical aspects - angiomatous lesion and paraneoplastic ichtyosis - are exceptional.

Diagnosis of retroperitoneal fibrosis is generally delayed and revealed by various non-specific signs. We report the case of an isolated lymphedema of the lower limb revealing retroperitoneal fibrosis complicating a metastatic squamous cell carcinoma.In an 83-year-old women, a lymphedema appeared that remained isolated for several months before being associated with alteration in general health. Morphological examinations showed bilateral compression of the urinary excretory tracts and led to the diagnosis of retroperitoneal fibrosis. Histological examination of a sub-clavicular adenopathy that had evolved over 9 months, confirmed the diagnosis of a metastatic squamous cell carcinoma of pulmonary cancer.Retroperitoneal fibrosis is an exceptional etiology that must be recognized in isolated lymphadomas of the lower limbs. In view of the possible tumoral origin of retroperitoneal fibrosis, any evocative sign accompanying the lymphedema must be searched for.

Immune restoration syndrome was first described in 1998 and involved mycobacterium avium complex. We report the case of a patient with acquired immunodeficiency syndrome who had disseminated cutaneous lesions due to Mycobacterium tuberculosis, following initiation of highly active antiretroviral therapy.A 42 year-old HIV-infected man, was admitted for fever, cough, nocturnal sweat and impaired of general condition. He had a viral load of 127,200 copies/ml and 199/ml CD4 T-cells. He was treated with triple tuberculosis combination therapy according to tuberculous contagium, positivity of the tuberculin intradermoreaction (15 mm) and right upper lung nodule on thoracic scan. M. tuberculosis was not found. Fever improved at day 3. Highly active antiretroviral therapy with zidovudine, lamivudine, indinavir, was started at day 11 and 33 days after, fever and dermohypodermal nodules with necrotising evolution appeared. Skin biopsy specimen showed tuberculoid granuloma. The levels of viral load and CD4 T-cells were less than 200 copies/ml and 497/ml respectively. Fever and cutaneous lesions spontaneously resolved without changing therapy.Immune restoration syndrome appears after initiation of antiretroviral therapy, in patients with advanced HIV infection and without prophylactic treatment versus MAC. This case report probably involves mycobacterium tuberculosis. Bacterial lysis and immune restoration take part in cutaneous pathogenesis. Subclinical mycobacterial infection should be monitored during initiation of antiretroviral therapy in patients with advanced HIV infection.

Systemic corticosteroid is the main treatment of severe forms of pemphigoid gestationis. We report a case of generalised pemphigoid gestationis successfully treated with very potent topical corticosteroid.A 37-year-old woman developed during her third pregnancy with a new partner an urticated generalised eruption associated with bullous lesions. The diagnosis of pemphigoid gestationis was confirmed by direct immunofluorescence which detected a linear C3 deposition along the basement membrane zone and the positivity of Herpes Gestationis Factor (10 units). Local treatment with potent corticosteroid (betamethasone dipropionate 0.05 p. 100) failed and the patient was successfully treated by clobetasol propionate 0.05 p. 100 cream. The infant, in good health, was not delivered prematurely.Severe form of pemphigoid gestationis are currently treated with 0.5 to 1 mg/kg/day of systemic corticosteroids, with maternal and pediatric possible side effects. As in bullous pemphigoid, this observation underlines the efficiency and good tolerance of very potent corticosteroid in severe forms of pemphigoid gestationis.

Background Reactive oxygen species (ROS) are suspected to play a role in human RA. Transient hypoxia and ischaemic-reperfusion phenomenon are involved in their chronic production, by monocytes and macrophages. Nevertheless the clinical interest of ROS scavengers is difficult to evaluate. Objectives Methods We studied the effects of Vitamin E (natural alpha tocopherol) on transgenic KRN/NOD mice, new model for rheumatoid arthritis. We determined clinical, histological and biochemical parameters so as to define the long-term benefit of Vitamin E in the disease. Results We found an increase in plasma Vit E, in treated mice. Vitamin E treatment did not modify the date of onset of the disease, neither the intensity of the disease (arthritic index and weight), but Vit-E prevented from articular destruction, suggesting uncoupling of the two phenomenon. Biochemical analysis, blood glutathion, urine isoprostane and the plasma hydroperoxide did not differ between Vit E-treated transgenic KRN/NOD mice and control mice. Blood chemiluminescence was increased in transgenic KRN/NOD mice as compared to non-transgenic mice, and was decreased in Vit E-treated transgenic KRN/NOD mice. TNF-a and IL1-b, low in control non-transgenic mice, were increased in transgenic KRN/NOD mice, however a significant decreased in IL-1b appeared in Vit E-treated mice. Conclusion These results suggest that ROS scavengers can modulate cytokines responses and provide new arguments for the interest of ROS scavengers in RA therapy.

We report a typical case of bullous pemphigoid (BP) associated with a neurological disorder and study a possible link between neurological disorders and BP. An 84-year-old hemiplegic woman presented with unilateral BP on the hemiparetic side. BP was confirmed by histological and immunofluorescence data. The medical records of the previous 46 consecutive patients with BP were retrospectively analyzed (average age: 79; median age: 85). Thirty of the 46 patients with BP had neurological disorders. These disorders included dementia, epilepsy, multiple sclerosis, cerebral stroke, Parkinson's disease, gonadotropic adenoma, trembling, dyskinesia, lumbar spinal stenosis. In a control group of the 46 consecutive oldest patients (older than 71; average age: 82,5; median age: 80) with another skin disease referred during the previous two-year-period to our one-day-unit only, 13 patients had a neurological disorder. This study demonstrates that there is a high prevalence of neurological disorders in patients with BP (p = 0.0004). A prospective case control study with neurological examination and psychometrical evaluation is warranted to confirm these data. We speculate that neuroautoimmunity associated with the aging process or neurological disorders may be involved in pemphigoid development via an autoimmune response against dystonin which shares homology with bullous pemphigoid antigen 1. Bullous pemphigoid could be considered to be a marker of neurological disorder.

Insulin injection sites rarely become infected. We report a case of Mycobacterium kansasii infection at the sites of insulin injection.An 84-year-old woman with insulin-dependent diabetes consulted for lesions on the anterior aspect of the thighs. She had papulonodules at the sites of insulin injection. These lesions progressed to hard fibrinonecrotic ulcerations with a raised erythematous border. There were no enlarged nodes locally. Laboratory tests were normal. Bacteriological samples were negative at direct examination. Histology was not specific. The biopsy cultures finally found an atypical mycobacteria, Mycobacterium kansasii. The clinical course was favorable with clarithromycin alone.Only 35 cases of Mycobacterium kansasii skin infections have been reported in the literature. This is the first case observed in a diabetic subject and the first treated with clarithromycin alone. This case illustrates the potential, though exceptional, risk of insulin bottle contamination by an environmental germ.

There is a current debate regarding the association of human herpesvirus 6 (HHV-6) infection and drug reaction with eosinophilia and systemic symptoms (DRESS).Seven consecutive patients hospitalized with DRESS were enrolled in a prospective study to evaluate evidence of active HHV-6 infection.The imputable drugs were carbamazepine (5 patients), ibuprofen (1 patient), and sulfasalazine (1 patient). All patients were seropositive for anti-HHV-6 IgG antibodies. Anti-HHV-6 IgM antibodies were detected in 4 of the 7 patients with a seroconversion in 2 patients. Neither anti-cytomegalovirus nor anti-Epstein-Barr virus early antigen IgM antibody was detected. Human herpesvirus 6 genome was not detected by polymerase chain reaction in the first serum sample of all patients. It was weakly detected in skin lesions in the last patient tested by polymerase chain reaction but was not found in uninvolved skin.The results suggest an association between HHV-6 active infection (primo-infection or reactivation) and severe DRESS. Absence of anti-cytomegalovirus or anti-Epstein-Barr virus early antigen IgM antibodies argues against a nonspecific viral reactivation. Human herpesvirus 6 infection may play a role in the development of DRESS in susceptible patients. Some drugs with reactive metabolites could favor reactivation and propagation of HHV-6.

Clinical manifestations of primary parvovirus B19 infection vary greatly. Epidermal megalerythema is the most common feature. We report a particular form resembling a drug-induced hypersensitivity reaction.A 19-year-old man had a scarlatiniform eruption associated with multiple node enlargement, elevated liver enzymes and a abnormal white cell count with mononucleosis and lymphopenia, similar to that observed in hypersensitivity reactions. Seroconversion and positive PCR search for viral DNA established the diagnosis of primary parvovirus B19 infection. The spontaneous course was favorable with no recurrence at one month.The clinical features and laboratory findings in this case of parvovirus B19 infection closely resembled drug-induced hypersensitivity syndrome. The role of viral agents in the development of hypersensitivity reactions have been suggested. It is important to look for viral infections in clinical presentations mimicking drug-induced hypersensitivity.

We report four cases of eczema induced by alpha interferon in atopic patients treated for chronic hepatitis C.Eczema developed in 4 patients with certain (3 cases) or possible (1 case) atopy treated by subcutaneous injections of alpha interferon for hepatitis C virus infections. Delay to onset was 3 weeks to 6 months. Interferon was highly likely the causal agent: lesions started at site of interferon injection, followed the rhythm of interferon injections (three cases), disappeared at interferon withdrawal. In two patients, the lesions diffused to other sites. Both Introna and Roféron were used. Three patients also took ribavirine. The possible role of a contact factor (antiseptic.) was ruled out. Skin tests (patch tests, prick tests, intradermal reactions) were negative for interferon alpha and for a standard battery.The role of interferon in the induction of skin diseases or its influence on the course of certain dermatoses is well known. In atopic patients, interferon might induce eczema via an immunomodulator rather than an allergic mechanism since skin tests (performed in one patient) were negative. This observation is similar to that in psoriasis induced by interferon in predisposed subjects who develop skin lesions at injection sites which sometimes diffuse to distant localizations. The role of other factors (hepatitis C virus infection, ribavirine) remains unknown; they might participate in this mechanism by aggravating skin dryness.

Langerhans cell histiocytosis is an uncommon clonal disorder. Its reactional or genetic nature is debated.Three patients aged 71, 75 and 73 years with Langerhans cell histiocytosis also developed myelomoncytic leukemia (AML4, AML5, and chronic myelomonocytic leukemia). In two cases, there was no prior treatment which could potentially induce leukemia. In the third case, AML4 occurred shortly (10 months) after initiation of a vinblastin treatment.This pathogenic association suggests a common origin for these two conditions, corresponding to an anomalous pluripotent stem cell line. These cases provide further evidence favoring the hypothesis of a genetic origin rather than a reactional origin for Langerhans cell histiocytosis.

Coexistence of a systemic vasculitis and an epithelioma is rare. The prevalence of malignancies in patients with vasculitis has been estimated at about 5%, with about two thirds hematological malignancies and one third solid tumors, although the actual figure may be higher. We report three cases of vasculitis in patients with solid tumors. A review of the literature yielded 77 additional cases. A total of 56 cases were fully documented. The sex ratio was 1.26, and mean age was 62.5 years. The most common vasculitides were leukocytoclastic vasculitis (33%), polyarteritis nodosa (16.5%) and Henoch-Schönlein purpura. The most common sites of cancer formation were the lung (23%), the digestive system (17.5%) and the kidney (14%). The diagnosis of vasculitis antedated that of cancer in most patients. Radical cancer treatment was often followed by resolution of the vasculitis and vasculitis recurrences occurred concomitantly with tumor recurrences in some patients, suggesting that the vasculitis was a paraneoplastic syndrome. Patients with unexplained chronic vasculitis with or without a history of cancer should undergo extensive investigations for an occult neoplasm.

We report the first case of cutaneous and articular mucinosis with presence of mucin deposits within the synovium in an HIV-positive patients. Presentation was asymmetric polyarthritis, and subcutaneous nodules developed two months later. Treatment was with hydroxychloroquine. The outcome of both the skin and joint lesions was favourable.

Resume Introduction Typiquement, la maladie de Horton evolue de maniere chronique. Fait clinique Les auteurs rapportent l’observation d’une femme de 74 ans presentant deux poussees aigues de maladie de Horton avec cephalees, fievre et syndrome inflammatoire biologique, spontanement resolutives. Discussion Les observations d’acces auto-resolutifs de maladie de Horton sont rares mais peut-etre sous estimees. Leur physiopathologie reste incertaine.

Paraneoplastic pemphigus is an autoimmune bullous disease described by precise clinical, histological and immunological features presented by Anhalt in 1990. Prognosis is very severe and depends on the associated neoplasia and the gravity of the mucosal damage.Paraneoplastic pemphigus was diagnosed in a 62-year-old man with chronic lymphoid leukemia. The course was favorable up to one-year follow-up after general corticosteroid therapy.This case illustrates that paraneoplastic pemphigus can be controlled by general corticosteroids and would suggest the severe prognosis may be improved.

We report five cases of pigmented erythroderma occurring during AIDS, noteworthy for its unusual hyperpigmented feature, its advent at the terminal stages of AIDS, and an CD8 cells dermal infiltrate.It is a retrospective study of five patients infected with HIV: a woman infected by transfusion and four homosexual men, average 55 years old. No one was intravenous drug user. They were all severely immunocompromised; HTLV I/II serology was negative. Skin biopsies were studied with light microscopy (Hematoxylin-eosin) and immunohistochemical studies were performed on frozen sections.The patients had an erythroderma of particular interest because of the associated hyperpigmentation, the severe repercussion (pruritus, weight loss), and the difficulty in treating (except systemic corticosteroids). The histology demonstrated a mononuclear dermal lymphocytic infiltrate, without epidermotropism and atypical cytonuclear feature. The phenotype of the infiltrate was uniformly of the suppressor-cytotoxic subset (CD8+, CD4-).Our cases are like those previously described as "Pseudo-Sezary", mimicking a lymphoma during AIDS. Numerous factors are probably the cause of this hyperpigmented erythroderma: HIV, CD8 cells...This severe skin disease, complicating AIDS, seems very particular, but not yet clearly defined. In practice, the problem remains the treatment of this severe erythroderma, because only the systemic corticosteroids are effective, but this is debatable during the treatment of AIDS.

Extrapulmonary sarcoidosis, and particularly the presence of musculoskeletal complications of the disease, may require chronic corticosteroid therapy. In five patients with biopsy-proven sarcoidosis and presenting recalcitrant forms of the disease, we introduced low-dose oral methotrexate (MTX) [10 mg/week (7.5-15)] for 30 months (16-34) to control the clinical and biological symptoms as well as to try to reduce the intolerated steroid posology. Beneficial effects were observed within 8-12 weeks in all patients, which allowed a reduction of 59% (35-75) of the corticosteroid posology, and maintained with a follow-up of 3 yr in 4/5 patients. No significant toxicity was observed. MTX appears to be an efficient, safe and corticosteroid-sparing therapeutic agent for the treatment of recalcitrant musculoskeletal manifestations of sarcoidosis.