RESUMO
The breasts are a common location for diffuse dermal angiomatosis (DDA) in a context of obesity and macromastia. The typical clinical presentation includes erythematous or purplish plaques, reticulated telangiectasias, and sometimes livedo reticularis, often complicated by painful ulcerations of the breasts. Biopsy usually confirms a dermal proliferation of endothelial cells staining positively for CD31, CD34 and SMAa and negatively for HHV8. We report herein a woman with DDA of the breasts presenting as diffuse livedo reticularis and acrocyanosis, both long-standing and considered idiopathic following extensive investigations. Since a biopsy of the livedo did not document DDA features in our case, we suggest that our patient's livedo reticularis and telangiectasias could constitute a vascular predisposition for DDA, as its pathogenesis frequently involves an underlying disease involving ischemia, hypoxia, or hypercoagulability.
Assuntos
Angiomatose , Livedo Reticular , Telangiectasia , Feminino , Humanos , Células Endoteliais/patologia , Angiomatose/patologia , Mama/patologia , Telangiectasia/complicaçõesRESUMO
BACKGROUND: Primary cutaneous CD8+ aggressive, epidermotropic, cytotoxic T-cell lymphoma is a rare disease with a poor prognosis. Herein we report a new case, with facial lesions, which was difficult to diagnose. PATIENTS AND METHODS: A 39-year-old woman was hospitalized for ulcerated nodules on the face that had been developing rapidly for 8 weeks. She had visited Djerba, Tunisia, 3 months earlier. No abnormalities were found on previous routine blood tests. Histopathological analysis of a skin biopsy had revealed non-specific lymphocytic infiltrate. Various therapies, including amoxicillin/clavulanic acid, valaciclovir, corticosteroids, colchicine and doxycycline, proved ineffective. Screening of the cutaneous sample for leishmaniasis proved positive using PCR but negative by direct examination and culture. Treatment was initiated with meglumine antimoniate. A further cutaneous biopsy revealed diffuse lymphocytic proliferation and led to a diagnosis of cutaneous CD8+ aggressive, epidermotropic, cytotoxic T-cell lymphoma. A PET scan showed multiple sites of hypermetabolism affecting the face and lymph nodes. Meglumine antimoniate was stopped and the patient experienced complete remission after chemotherapy. CONCLUSION: Ulcerated nodules with acute progression on acral sites are characteristic of cutaneous CD8+ aggressive, epidermotropic, cytotoxic T-cell lymphoma. In our case, the positive result of PCR screening for Leishmania that was ultimately considered a false positive was a confounding factor in the diagnostic process. Regarding therapy, aggressive treatment strategies such as multiagent chemotherapy and hematopoietic stem-cell transplantation are needed due to the rapid progression of the lymphoma.
Assuntos
Linfoma Cutâneo de Células T , Neoplasias Cutâneas , Adulto , Linfócitos T CD8-Positivos , Feminino , Humanos , Linfonodos , Linfoma Cutâneo de Células T/diagnóstico , Linfoma Cutâneo de Células T/tratamento farmacológico , Pele , Neoplasias Cutâneas/diagnóstico , Neoplasias Cutâneas/tratamento farmacológicoRESUMO
BACKGROUND: Mutations of BRAF, NRAS and c-KIT oncogenes are preferentially described in certain histological subtypes of melanoma and linked to specific histopathological features. BRAF-, MEK- and KIT-inhibitors led to improvement in overall survival of patients harbouring mutated metastatic melanoma. OBJECTIVES: To assess the prevalence and types of BRAF, NRAS, c-KIT and MITF mutations in cutaneous and mucous melanoma and to correlate mutation status with clinicopathological features and outcome. METHODS: Clinicopathological features and mutation status of 108 samples and of 98 consecutive patients were, respectively, assessed in one retrospective and one prospective study. Clinicopathological features were correlated with mutation status and the predictive value of these mutations was studied. RESULTS: This work identified significant correlations between BRAF mutations and melanoma occurring on non-chronic sun-damaged skin and superficial spreading melanoma (P < 0.05) on one hand, and between NRAS mutations and nodular melanoma (P < 0.05) on the other hand. Younger age (P < 0.05), microscopic (P < 0.05) and macroscopic (P < 0.05) lymphatic involvement at diagnosis of primary melanoma were significantly linked to BRAF mutations. A mutated status was a positive predictive factor of a response to BRAF inhibitors (OR = 3.44). Mutated melanoma showed a significantly (P = 0.038) higher objective response rate to cytotoxic chemotherapy (26.3%) than wild-type tumours (6.7%). CONCLUSION: Clinical and pathological characteristics of the primary melanoma differed between wild-type and BRAF- or NRAS-mutated tumours. Patients with BRAF-mutated tumours were younger at diagnosis of primary melanoma. Patients carrying mutations showed better responses better to specific kinase inhibitors and interestingly also to systemic cytotoxic chemotherapy.
Assuntos
GTP Fosfo-Hidrolases/genética , Melanoma/genética , Proteínas de Membrana/genética , Fator de Transcrição Associado à Microftalmia/genética , Mutação , Proteínas Proto-Oncogênicas B-raf/genética , Proteínas Proto-Oncogênicas c-kit/genética , Neoplasias Cutâneas/genética , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Mucosa , Prognóstico , Estudos Prospectivos , Estudos Retrospectivos , Adulto JovemRESUMO
Antibiotics, of which Fleming has identified the first representative, penicillin, in 1928, allowed dramatical improvement of the treatment of patients presenting with infectious diseases. However, once an antibiotic is used, resistance may develop more or less rapidly in some bacteria. It is thus necessary to develop therapeutic alternatives, such as the use of probiotics, defined by the World Health Organization (WHO) as "micro-organisms which, administered live and in adequate amounts, confer a benefit to the health of the host". The scope of these micro-organisms is broad, concerning many areas including that of infectious diseases, especially respiratory infections. We describe the rational use of probiotics in respiratory tract infections and detail the results of various clinical studies describing the use of probiotics in the management of respiratory infections such as nosocomial or community acquired pneumonia, or on specific grounds such as cystic fibrosis. The results are sometimes contradictory, but the therapeutic potential of probiotics seems promising. Implementing research to understand their mechanisms of action is critical to conduct therapeutic tests based on a specific rational for the strains to be used, the dose, as well as the chosen mode and rhythm of administration.
Assuntos
Pneumonia Bacteriana/terapia , Probióticos/uso terapêutico , Animais , Infecções Comunitárias Adquiridas/microbiologia , Infecções Comunitárias Adquiridas/terapia , Infecção Hospitalar/microbiologia , Infecção Hospitalar/terapia , Fibrose Cística/complicações , Suscetibilidade a Doenças , Método Duplo-Cego , Humanos , Sistema Imunitário/imunologia , Camundongos , Microbiota , Pneumonia Bacteriana/microbiologia , Pneumonia Associada à Ventilação Mecânica/microbiologia , Pneumonia Associada à Ventilação Mecânica/fisiopatologia , Pneumonia Associada à Ventilação Mecânica/terapia , Probióticos/efeitos adversos , Doença Pulmonar Obstrutiva Crônica/complicações , Percepção de Quorum , Ensaios Clínicos Controlados Aleatórios como Assunto , Sistema Respiratório/microbiologia , Especificidade da EspécieRESUMO
BACKGROUND: Several case reports suggested that tumour necrosis factor-α (TNF) inhibitors might increase the incidence and/or alter the natural course of melanoma towards a more aggressive behaviour. OBJECTIVE: Our objective was to point if history of melanoma in patients exposed to TNF inhibitors could present with a particular pattern at diagnosis or during follow-up. METHODS: We performed a retrospective multicentre study settled in the West part of France to collect and analyse all cases of patients with melanoma who received anti-TNF therapy. RESULTS: Fifteen cases were included. First, 10 patients (mean age: 55.6 years; sex ratio: 1) had a melanoma diagnosed after TNF inhibitors initiation. The mean duration between initiation of treatment and melanoma was 48.7 months. Two patients died of metastatic disease. Second, four patients had a past history of melanoma before anti-TNF therapy (mean duration of treatment: 10.8 months). None experienced a progression of melanoma disease. Last, one woman had a past history of melanoma before and then developed a second melanoma when exposed to biotherapy. CONCLUSION: Our case series does not reveal a distinct profile of melanoma in the patients exposed to TNF inhibitors. Additional prospective trials including larger number of patient are needed to demonstrate the possible link between biological therapy with TNF inhibitors and development of melanoma.
Assuntos
Produtos Biológicos/efeitos adversos , Produtos Biológicos/uso terapêutico , Melanoma/epidemiologia , Doenças Reumáticas/tratamento farmacológico , Neoplasias Cutâneas/epidemiologia , Fator de Necrose Tumoral alfa/antagonistas & inibidores , Adulto , Idoso , Idoso de 80 Anos ou mais , Antirreumáticos/efeitos adversos , Antirreumáticos/uso terapêutico , Feminino , Seguimentos , França , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , Recidiva Local de Neoplasia/epidemiologia , Estudos Retrospectivos , Fatores de RiscoRESUMO
We report herein a patient treated with vemurafenib for a stage IV melanoma who developed an eruption related to eccrine squamous syringometaplasia (ESS). This entity is a well-described side effect of cytostatic therapies used for malignant neoplasia and is clinically characterized by a symmetric cutaneous eruption composed of papules and vesicles preferentially located on fold and intertriginous areas. It is histologically defined by a squamous metaplasia of eccrine ductal epithelium. ESS represents another skin eruption to be added to the list of cutaneous adverse events associated with vemurafenib, a selective BRAF inhibitor used to treat patients with metastatic melanoma harboring the V600E mutation. The discussion focuses on the pathogenesis of ESS secondary to vemurafenib and on alternative diagnoses.
Assuntos
Antineoplásicos/efeitos adversos , Toxidermias/etiologia , Glândulas Écrinas/patologia , Células Epiteliais/patologia , Indóis/efeitos adversos , Melanoma/tratamento farmacológico , Neoplasias Cutâneas/tratamento farmacológico , Sulfonamidas/efeitos adversos , Adulto , Toxidermias/patologia , Humanos , Masculino , Melanoma/secundário , Metaplasia/induzido quimicamente , Metaplasia/patologia , Neoplasias Cutâneas/patologia , VemurafenibAssuntos
Amiodarona/análogos & derivados , Antiarrítmicos/efeitos adversos , Fibrilação Atrial/tratamento farmacológico , Toxidermias/diagnóstico , Febre/induzido quimicamente , Idoso , Amiodarona/efeitos adversos , Biópsia , Diagnóstico Diferencial , Dronedarona , Toxidermias/patologia , Febre/patologia , Humanos , Masculino , Pele/efeitos dos fármacos , Pele/patologiaRESUMO
BACKGROUND: EB naevus (EBN) are little-known, atypical, eruptive, pigmented melanocytic lesions that may occur in former sites of bullae occurring in epidermolysis bullosa hereditaria (EBH). We sought to describe the characteristics of such lesions and assess their course. PATIENTS AND METHODS: This was a retrospective, two-centre study in which data was collated from the medical files of patients with EBN. We analyzed the patients' demographical data as well as the clinical, dermatoscopic, pathological features of EBN and their progression. RESULTS: Eight patients were studied: they were principally Caucasian (5/8), with a sex ratio of 1. All variants of EBH were represented and most were recessive (63%). We analysed 22 EBN, all atypical and emerging before the age of 10 years (73%), ubiquitously distributed and measuring greater than 5 cm(2) (25%). Of the 13 EBN subjected to dermatoscopy, 12 exhibited a benign reticular pattern. Four were biopsied, and analysis revealed three common naevi and one lentigo. After a median follow-up of 8 years, the EBN seen were either stable (68%), had regressed (23%) or had disappeared (one case). No cases of melanoma were diagnosed. DISCUSSION: EBN are acquired and atypical pigmented naevi. Sixty-four cases of EBN have been reported in the literature up to date. The dermatoscopic features may be evocative of melanoma (17/23 EBN), but to our knowledge no cases of melanoma at a naevus site have been reported. Recessive transmission of EBH appears to be a risk factor (63% of cases), a finding supported by certain pathophysiological hypotheses. CONCLUSION: EBN present atypical clinical and dermatoscopic features. However, while prophylactic total excision did not appear warranted in the absence of any reported cases of melanoma, regular clinical follow-up is recommended.
Assuntos
Epidermólise Bolhosa Distrófica/diagnóstico , Nevo Pigmentado/diagnóstico , Neoplasias Cutâneas/diagnóstico , Adolescente , Biópsia , Criança , Pré-Escolar , Dermoscopia , Diagnóstico Diferencial , Epidermólise Bolhosa Distrófica/patologia , Feminino , Humanos , Lactente , Lentigo/diagnóstico , Lentigo/patologia , Masculino , Nevo Pigmentado/patologia , Fatores de Risco , Pele/patologia , Neoplasias Cutâneas/patologiaAssuntos
Pênfigo/diagnóstico , Administração Tópica , Anti-Inflamatórios/uso terapêutico , Biópsia , Clobetasol/uso terapêutico , Diagnóstico Diferencial , Seguimentos , Humanos , Imunoglobulina A/metabolismo , Masculino , Microscopia de Fluorescência , Pênfigo/tratamento farmacológico , Pênfigo/patologia , Pele/patologia , Adulto JovemRESUMO
INTRODUCTION: Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a severe form of adverse drug reaction. Strontium ranelate has recently been authorised for postmenopausal osteoporosis. We report a case of strontium ranelate-induced DRESS complicated by linear Ig A dermatosis due to vancomycin. CASE REPORT: A 77-year-old woman with osteoporosis had been treated by strontium ranelate for 4 weeks when she developed a febrile generalized skin rash. Blood tests showed eosinophilia (12.74 × 10(9)/L) and liver damage. A diagnosis of DRESS was made, leading to discontinuation of strontium ranelate and prescription of systemic corticosteroids. Two days later, methicillin-resistant Staphylococcus aureus bacteraemia occurred and treatment with vancomycin was started. The liver dysfunction resolved. After two weeks of antibiotherapy, bullous lesions were noted on the thighs. Skin biopsy results suggested a diagnosis of linear IgA bullous dermatosis. Vancomycin was stopped. Two weeks later, the eruption resolved. The eosinophil count gradually returned to normal after four months of corticosteroid therapy. DISCUSSION: More than 15 cases of DRESS syndrome have been reported in Europe, including 2 deaths related to ranelate strontium, prompting European health authorities to publish a warning concerning the risk of strontium ranelate-induced DRESS. A particular feature of our patient was complication with linear IgA bullous dermatosis caused by vancomycin. In conclusion, it is essential to be aware of the risk of severe cutaneous reaction to strontium ranelate, a new drug used to treat osteoporosis.
Assuntos
Conservadores da Densidade Óssea/toxicidade , Doença Hepática Induzida por Substâncias e Drogas/diagnóstico , Toxidermias/diagnóstico , Eosinofilia/induzido quimicamente , Compostos Organometálicos/toxicidade , Osteoporose Pós-Menopausa/tratamento farmacológico , Tiofenos/toxicidade , Idoso , Antibacterianos/efeitos adversos , Antibacterianos/uso terapêutico , Anti-Inflamatórios/administração & dosagem , Anti-Inflamatórios/efeitos adversos , Bacteriemia/diagnóstico , Biópsia , Conservadores da Densidade Óssea/administração & dosagem , Doença Hepática Induzida por Substâncias e Drogas/tratamento farmacológico , Toxidermias/tratamento farmacológico , Eosinofilia/diagnóstico , Feminino , Técnica Direta de Fluorescência para Anticorpo , Humanos , Imunoglobulina A/metabolismo , Staphylococcus aureus Resistente à Meticilina , Compostos Organometálicos/administração & dosagem , Prednisona/administração & dosagem , Prednisona/efeitos adversos , Pele/patologia , Dermatopatias Vesiculobolhosas/induzido quimicamente , Dermatopatias Vesiculobolhosas/diagnóstico , Dermatopatias Vesiculobolhosas/patologia , Infecções Estafilocócicas/diagnóstico , Síndrome , Tiofenos/administração & dosagem , Vancomicina/efeitos adversos , Vancomicina/uso terapêuticoRESUMO
In a context of viral gastroenteritis, we report an unusual case of sudden death in an 8-year-old child. The only macroscopic abnormality observed in the autopsy was a diffuse mesenteric adenitis. Organ samples were taken for histopathological examination and a diffuse lymphocytic infiltration was observed. A sinusoidal histiocytic hyperplasia was found in the lymph nodes. Microscopic examination of the lungs and the lymph nodes revealed haemophagocytic lesions (lymphocytes within the cytoplasm of histiocytic macrophages). Immunohistochemical studies demonstrated that the histiocytes were CD68+ PS100- CD1A-. Following this microscopic examination macrophage activation or haemophagocytic syndrome was diagnosed. The syndrome is a distinct clinical entity characterised by fever, pancytopaenia, splenomegaly, and haemophagocytosis in the bone marrow, liver and lymph nodes. It is a clinical entity that is very difficult to diagnose due to the lack of specific clinical signs. It is generally a complication of an infectious process, an aggravation of an auto-immune disease or a complication of a neoplastic process. The physiopathology involves a disregulation of T lymphocytes and particularly T helper lymphocytes. To make this diagnosis the anatomopathological examination must be performed by an experienced practitioner. The presence of a lymphocyte infiltrate of macrophagic histiocytes in myeloid organs and especially positive CD68+ immune markers are the anatomopathological proofs of diagnosis. The autopsy examination must be carefully performed and include systematic sample harvesting for anatomopathological examination. The results of all these examinations taken together allow the diagnosis of haemophagocytic syndrome to be finally made.
Assuntos
Morte Súbita/patologia , Linfo-Histiocitose Hemofagocítica/patologia , Autopsia , Criança , Diagnóstico Diferencial , Medicina Legal , Humanos , MasculinoRESUMO
BACKGROUND: Reticular erythematous mucinosis (REM) syndrome is a rare disorder. Its clinical course is cyclic with remissions and exacerbations. In this disease, photosensitivity has previously been noticed but rarely demonstrated. We report three new cases with positive photobiological investigation. CASE REPORTS: Three patients (two males, one female) with a mean age 47 years were seen with reticular erythematous papules on the upper chest and or back. After sun exposure, the lesions were exacerbated. Skin biopsies showed dermal lymphocytic perivascular infiltration with mucin deposition between collagen bundles. Direct immunofluorescence was negative. Antinuclear antibodies were absent. In cabin, ultraviolet (UV)A exposure reproduced clinically and histologically REM lesions in our cases. UVA and UVB provocating phototests were negative. In all patients treatment with oral antimalarials and external photoprotection was effective. CONCLUSIONS: In our patients, we confirm the photosensitive feature of REM syndrome by provocative irradiation in UVA cabin. The mechanism of triggering is actually unclear. It is supposed that UV radiation, heat, and perspiration are necessary to reveal this affection.
Assuntos
Mucinoses/fisiopatologia , Transtornos de Fotossensibilidade/fisiopatologia , Luz Solar/efeitos adversos , Adulto , Idoso , Biópsia , Colágeno , Eritema/fisiopatologia , Feminino , Humanos , Linfócitos/patologia , Masculino , Pessoa de Meia-Idade , Mucinoses/patologia , Mucinas , Fotobiologia , Transtornos de Fotossensibilidade/patologia , Síndrome , Raios Ultravioleta/efeitos adversosAssuntos
Transplante de Medula Óssea , Doença Enxerto-Hospedeiro/etiologia , Dermatopatias Vasculares/etiologia , Adulto , Evolução Fatal , Doença Enxerto-Hospedeiro/patologia , Humanos , Leucemia Linfocítica Crônica de Células B/terapia , Masculino , Dermatopatias Vasculares/patologia , Transplante HomólogoRESUMO
Isolated aspergillosis of the sphenoid sinus is a rare condition that is frequently diagnosed at a late stage because of its nonspecific and varying symptomatology. We report the case of a 75-year-old diabetic woman with a long history of retroorbital pain before she developed a subacute cavernous sinus syndrome. Neuroimaging including CT scan and MRI suggested a malignant tumor involving the sphenoid sinus but the diagnosis of aspergillosis was made intraoperatively and by histopathological examination. Soon after surgical drainage of the sphenoid sinus and systemic anti-fungal drug therapy, both retroorbital pain and cavernous sinus syndrome had completely resolved. This case emphasizes the fact that invasive isolated sphenoid sinus aspergillosis must be considered in the list of lesions causing sinus cavernous syndrome.
Assuntos
Aspergilose/microbiologia , Aspergilose/patologia , Seio Esfenoidal/microbiologia , Seio Esfenoidal/patologia , Idoso , Aspergilose/cirurgia , Complicações do Diabetes , Feminino , Imunofluorescência , Humanos , Imageamento por Ressonância Magnética , Dor/etiologia , Seio Esfenoidal/cirurgia , Tomografia Computadorizada por Raios XRESUMO
INTRODUCTION: Cutaneous metastatic Crohn's disease is a granulomatous inflammation of the skin that is noncontiguous to the gastrointestinal tract. CASE REPORT: A 42-year-old man with Crohn's disease is admitted for antibiotic resistant erysipela-like dermo-hypodermitis. The presence of granulomatous lesions on skin biopsy, the absence of any infectious agent identified despite extensive investigations and the dramatic improvement observed with corticosteroid eventually lead to the diagnosis of cutaneous metastatic Crohn's disease. CONCLUSION: During Crohn's disease, biopsy should be considered for every undiagnosed skin lesion as the characteristics of cutaneous metastatic Crohn's disease are not specific. The presence of a non caseous granulomatous dermal infiltration suggests the diagnosis and should make consider the initiation of corticosteroid and the discontinuation of others inappropriate therapeutics (i.e. surgical treatment, antibiotics).
Assuntos
Doença de Crohn/patologia , Granuloma/etiologia , Dermatopatias/etiologia , Adulto , Doença de Crohn/diagnóstico , Diagnóstico Diferencial , Granuloma/diagnóstico , Granuloma/patologia , Humanos , Masculino , Dermatopatias/diagnóstico , Dermatopatias/patologiaRESUMO
BACKGROUND: We present a case of a myospherulosis, describe the underlying cause and discuss ways of preventing its development. CASE REPORT: A 35-year-old man presented myospherulosis of the maxillary sinus caused by an antibiotic ointment placed in the sinus three years earlier during a Caldwell Luc procedure. The patient underwent a second Calwell Luc procedure. Outcome was uneventful and no recurrence was noted. DISCUSSION: Histological examination should be ordered at reoperation and petroleum-based ointments and packings should be avoided in sinus surgery.
Assuntos
Seio Maxilar , Doenças dos Seios Paranasais/cirurgia , Adulto , Humanos , Doença Iatrogênica , Masculino , Doenças dos Seios Paranasais/etiologia , Doenças dos Seios Paranasais/patologiaRESUMO
Primary adenocarcinoma of the urinary tract are uncommon. But secondary involvement of pyelocalyceal system by metastasis of colorectal origin is rare. We report a case of late rectal metastasis with renal pelvis growth presenting as a pyonephrosis. This study emphasizes the relevance of cytokeratin 7 and 20 immunostaining in such differential diagnosis.
Assuntos
Adenocarcinoma/patologia , Adenocarcinoma/secundário , Neoplasias Renais/secundário , Neoplasias Retais/patologia , Idoso , Diagnóstico Diferencial , Humanos , Imuno-Histoquímica , Proteínas de Filamentos Intermediários/análise , Queratina-20 , Queratina-7 , Queratinas/análise , Neoplasias Renais/patologia , MasculinoRESUMO
Single chain variable fragments (scFv) of the murine monoclonal antibody HD37 specific to human B-cell antigen CD19 were constructed by joining the VH and VL domains with linkers of 18, 10, 1 and 0 residues. ScFv-18 formed monomers, dimers and small amounts of tetramers; scFv-10 formed dimers and small amounts of tetramers; scFv-1 formed exclusively tetramers; scFv-0 formed exclusively trimers. The affinities of the scFv-10 (diabody) and scFv-1 (tetrabody) were approximately 1.5- and 2.5-fold higher, respectively, than that of the scFv-0 (triabody). The tetrabody displayed a significantly prolonged association with cell-bound antigen (t1/2 cell surface retention at 37 degrees C of 26.6 min) compared to both the diabody (13.3 min) and triabody (6.7 min). This increase in avidity of the tetrabody combined with its larger size could prove to be particularly advantageous for imaging and the immunotherapy of B-cell malignancies.