RESUMO
Langerhans cell histiocytosis (LCH) is a rare systemic disease caused by proliferation of mature histiocytes; its association to histiocyto fibroma is rarely reported. It rarely affects adults. We report a case of systemic LCH, in an adult patient with osteolytic lesion causing a fistula between the left nasal cavity and hard palate, involving the bone, lung, lymph node and associated to multiple histiocyto fibroma. The patient was operating for a fistula, and he was treated by chemotherapy and corticosteroids. Langerhans´ cell histiocytosis is a rare case, especially in adult patient. The diagnosis was based on histological and immunohistochemical analyses. This patient was treated by steroids and chemotherapy.
Assuntos
Histiocitose de Células de Langerhans/diagnóstico , Cavidade Nasal/anormalidades , Fístula Bucal/diagnóstico , Corticosteroides/administração & dosagem , Adulto , Histiocitose de Células de Langerhans/complicações , Histiocitose de Células de Langerhans/tratamento farmacológico , Humanos , Masculino , Cavidade Nasal/cirurgia , Fístula Bucal/etiologia , Fístula Bucal/cirurgia , Vimblastina/administração & dosagemRESUMO
Lysosomial diseases and autoimmune diseases are systemic disorders. Their clinical manifestations can overlap with the broad spectrum of one another. Their association has been rarely reported. We report a new case of systemic lupus erythematous (SLE) associated to antiphospholipid syndrome (APS) and Hashimoto thyroiditis occurring in Niemann-Pick disease (NPD) type B patient. A 42-year-old woman with a familial history of NPD was diagnosed with a NPD type B at the age of ten. Twenty years later (2008), she complained of inflammatory arthralgia with acute dyspnea. She was diagnosed with SLE (according to ACR criteria) and Hashimoto disease with positive IgG anti-cardiolipin and IgA anti-beta2 glycoprotein. In 2018, she presented a left segmental pulmonary embolism. Antiphospholipid syndrome was retained. She was treated with steroids, hydroxychloroquine, anticoagulation therapy and levothyroxine. Her SLE treatment was re-enforced by cyclophosphamide because of corticosteroid dependence and recurrent hemolytic crises.
Assuntos
Síndrome Antifosfolipídica/diagnóstico , Doença de Hashimoto/diagnóstico , Lúpus Eritematoso Sistêmico/diagnóstico , Doenças de Niemann-Pick/diagnóstico , Adulto , Síndrome Antifosfolipídica/tratamento farmacológico , Dispneia/etiologia , Feminino , Doença de Hashimoto/tratamento farmacológico , Humanos , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Doenças de Niemann-Pick/tratamento farmacológico , Embolia Pulmonar/diagnóstico , Tomografia Computadorizada por Raios XRESUMO
Cellulitis is a frequent soft tissue and skin infection. The lower limbs are affected in 70 to 80% of cases. Cellulitis in aged persons is not yet well described in literature. A retrospective descriptive study conducted in the Internal Medicine Department of Sahloul hospital in Sousse in Tunisia. It included patients whose age was up to 65 years old admitted into hospital for cellulitis of the legs, the arms or the face. One hundred fifty eight patients with a mean age of 73 years old (range: 65 to 94 years old) were included. Female to male sex ratio was 0.68. Among them, we noted diabetes mellitus in 81 cases (50.6%). The infection was located in the lower limbs in 155 cases (98%), in the face in two cases (1.3%) and in the upper limb in one case (0.7%). Twenty one patients (13.3%) presented with severe cellulitis and one presented with necrotizing fasciitis. All patients received intra venous antibiotic therapy. Surgical treatment was indicated in 14 cases. Cefazolin was prescribed in 77 cases (48%). Favorable evolution was noted in 144 patients (91.1%). Forty four patients (27.8%) received prophylactic antibiotics. Prevention of skin and soft tissue infection is a crucial step to preserve health in aged persons.
Assuntos
Antibacterianos/uso terapêutico , Celulite (Flegmão)/epidemiologia , Fasciite Necrosante/epidemiologia , Idoso , Idoso de 80 Anos ou mais , Antibioticoprofilaxia/métodos , Braço , Celulite (Flegmão)/terapia , Face , Feminino , Hospitalização , Humanos , Perna (Membro) , Masculino , Estudos Retrospectivos , Índice de Gravidade de Doença , Distribuição por Sexo , Tunísia/epidemiologiaRESUMO
Multifocal tuberculosis is defined as the presence of lesions affecting at least two extrapulmonary sites, with or without pulmonary involvement. This retrospective study of 10 cases aims to investigate the clinical and evolutionary characteristics of multifocal tuberculosis. It included 41 cases with tuberculosis collected between 1999 and 2013. Ten patients had multifocal tuberculosis (24%): 9 women and 1 man, the average age was 50 years (30-68 years). Our patients were correctly BCG vaccinated. The evaluation of immunodepression was negative in all patients. 7 cases had lymph node tuberculosis, 3 cases digestive tuberculosis, 2 cases pericardial tuberculosis, 2 cases osteoarticular tuberculosis, 1 case brain tuberculosis, 2 cases urinary tuberculosis, 4 cases urogenital tuberculosis, 1 case adrenal tuberculosis, 1 case cutaneous and 1 case muscle tuberculosis. All patients received anti-tuberculosis treatment for a mean duration of 10 months, with good evolution. Multifocal tuberculosis is difficult to diagnose. It can affect immunocompetent patients but often has good prognosis. Anti-tuberculosis therapy must be initiated as soon as possible to avoid sequelae.
Assuntos
Antituberculosos/uso terapêutico , Imunocompetência , Tuberculose/epidemiologia , Adulto , Idoso , Vacina BCG/administração & dosagem , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Retrospectivos , Resultado do Tratamento , Tuberculose/tratamento farmacológico , Tuberculose/patologiaRESUMO
The occurrence of renal and multiple organ Amyloïdosis is currently considered exceptional in the course of systemic lupus erythematosus. We report a case of a concomitant SLE and Amyloïdosis in a 57 year old female patient with hypothyroidism history, who presented with erythema nodosum, fever, arthralgia and sicca syndrome. Biological findings showed an inflammatory syndrome, renal failure, proteinuria (1g / 24h), positive auto antibodies and anti DNA. Lung radiology revealed medistinal lymphadenopathy, pleural nodules, ground glass infiltrates and pleuritis. Bronchial biopsy showed non specific inflammation. The salivary gland biopsy showed amyloïd deposits. This case report reminds us that lupus and Amyloïdosis association, although exceptional remains possible. The occurrence of Lofgren syndrome in this situation make the originality of this report.