RESUMO
Background: Blue rubber bleb nevus syndrome is a rare clinical entity. Case characteristics: A 13-year-old Indian boy presented with characteristic cutaneous lesions, gastrointestinal malformations, skeletal involvement and pulmonary stenosis. Observation: Diagnosis was confirmed on skin biopsy, radiographic evaluation, colonoscopy and echocardiography. Echocardiography revealed pulmonary stenosis, an association hitherto undescribed. Message: Detailed evaluation in a patient of blue rubber bleb nerves syndrome is mandatory.
RESUMO
Solitary mastocytoma is known to occur predominantly in children below 2 years of age and onset in adulthood is rare. Lesions are hyperpigmented in the majority of cases owing to the stimulation of melanin synthesis by mast cell growth factor. We hereby report two patients with adult onset solitary mastocytoma presenting as hypopigmented plaque. The first case was a 24-year-old man who presented with a plaque on the back of the neck of 5 years duration. The second case was a 30-year-old man who had a well-defined solitary, oval 3 x 2.5 cm plaque on the nape of the neck. Stroking of lesion resulted in a wheal with flare (Darier's sign) in both cases. Systemic examination was within normal limits in both cases. Histopathology revealed a dense toluidine blue-positive infiltrate of mast cells in the upper dermis in both cases.
Assuntos
Adulto , Idade de Início , Biópsia , Derme/patologia , Eosinófilos/patologia , Humanos , Hipopigmentação/patologia , Masculino , Mastócitos/patologia , Mastocitoma Cutâneo/patologia , PescoçoAssuntos
Adulto , Doadores de Sangue , DNA Viral/sangue , Feminino , Hepatite B/imunologia , Anticorpos Anti-Hepatite B/sangue , Antígenos do Núcleo do Vírus da Hepatite B/sangue , Antígenos de Superfície da Hepatite B/sangue , Antígenos E da Hepatite B/sangue , Humanos , Testes de Função Hepática , MasculinoAssuntos
Humanos , Índia , Hanseníase Dimorfa/complicações , Masculino , Pessoa de Meia-Idade , Psoríase/complicaçõesRESUMO
A case of hepatic hemangioendothelioma presenting as congestive cardiac failure in a neonate is reported. There was also evidence of platelet consumption. The case was managed successfully with oral prednisolone, resulting in improvement of symptoms and tumor regression over 3 months.
Assuntos
Progressão da Doença , Feminino , Glucocorticoides/uso terapêutico , Hemangioendotelioma/diagnóstico , Humanos , Recém-Nascido , Neoplasias Hepáticas/diagnóstico , Prednisolona/uso terapêuticoRESUMO
Eye involvement in chronic cutaneous lupus erythematosus (CCLE) has been reported infrequently. We describe two cases of disseminated CCLE, a 50-year-old woman and a 45-year-old man, with eyelid involvement and disease duration of 4 and 20 years respectively. The first patient showed an excellent response to chloroquine while there was lack of response in the second patient.
Assuntos
Doenças Palpebrais/complicações , Feminino , Humanos , Lúpus Eritematoso Discoide/complicações , Masculino , Pessoa de Meia-IdadeRESUMO
Bilateral lagophthalmos secondary to facial nerve is extremely uncommon. Further, the aetiology in most of these cases is of central origin unlike the peripheral involvement in leprosy. A patient of lepromatous leprosy (LL) may be euthyroid or hypothyroid on account of leprous involvement of the thyroid gland. A case of LL with bilateral lagophthalmos and hyperthyroidism is reported.
Assuntos
Adulto , Nervo Facial/fisiopatologia , Paralisia Facial/microbiologia , Humanos , Hipertireoidismo/complicações , Hanseníase Virchowiana/complicações , Masculino , Mycobacterium lepraeRESUMO
Extralobar pulmonary sequestration is a rare pulmonary parenchymal anomaly which rarely may be present in an intra-abdominal location. The authors report a case of intra-abdominal extralobar pulmonary sequestration which presented to us as an antenatally diagnosed suprarenal mass and was worked up as such. The diagnosis was revealed only at laparotomy. Intra-abdominal extralobar pulmonary sequestration should also be kept in differential diagnosis in cases of masses in the suprarenal location, especially on the left side.
Assuntos
Doenças das Glândulas Suprarrenais/diagnóstico por imagem , Sequestro Broncopulmonar/diagnóstico por imagem , Diagnóstico Diferencial , Feminino , Humanos , Recém-Nascido , Masculino , Gravidez , Ultrassonografia Pré-NatalAssuntos
Pré-Escolar , Procedimentos Cirúrgicos do Sistema Digestório/métodos , Seguimentos , Hemorragia Gastrointestinal/etiologia , Humanos , Íleo/anormalidades , Mucosa Intestinal/patologia , Masculino , Doenças Retais/etiologia , Medição de Risco , Sensibilidade e Especificidade , Pertecnetato Tc 99m de Sódio/diagnóstico , Resultado do TratamentoRESUMO
One hundred and six consecutive patients (old and newly diagnosed) attending the leprosy clinic at Guru Teg Bahadur Hospital, Delhi, underwent a detailed ophthalmic examination to identify factors associated with the prevalence of sight threatening lesions of leprosy so as to delineate a sub-group of patients who should continue under active ophthalmic surveillance to prevent blindness. All of them were receiving multidrug therapy as recommended by WHO. Demographic data, systemic parameters and ophthalmic lesions attributable to the disease were endorsed on to a proforma. The data were analysed by chi-square test, discriminant analysis and multivariate regression analysis to isolate factors that could be associated with sight threatening lesions of leprosy. In 106 leprosy patients 212 eyes were examined; sight threatening lesions were found in 37 eyes (17.4%). The comea was involved most commonly (78.3%). The lesions were still present in 15.8% of patients who had completed multidrug therapy. Leprosy related visual impairment was seen in 2.3% eyes and blindness in 0.9% eyes. Factors significantly associated with sight threatening lesions included female gender, multibacillary leprosy, increasing duration of leprosy, thickened corneal nerves, subepithelial punctate corneal opacities and presence of any academic lesion of leprosy. On multivariate and discriminant analysis, female gender, thickened corneal nerves and duration of leprosy > 7 years were significantly associated with sight threatening lesions.
Assuntos
Adolescente , Adulto , Idoso , Cegueira/epidemiologia , Criança , Progressão da Doença , Feminino , Humanos , Incidência , Índia/epidemiologia , Hanseníase/complicações , Masculino , Pessoa de Meia-Idade , Polimedicação , Fatores de RiscoRESUMO
Fibromatosis, arare non-neoplastic spindle cell proliferation of unknown aetiology, can occur anywhere in the body. Though extra-abdominal sites are commonly involved, intra-abdominal fibromatosis has also been described. Described herein is an unusual case of diffuse intra-abdominal fibromatosis in a 9-year-old boy, who could not be salvaged despite extensive medical management.
Assuntos
Criança , Árvores de Decisões , Diagnóstico Diferencial , Evolução Fatal , Fibromatose Abdominal/diagnóstico , Humanos , Masculino , Tomografia Computadorizada por Raios XRESUMO
The case of a male patient diagnosed to have lepromatous leprosy with type 2 reaction on multibacillary multidrug therapy, with unusual, widespread involvement of genitalia in the form of plaque and nodules of leprosy over scrotum and perimeatal region of glans, necrotic lesions of erythema nodosum leprosum over scrotum, neuritis of genital branch of genitofemoral nerve bilaterally, and azoospermia, is reported.
Assuntos
Genitália Masculina/patologia , Humanos , Hansenostáticos/uso terapêutico , Hanseníase Virchowiana/tratamento farmacológico , Masculino , Pessoa de Meia-IdadeRESUMO
Twenty patients with Schamberg's disease were started on pentoxifylline (400 mg once daily) for a period of 8 weeks. Improvement was assessed at 2 weekly intervals by two observers independently and graded as mild (< 25%, moderate (25-50%) and marked (>50%). Marked improvement was observed in 10/20(50%) patients. We conclude that pentoxifylline should be considered as first line therapy in all patients with Schamberg's disease.
RESUMO
A 24-year-old male presenting with pityriasis versicolor developing on a pre-existing lesion of Becker's naevus is being reported for its rare occurrence.
RESUMO
Forty-five non-cirrhotic patients undergoing splenoportovenography (SPV) were studied. SPV was performed successfully in 43 (95.5%) patients. Local pain was encountered in 12 (26.6%) and lasted for a mean (+/- SD) of 7 +/- 3.8 hours. Local tenderness was noted in 17 (37.7%) and lasted for 6.8 +/- 3.7 hours. The mean rise in the pulse rate after the procedure was 6.5 +/- 4.7 beats per minute and the fall in the systolic and diastolic blood pressures was 3.4 +/- 2.5 and 0.8 +/- 1.7 mmHg respectively. Post-SPV splenic hematoma was noted in 3 (6.6%) patients. All were subcapsular, at the site of puncture, and disappeared by the seventh day. There was no difference in the age, change in the pulse rate, and fall in systolic or diastolic blood pressures between those who developed and those who did not develop hematoma. One patient required one unit of blood transfusion. There were no deaths. SPV was noted to be a simple and safe procedure with a high success rate with minimal morbidity and no mortality.