RESUMO
Porokeratotic eccrine ostial and dermal duct nevus is a rare disorder characterized by distinctive histopathology. We describe a 6-year-old boy who had the typical palmar involvement and small discrete areas involving the midline of his back.
Assuntos
Glândulas Écrinas , Nevo Intradérmico/patologia , Poroceratose/patologia , Neoplasias Cutâneas/patologia , Criança , Mãos , Humanos , Masculino , Nevo Intradérmico/terapia , Poroceratose/terapia , Neoplasias Cutâneas/terapiaRESUMO
A patient with both neurofibromatosis type 1 (NF-1) and McCune-Albright syndrome is described. NF-1 and McCune-Albright are separate entities and this is the first report of a patient with clear evidence of both conditions.
Assuntos
Displasia Fibrosa Poliostótica/complicações , Neurofibromatose 1/complicações , Criança , Feminino , Displasia Fibrosa Poliostótica/diagnóstico , Displasia Fibrosa Poliostótica/fisiopatologia , Humanos , Imageamento por Ressonância Magnética , Neurofibromatose 1/diagnóstico , Neurofibromatose 1/fisiopatologia , Glioma do Nervo Óptico/complicações , Glioma do Nervo Óptico/diagnóstico , Glioma do Nervo Óptico/fisiopatologia , Osso Esfenoide , Acuidade VisualRESUMO
Common or relatively common skin conditions which may be seen in the first month of life are considered. These include benign transient lesions, developmental and pigmentary abnormalities, vascular disorders and infections.
Assuntos
Dermatopatias , Humanos , Recém-Nascido , Dermatopatias/diagnóstico , Dermatopatias/terapiaRESUMO
Angiosarcoma has frequently been described arising within chronic lymphoedema of the upper limb following mastectomy and radiotherapy for carcinoma of the breast. We report a case of angiosarcoma arising in a lymphoedematous leg that had been subjected to radiotherapy 20 years previously for Hodgkin's disease. The diagnosis was expedited once the patient noticed the development of bleeding nodules. Prognosis of angiosarcoma is poor with treatment options being wide-excision surgery, palliative radiotherapy or chemotherapy. Unusual bruised areas or bleeding nodules developing within chronic lymphoedematous limbs should be biopsied to exclude the diagnosis.
Assuntos
Perna (Membro) , Linfangiossarcoma/etiologia , Linfedema/complicações , Segunda Neoplasia Primária/etiologia , Neoplasias Cutâneas/etiologia , Idoso , Idoso de 80 Anos ou mais , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Doença Crônica , Evolução Fatal , Doença de Hodgkin/radioterapia , Humanos , Perna (Membro)/efeitos da radiação , Linfoma não Hodgkin/tratamento farmacológico , Masculino , Segunda Neoplasia Primária/tratamento farmacológico , Cuidados Paliativos , PrognósticoAssuntos
Anti-Infecciosos/uso terapêutico , Dermatoses do Pé/tratamento farmacológico , Dermatoses da Mão/tratamento farmacológico , Ácido Salicílico/uso terapêutico , Verrugas/tratamento farmacológico , Adolescente , Criança , Pré-Escolar , Dermatoses do Pé/diagnóstico , Dermatoses da Mão/diagnóstico , Humanos , Verrugas/diagnósticoRESUMO
A pedigree showing the uncommon association of palmoplantar keratoderma with sensorineural deafness is described.
Assuntos
Surdez/complicações , Ceratodermia Palmar e Plantar Difusa/complicações , Criança , Pré-Escolar , Surdez/genética , Feminino , Humanos , Ceratodermia Palmar e Plantar Difusa/genética , Ceratodermia Palmar e Plantar Difusa/patologia , Masculino , LinhagemRESUMO
A 14-year-old girl with partial unilateral lentiginosis affecting the left side of the neck, left shoulder and left upper arm is described. She also had a further patch of multiple lentigines over the right side of the face.
Assuntos
Lentigo , Adolescente , Diagnóstico Diferencial , Feminino , Humanos , Lentigo/diagnóstico , Lentigo/patologia , Lentigo/fisiopatologiaRESUMO
We have previously shown that cimetidine, given concurrently for 2 weeks to patients on chronic dapsone therapy, reduced methaemoglobinaemia by inhibiting the formation of the toxic hydroxylamine metabolite of dapsone. The aim of the present study was to examine the effect of this combination on the benefit/toxic ratio of dapsone over a longer period. Eight patients (six dermatitis herpetiformis, one linear IgA disease, one folliculitis decalvans) on long-term dapsone 50-100 mg daily, took cimetidine 1.6 g daily concurrently for 3 months. At 3-weekly intervals, a clinical assessment was made, plasma dapsone and methaemoglobin were measured, and parameters of oxidative haemolysis were monitored. The dapsone level rose from 2298 +/- 849 ng/ml (mean +/- SD) at baseline to 3006 +/- 1131 ng/ml at week 3 of cimetidine (P < 0.01). This rise in plasma dapsone was sustained during cimetidine administration, falling to 2446 +/- 954 ng/ml when cimetidine was stopped (P < 0.02). The methaemoglobin fell from 5.5 +/- 2.2% (mean +/- SD) at baseline to 3.9 +/- 1.1% at week 3 (P < 0.01), and remained low until week 12, when there was a return to baseline values (P < 0.01). The haemoglobin did not change from the baseline of 12.7 +/- 0.3 g/dl (mean +/- SD), and other parameters of haemolysis were unaltered. There was a fall in the visual analogue score for headache (P < 0.05), but this was not associated with any deterioration in control of the skin disorders. Hence, long-term concurrent cimetidine results in increased plasma dapsone levels without increased haemolysis, and is accompanied by reduced methaemoglobinaemia for more than 2 months.(ABSTRACT TRUNCATED AT 250 WORDS)