RESUMO
Long term D-penicillamine therapy, especially when used to treat Wilson's disease has been shown to cause elastosis perforans serpiginosa, pseudoxanthoma elasticum perforans and other degenerative dermatoses. We report a 23-year-old male patient who presented with multiple firm papules, nodules over the neck, axillae, front of elbows for five years. He was a known case of Wilson's disease on long-term treatment with penicillamine for the past 12 years. The papulonodular lesions were non-tender and some were discrete while others were arranged in a circinate pattern. There was central scarring of the skin within the circinate lesions. In addition, there were several small yellowish papules on both sides of the neck which eventually became confluent to form plaques. Histopathology confirmed the diagnosis of elastosis perforans serpiginosa and pseudoxanthoma elasticum. He was treated with cryotherapy (using liquid nitrogen through cryojet) for former lesions. The lesions showed remarkable improvement after five sittings. Now the patient is under trientine hydrochloride (750 mg twice daily) for Wilson's disease.
Assuntos
Tecido Elástico/patologia , Penicilamina/efeitos adversos , Pseudoxantoma Elástico/induzido quimicamente , Pseudoxantoma Elástico/patologia , Adulto , Biópsia por Agulha , Crioterapia/métodos , Relação Dose-Resposta a Droga , Seguimentos , Degeneração Hepatolenticular/diagnóstico , Degeneração Hepatolenticular/tratamento farmacológico , Humanos , Imuno-Histoquímica , Masculino , Penicilamina/uso terapêutico , Pseudoxantoma Elástico/terapia , Índice de Gravidade de Doença , Resultado do TratamentoRESUMO
Keratoderma of the palms and soles is not of rare occurrence. Its association with leukokeratosis of the mouth is well recognized. We describe two siblings having callosities like keratoderma over the weight bearing areas of soles, pressure areas of palms and palmar creases with leukokeratosis of cheeks. On study of the pedigree of these probands we could trace the trait transmission by a rare X-linked dominant mechanism.
Assuntos
Herança Extracromossômica , Ceratodermia Palmar e Plantar/diagnóstico , Ceratodermia Palmar e Plantar/genética , Doenças da Boca/diagnóstico , Doenças da Boca/genética , Adulto , Diagnóstico Diferencial , Humanos , Ceratodermia Palmar e Plantar/complicações , Ceratodermia Palmar e Plantar/patologia , Masculino , Doenças da Boca/complicações , Doenças da Boca/patologia , Linhagem , IrmãosAssuntos
Vacinas Bacterianas/uso terapêutico , Mycobacterium/imunologia , Psoríase/tratamento farmacológico , Vacinas Bacterianas/administração & dosagem , Humanos , Injeções Intradérmicas , Projetos Piloto , Psoríase/patologia , Índice de Gravidade de Doença , Resultado do Tratamento , Vacinas de Produtos Inativados/administração & dosagem , Vacinas de Produtos Inativados/uso terapêuticoRESUMO
A 70-year-old woman was having bullous pemphigoid. The blood tests revealed leucocytosis and eosinophilic leukemoid picture, confirmed later on with bone marrow aspiration.
