RESUMO
A 63-year-old black female patient with blisters and exulcerations on the face, neck, upper limbs, and subsequent evolution with hypochromic sclerotic areas and alopecia, is reported. Chronic hepatitis C and presence of high levels of porphyrins in urine were demonstrated. There was complete remission with the use of hydroxychloroquine, photoprotection, and treatment of hepatitis. Significant sclerodermoid involvement of the skin as a manifestation of porphyria cutanea tarda secondary to hepatitis C emphasizes the importance of diagnostic suspicion regarding skin manifestation in order to indicate the appropriate therapy, and to minimize the hepatic morbidity.
Assuntos
Hepatite C Crônica/complicações , Hepatite C Crônica/patologia , Porfiria Cutânea Tardia/etiologia , Porfiria Cutânea Tardia/patologia , Esclerodermia Localizada/etiologia , Esclerodermia Localizada/patologia , Alopecia/etiologia , Feminino , Hepatite C Crônica/terapia , Humanos , Pessoa de Meia-Idade , Porfiria Cutânea Tardia/terapia , Esclerodermia Localizada/terapia , Resultado do TratamentoRESUMO
Morphea, or localized scleroderma, is a rare disease of the connective tissue that manifests itself with localized sclerosis of the skin and, in some cases, with extracutaneous manifestations. Its etiology is not fully understood, but it is believed that there is genetic predisposition, in addition to environmental triggering factors. Classification of the disease is not simple due to its multiple presentations; however, it is useful in order to define the treatment, which should be individualized and started early to avoid cosmetic and functional complications. In this review, we summarize the most important practical aspects of the classification, diagnostic methods and evaluation of morphea activity, as well as available therapeutic options, with an emphasis on existing clinical evidence regarding their efficacy and safety.
Assuntos
Humanos , Masculino , Feminino , Esclerodermia Localizada/classificação , Esclerodermia Localizada/diagnóstico , Esclerodermia Localizada/etiologia , Esclerodermia Localizada/terapia , Fototerapia/métodos , Prognóstico , Índice de Gravidade de Doença , Fatores Sexuais , Terapia por Exercício , ImunossupressoresRESUMO
Abstract: A 63-year-old black female patient with blisters and exulcerations on the face, neck, upper limbs, and subsequent evolution with hypochromic sclerotic areas and alopecia, is reported. Chronic hepatitis C and presence of high levels of porphyrins in urine were demonstrated. There was complete remission with the use of hydroxychloroquine, photoprotection, and treatment of hepatitis. Significant sclerodermoid involvement of the skin as a manifestation of porphyria cutanea tarda secondary to hepatitis C emphasizes the importance of diagnostic suspicion regarding skin manifestation in order to indicate the appropriate therapy, and to minimize the hepatic morbidity.
Assuntos
Humanos , Feminino , Pessoa de Meia-Idade , Esclerodermia Localizada/etiologia , Porfiria Cutânea Tardia/etiologia , Porfiria Cutânea Tardia/patologia , Hepatite C Crônica/complicações , Hepatite C Crônica/patologia , Esclerodermia Localizada/patologia , Esclerodermia Localizada/terapia , Resultado do Tratamento , Porfiria Cutânea Tardia/terapia , Hepatite C Crônica/terapia , Alopecia/etiologiaRESUMO
Morphea, or localized scleroderma, is a rare disease of the connective tissue that manifests itself with localized sclerosis of the skin and, in some cases, with extracutaneous manifestations. Its etiology is not fully understood, but it is believed that there is genetic predisposition, in addition to environmental triggering factors. Classification of the disease is not simple due to its multiple presentations; however, it is useful in order to define the treatment, which should be individualized and started early to avoid cosmetic and functional complications. In this review, we summarize the most important practical aspects of the classification, diagnostic methods and evaluation of morphea activity, as well as available therapeutic options, with an emphasis on existing clinical evidence regarding their efficacy and safety.
Assuntos
Esclerodermia Localizada , Terapia por Exercício , Feminino , Humanos , Imunossupressores , Masculino , Fototerapia/métodos , Prognóstico , Esclerodermia Localizada/classificação , Esclerodermia Localizada/diagnóstico , Esclerodermia Localizada/etiologia , Esclerodermia Localizada/terapia , Índice de Gravidade de Doença , Fatores SexuaisRESUMO
Scleroderma is a rare connective tissue disease that is manifested by cutaneous sclerosis and variable systemic involvement. Two categories of scleroderma are known: systemic sclerosis, characterized by cutaneous sclerosis and visceral involvement, and localized scleroderma or morphea which classically presents benign and self-limited evolution and is confined to the skin and/or underlying tissues. Localized scleroderma is a rare disease of unknown etiology. Recent studies show that the localized form may affect internal organs and have variable morbidity. Treatment should be started very early, before complications occur due to the high morbidity of localized scleroderma. In this review, we report the most important aspects and particularities in the treatment of patients diagnosed with localized scleroderma.
Assuntos
Esclerodermia Localizada/patologia , Esclerodermia Localizada/terapia , Pele/patologia , Biópsia , Feminino , Humanos , Masculino , Esclerodermia Localizada/classificação , Esclerodermia Localizada/etiologia , Escleroderma Sistêmico/patologiaRESUMO
Scleroderma is a rare connective tissue disease that is manifested by cutaneous sclerosis and variable systemic involvement. Two categories of scleroderma are known: systemic sclerosis, characterized by cutaneous sclerosis and visceral involvement, and localized scleroderma or morphea which classically presents benign and self-limited evolution and is confined to the skin and/or underlying tissues. Localized scleroderma is a rare disease of unknown etiology. Recent studies show that the localized form may affect internal organs and have variable morbidity. Treatment should be started very early, before complications occur due to the high morbidity of localized scleroderma. In this review, we report the most important aspects and particularities in the treatment of patients diagnosed with localized scleroderma.
Assuntos
Feminino , Humanos , Masculino , Esclerodermia Localizada/patologia , Esclerodermia Localizada/terapia , Pele/patologia , Biópsia , Esclerodermia Localizada/classificação , Esclerodermia Localizada/etiologia , Escleroderma Sistêmico/patologiaRESUMO
Scleroderma is a multiorganic disease characterized by inflammatory, vascular and sclerotic changes in skin and internal organs. It is considered as a tripartite disease, associated to autoimmune, fibroblast and endothelial defect, due to genetic, environmental and infectious factors. This disease can be classified in systemic and localized form. The Raynaud phenomenon occurs in 90% of the patients with the diagnosis. It explains the microcirculation involvement and the reduction in the number of capillaries. Malformation of nail bed capillaries is readily demonstrated by nail bed microscopy and has been shown to correlate both with disease severity and with degree of internal organ involvement. The MRSS-51 validates the skin involvement and has the main predictive value to determine the patient survival. MRSS-51 should not be considered as an activity disease parameter or used to validate the effectiveness of treatment. Nowadays, multiple treatment alternatives exist for scleroderma disease; however these treatments offer poor results for the cutaneous manifestations.
Assuntos
Esclerodermia Localizada/etiologia , Esclerodermia Localizada/terapia , Escleroderma Sistêmico/etiologia , Escleroderma Sistêmico/terapia , HumanosRESUMO
We report the case of a caucasian woman of 44 years old with no personal medical history. She was diagnosed breast carcinoma and underwent right partial mastectomy with adjuvant radiotherapy and chemotherapy. Four years after the intervention, presented a indurated plaque. Biopsy was performed and the diagnosis of morpheawas confirmed by histopathological examination.