Assuntos
Doenças Ósseas Metabólicas/diagnóstico , Cútis Laxa/diagnóstico , Ossificação Heterotópica/diagnóstico , Pseudoxantoma Elástico/diagnóstico , Dermatopatias Genéticas/diagnóstico , Adulto , Doenças Ósseas Metabólicas/complicações , Cútis Laxa/complicações , Feminino , Humanos , Ossificação Heterotópica/complicações , Pseudoxantoma Elástico/complicações , Dermatopatias Genéticas/complicaçõesRESUMO
Eosinophilic fasciitis is an uncommon connective tissue disease that may mimic and overlap with other sclerosing disorders such as morphea and lichen sclerosus. Herein, we report four patients (two men and two women, aged 16-64 yeas) with eosinophilic fasciitis. There was overlap with both morphea and lichen sclerosus in 2 patients and with morphoea alone in 1 patient. Magnetic resonance imaging (MRI) was used for diagnosis in three patients and for assessing treatment response in one patient. Eosinophilic fasciitis may co-exist with morhoea and lichen sclerosus. In view of the overlapping clinical and histopathological features of these disorders, MRI may be helful in delineating the conditions by detecting involvement of fascia.
Assuntos
Corticosteroides/administração & dosagem , Imagem Ecoplanar/métodos , Eosinofilia/patologia , Fasciite/patologia , Líquen Escleroso e Atrófico/patologia , Esclerodermia Localizada/patologia , Adolescente , Biópsia por Agulha , Diagnóstico Diferencial , Eosinofilia/diagnóstico , Eosinofilia/tratamento farmacológico , Fasciite/diagnóstico , Fasciite/tratamento farmacológico , Feminino , Seguimentos , Humanos , Imuno-Histoquímica , Líquen Escleroso e Atrófico/diagnóstico , Líquen Escleroso e Atrófico/tratamento farmacológico , Masculino , Pessoa de Meia-Idade , Terapia PUVA/métodos , Medição de Risco , Estudos de Amostragem , Esclerodermia Localizada/diagnóstico , Esclerodermia Localizada/tratamento farmacológico , Resultado do TratamentoAssuntos
Acantoma/diagnóstico , Acantoma/terapia , Idoso de 80 Anos ou mais , Crioterapia , Feminino , HumanosAssuntos
Pênfigo/diagnóstico , Pênfigo/terapia , Pênis/patologia , Humanos , Masculino , Pessoa de Meia-IdadeRESUMO
BACKGROUND: Pemphigus autoantibodies have been reported in healthy relatives of pemphigus patients suggesting a genetic predisposition in the pathogenesis of the disease. AIMS: To test for the presence of pemphigus autoantibodies in healthy relatives of Turkish patients of pemphigus. METHODS: The study group comprised 45 pemphigus patients, 75 unaffected family members and 47 healthy individuals in the control group. Direct and indirect immunofluorescence techniques were performed to determine the presence of pemphigus autoantibodies. RESULTS: By indirect immunofluorescence staining, circulating pemphigus autoantibodies were found in 26.7% of the relatives and in only two of the controls (P value = 0.0001). A direct immunofluorescence technique revealed positive results in three (4%) of the relatives and none of the controls. CONCLUSION: The presence of pemphigus autoantibodies in clinically healthy relatives indicates that genetic predisposition is necessary but not sufficient for the development of clinical disease.
Assuntos
Autoanticorpos/sangue , Predisposição Genética para Doença , Pênfigo/imunologia , Adulto , Idoso , Biópsia , Feminino , Imunofluorescência , Humanos , Imunoglobulina G/sangue , Masculino , Pessoa de Meia-Idade , Pênfigo/genética , Pele/imunologia , TurquiaRESUMO
Bullae occurring in lesions of morphea are uncommon. The cause of bullae formation in morphea is multifactorial, although lymphatic obstruction from the sclerodermatous process is considered the likeliest cause. Bullous morphea may be confused clinically with lichen sclerosus et atrophicus since both diseases may cause bullae in sclerodermatous plaques. A 69-year-old woman presented with a history of generalized morphea diagnosed 9 years earlier; and a 1-month history of pruritic bullae on her inframammary folds, axillary regions, lower abdomen, upper extremities and inguinal folds. Physical examination revealed multiple erythematous erosions, hemorrhagic vesicles and eroded bullae with slight scale or crusts overlying hypopigmented, indurated, shiny plaques. Skin biopsy revealed prominent edema in the papillary dermis, resulting in bulla formation and thickening of collagen fibers within the dermis. Direct immunofluorescence was negative. According to histologic and clinical features, the diagnosis of bullous morphea was established.