RESUMO
A diagnosis was arrived at by doing something that the patient's other doctors hadn't: perform a biopsy.
Assuntos
Aspirina/uso terapêutico , Papulose Atrófica Maligna/diagnóstico , Papulose Atrófica Maligna/tratamento farmacológico , Papulose Atrófica Maligna/fisiopatologia , Pentoxifilina/uso terapêutico , Inibidores da Agregação Plaquetária/uso terapêutico , Dermatopatias/diagnóstico , Dermatopatias/tratamento farmacológico , Biópsia/métodos , Feminino , Humanos , Pessoa de Meia-Idade , Dermatopatias/fisiopatologia , Resultado do TratamentoRESUMO
Köhlmeier-Degos disease is rare idiopathic vasculopathy, the exact pathogenesis of which remains unclear. Here, we review pertinent literatutre and present a case of a Köhlmeier-Degos disease with central nervous system involvement followed-up over 11â¯years with various neuroimaging modalities. Evolution of neurovascular and neuropathological changes over an extended time period has not been previously described.
Assuntos
Circulação Cerebrovascular , Transtornos Cerebrovasculares/fisiopatologia , Papulose Atrófica Maligna/fisiopatologia , Adulto , Encéfalo/diagnóstico por imagem , Encéfalo/patologia , Transtornos Cerebrovasculares/diagnóstico por imagem , Transtornos Cerebrovasculares/etiologia , Progressão da Doença , Feminino , Humanos , Hidrocefalia/diagnóstico por imagem , Hidrocefalia/etiologia , Angiografia por Ressonância Magnética , Imageamento por Ressonância Magnética , Papulose Atrófica Maligna/complicações , Papulose Atrófica Maligna/diagnóstico por imagem , Neuroimagem , Siderose/diagnóstico por imagem , Siderose/etiologiaRESUMO
BACKGROUND: Atrophic papulosis is a rare thrombo-occlusive disease, characterized by the appearance of multiple atrophic porcelain-white skin papules, with a surrounding erythematous rim, which are histologically consisting of wedge-shaped necrosis of the dermis. OBJECTIVE: It consists of two variants: (i) the benign atrophic papulosis (BAP) only involving the skin and (ii) the malignant atrophic papulosis (MAP) also involving several internal organs with a cumulative five-year survival rate of approx. 55%. While the probability of only having a BAP at onset is approximately 70%, increasing to 97% after 7 years of monosymptomatic cutaneous course, no close long-term follow-up of the development of the skin lesions has been reported. METHODS: We present a precise visual documentation of the evolution of the disseminated skin lesions in a female patient with BAP spanning over two decades. RESULTS: A considerable improvement and/or clinical resolution of the majority of the lesions disputing the scarring character of the atrophic porcelain-white skin papules has been detected. CONCLUSION: BAP not only exhibits an excellent prognosis, but resolution of lesions can also occur after a considerable period of time.
