RESUMEN
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.
Asunto(s)
Papulosis Atrófica Maligna/patología , Piel/patología , Biopsia , Fármacos Dermatológicos/uso terapéutico , Femenino , Humanos , Papulosis Atrófica Maligna/tratamiento farmacológico , Papulosis Atrófica Maligna/fisiopatología , Persona de Mediana Edad , Necrosis , Tasa de SupervivenciaRESUMEN
A diagnosis was arrived at by doing something that the patient's other doctors hadn't: perform a biopsy.
Asunto(s)
Aspirina/uso terapéutico , Papulosis Atrófica Maligna/diagnóstico , Papulosis Atrófica Maligna/tratamiento farmacológico , Papulosis Atrófica Maligna/fisiopatología , Pentoxifilina/uso terapéutico , Inhibidores de Agregación Plaquetaria/uso terapéutico , Enfermedades de la Piel/diagnóstico , Enfermedades de la Piel/tratamiento farmacológico , Biopsia/métodos , Femenino , Humanos , Persona de Mediana Edad , Enfermedades de la Piel/fisiopatología , Resultado del TratamientoRESUMEN
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.