RESUMEN
Antiphospholipid syndrome (APS) is an acquired thrombophilic disorder in which autoantibodies are produced against a variety of phospholipids and phospholipid-binding proteins. The purpose of this article is to review cutaneous findings in patients with APS diagnosis. An overview regarding prevalence, description, pathogenesis and histopathology, are described for cutaneous manifestations of APS.
Asunto(s)
Anticuerpos Antifosfolípidos/inmunología , Síndrome Antifosfolípido/patología , Livedo Reticularis/patología , Enfermedades de la Piel/patología , Vasculitis/patología , Adulto , Anciano , Anetodermia/etiología , Anetodermia/patología , Anticuerpos Anticardiolipina/inmunología , Síndrome Antifosfolípido/diagnóstico , Síndrome Antifosfolípido/epidemiología , Síndrome Antifosfolípido/inmunología , Biopsia , Femenino , Gangrena/etiología , Gangrena/patología , Humanos , Livedo Reticularis/diagnóstico , Livedo Reticularis/etiología , Livedo Reticularis/inmunología , Inhibidor de Coagulación del Lupus/inmunología , Masculino , Papulosis Atrófica Maligna/etiología , Papulosis Atrófica Maligna/patología , Persona de Mediana Edad , Necrosis/diagnóstico , Necrosis/etiología , Prevalencia , Enfermedades de la Piel/inmunología , Úlcera/patología , Vasculitis/etiologíaRESUMEN
White diseases are a heterogenous group characterized by hypopigmentation or depigmentation. Skin and eye color are determined by the number and size of melanosomes present. Melanin is produced by melanosomes in the melanocytes present within the epidermis of the skin, uvea, and retinal pigmented epithelium (RPE). Conditions altering the number of melanocytes or concentration of melanin result in a lack of pigmentation, appearing as "white diseases" ranging from the well-known albinism and vitiligo to more esoteric white hand syndrome and Degos disease.
Asunto(s)
Hipopigmentación/diagnóstico , Hipopigmentación/etiología , Albinismo/diagnóstico , Albinismo/etiología , Albinismo/terapia , Color , Cosméticos/efectos adversos , Diagnóstico Diferencial , Humanos , Hipopigmentación/patología , Hipopigmentación/terapia , Inflamación/complicaciones , Liquen Escleroso y Atrófico/diagnóstico , Liquen Escleroso y Atrófico/etiología , Liquen Escleroso y Atrófico/patología , Liquen Escleroso y Atrófico/terapia , Papulosis Atrófica Maligna/diagnóstico , Papulosis Atrófica Maligna/etiología , Papulosis Atrófica Maligna/patología , Membrana Mucosa , Enfermedades de la Uña/etiología , Nevo con Halo/diagnóstico , Nevo con Halo/etiología , Nevo con Halo/patología , Pitiriasis Liquenoide/diagnóstico , Pitiriasis Liquenoide/etiología , Pitiriasis Liquenoide/terapia , Pronóstico , Preparaciones para Aclaramiento de la Piel/efectos adversos , Tiña Versicolor/diagnóstico , Tiña Versicolor/tratamiento farmacológico , Tiña Versicolor/etiología , Vibración/efectos adversos , Vitíligo/diagnóstico , Vitíligo/etiología , Vitíligo/terapia , Síndrome de Waardenburg/diagnóstico , Síndrome de Waardenburg/etiologíaRESUMEN
Degos' disease or malignant atrophic papulosis is a rare disseminated occlusive vasculopathy affecting the skin, gastrointestinal tract, central nervous system, and less often other organ systems. The exact etiology of this vasculopathy has not been established. Infections, autoimmune disease and coagulation defects have been proposed as underlying pathogenic mechanisms, but none have been confirmed. Here, we report the clinical, radiological and histopathologic features of Degos' disease in a 41-year-old man following streptococcal throat infection. Prior postulated hypothesis as post-infectious immunologic mechanism may be further supported by this case.
Asunto(s)
Papulosis Atrófica Maligna/complicaciones , Papulosis Atrófica Maligna/etiología , Infecciones Estreptocócicas/complicaciones , Enfermedades Vasculares/etiología , Adulto , Infecciones del Sistema Nervioso Central/microbiología , Progresión de la Enfermedad , Enfermedades Gastrointestinales/microbiología , Humanos , Masculino , Papulosis Atrófica Maligna/diagnóstico por imagen , Papulosis Atrófica Maligna/patología , Radiografía , Infecciones Cutáneas Estafilocócicas/complicaciones , Infecciones Estreptocócicas/diagnóstico por imagen , Infecciones Estreptocócicas/patología , Enfermedades Vasculares/diagnóstico por imagen , Enfermedades Vasculares/patologíaRESUMEN
Degos' disease or malignant atrophic papulosis is a rare vasculopathy characterized by the presence of a typical skin lesion and visceral vascular involvement of small vessels, mainly of the digestive tract or central nervous system. The most interesting fact in this disease is the benign appearance of cutaneous lesion, hiding the occlusion of skin and visceral vessels. The author reports the case of a female patient with systemic lupus erythematosus for eight years. During her follow up, generalized skin papules were observed on the trunk and limbs, sparing her face, hands and feet, compatible with Degos' disease. Additional imaging investigation excluded systemic involvement of the disease. Treatment with acetylsalicylic acid prevented the appearance of new cutaneous manifestations and the patient remains clinically stable on the Outpatient Clinic without complications, until this moment. Malign atrophic papulosis is a rare disease with a poor prognosis. However, its association with systemic lupus erythematosus seems to follow a more benign course, without the typical visceral involvement.